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Neurology RITE 2014 – Flashcards

Judith Simpson

7 July 2022

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Presenilin 2 (Chromosome)

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Chromosome 1

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Paternal Transmission of Chromosome 15

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Prader-Willi

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Voltage gated Sodium Channel Myotonia of eyelids, face, and hands

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Paramyotonia Congenita

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Motor Regression, cherry red spot, optic atrophy to blindness, severe retardation, seizures Decreased hexosaminidase A Chromosome 5/15

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Tay-Sachs (GM2 Gangliosidosis)

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Recurrent infections with B cell neoplasia Chromosome 11 affected

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Ataxia-Teleangectasia

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Birefringent Ca Oxalate and oxalic acid in urine

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Ethylene Glycol toxicity

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Pseudoperiodic high voltage sharp and slow waves

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EEG findings with CJD

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Reduces the function of B12

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NO Toxicty

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High-voltage polyphasic sharp and slow wave complexes of 0.5 to 2 second duration that recur every 4-15 seconds

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EEG fingins with SSPE

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Mechanism of Tetrodotoxin

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Blocks Sodium Channels

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Malabsorption, peripheral neurolopathy, pigmentary retinopathy Treated with Vitamin E

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Bassen-Kornzweig

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Palatal Neuropathy 10% Develop defuse sensorimotor polyneuropathy 8-12 weeks after infection Increase protein in CSF

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Diptheira

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Dopamine refractory parkinsonism and dystonia, associated with liver disease Globus Pallidus and midbrain increased on T1

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Manganese Intoxication

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Astrocytic Tumor (Chromosome/gene)

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Chromosome 17 P53 gene

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Mechanism of Lathyrus toxicity (alpha-amino-beta-oxalaminoproprionic acid)

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Glutamate Toxicity

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Retinoblastoma (Chromosome/gene)

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Chromosome 13 pRB gene

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Latrotoxin (Source/mechanism)

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Black Widow Venom Releases acetylcholine

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Dopamine Responsive Dystonia (Chromosome)

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Chromosome 14

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Kugelberg-Welander (SMA type/Chromosome)

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SMA Type III Chromosome 5

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Charcot Marie Tooth Disease 1 (Chromosome/gene) Hereditory motor and sensory neuropathy 1 (Chromosome/gene)

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Chromosome 17/PMP-22 Chromosome 1/ P0

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Familial Hemiplegic Migraine (Channel/Chromosome)

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P/Q Calcium Channel Chromosome 19

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Pyramidal signs, peripheral neuropathy Autosomal dominant 6 CAG trinucleotide repeat

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Spinocerebellar Degeneration Type I

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Associated wiht Meningioma, schwanoma, glioma Chromosome 22

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NF 2

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Hemangioblastoma of CNS (cerebellum and retina) Associated with pheochromocytoma and renal cancer

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Von Hipple-Lindau

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"Rod bodies" Static muscle weakness, congenital hypotonia Autosomal dominate when on Chromosome 1 (most common) Autosomal Recessive when on Chromosome 2

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Nemaline Myopathy

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Infantile Onset SMA Type 1 SMN Gene Chromosome 5

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Werdnig-Hoffman

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Progressive external opthalmoplegia pathology

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Mitochondrial Ragged Red Fibers

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Voltage Gated sodium channel Autosomal Dominant on Chromosome 17 Treatement with Carbonic anhydrase inhibitors

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Hyperkalemic periodic paralysis

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Subacute necrotizing encephalopathy, respiratory disturbance, abnormal ocular motility, hypotonia Brainstem and 3rd ventricle lesions Decreased cytochrome C oxidase, mitochondrial dysfunction Autosomal recessive or X

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Leigh's Syndrome

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Phosphofructokinase deficiency Decreased myophosphorylase Chromosome 11 Cramps with exercise, mild weakness Subsarcolemmal Blebs

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McArdle's Disease

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Dihydropyridine receptor mutation (Voltage cated calcium channel) Autosomal Dominant Chromosome 1 Treatment with Potassium and carbonic anyhydrase inhibitors

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Hypokalemic Periodic Paralysis

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"Happy Puppet" Maternal transmission of chromosome 15

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Angelman Syndrome

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Spreading limpness on stiffness, hand myokymia, continuous motor unit activity on EMG Potassium channel gene Chromosome 12 Treatment with Acetazolamide or Phenytoin

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Episodic ataxia - 1

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Delayed motor development, involuntary movements, compulsive self mutilation Decreased hypoxantine guanine phosphorribosyltransferase Increased uric acid Chromosome X

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Lesch-Nyhan

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Ataxia, retinitis pegmentosa, icthyosis Decreased phantic acid oxidase

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Refsum's Disease

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Ataxia, spasticity, optic atrophy, dementia, decreased MSR Lysosomal enzyme disorder Decreased aylsulftase A Chromosome 10

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Metachromatic Leukodystrophy

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Muscle stiffness (painless and worsened by cold) and hypertrophy Chloride channel Chromosome 7 Treatement with mexiletine, phenytoin, carbamazepine

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Myotonia congenita

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Peripheral involvement Van Recklinghausen Chromosome 17

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NF 1

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A form of spinocerebellar ataxia 6 Chromosome 19 half have migraines P/Q Calcium channel Treatment with acetazolamide

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Episodic Ataxia - 2

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Lyposomal enzyme disorder Decreased galactosylceramide beta galactosidase Chromosome 14 Onset at 3 to 6 months Progressive increased tone, decreased MSR, startle myoclonus, seizures, blindness, and slow development

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Krabbe's Leukodystrophy

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Acute infantile form Cherry red macula, hepatosplenomegaly, mental deterioration (psychomotor regression), postural hypotonia Decreased sphingomyelinase Vacuolated lymphocytes

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Niemann-Pick Type A

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Myotonia, facial and distal weakness, multiple endocrinopathies, temporal, masseter, and SCM wasting, distrubed GI motility 19 CTG trinucleotide repeat

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Myotonic Dystrophy

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Eponyms for Neuronal Ceroid Lipofuscinosis

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Santavuori-Haltia - 6-12 months on Chromosome 1 Jansky-Bielschowksy - 2-3 years on Chromosome 11 Batten or Speilmeyer-Vogt - 4-12 years on Chromosome 16

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Ataxia, dysarthia, decrease MSR, + babinski, decreased vibration sense, hypertrophic cardiomyopathy Frataxin 9 GAA trinucleotide repeat

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Friedreich's Ataxia

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Progressive dementia, hypotonia, ataxia, seizures (myclonus) Abnormal VEP and later blindness Lipofuscin strored in neurons

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Neuronal ceroid lipofuscinosis

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Dystonia, athetosis, rigidity, LMN signs and ataxia 14 CAG trinucleotide repeat

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Spinocerebellar Atrophy Type 3 (Machado-Joseph)

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Tuberous Sclerosis (Chromosome/Product)

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Chromosome 9, 16 Hemartin

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Narcolepsy (Chromosome)

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Chromosome 6

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Early onset primary dystonia DYT1 (Chromosome)

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Chromosome 9

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Amyloid Precursor Protein (Chromosome)

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Chromosome 21

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Apoplipoprotein E (Chromosome)

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Chromosome 19

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Alzheimer's - Late, familial (Chromosome)

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Chromosome 19

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Alzheimer's - Early, familial (Chromosome)

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Chromosome 14, 21

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Spastic Paraplegia (Chromosome)

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Chromosome X

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William's Syndrome (Chromosome)

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Chromosome 7

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Frontotemporal Dementia (Chromosome)

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Chromosome 17

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Wilson's Disease (Chromosome)

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Chromosome 13

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Acute Intermittent Porphyria (Chromosome)

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Chromosome 11

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CJD - Familial Gerstman-Schenker-Strauss (Chromosome)

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Chromosome 20

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Familial ALS (Chromosome)

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Chromosome 21

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medial medullary syndrome

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ipsilateral CNXII paralysis (fascicle of CNXII), contralateral hemiparesis (pyramid), and contralateral loss of position and vibratory sensation (medial lemniscus) anterior spinal artery distribution

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orbital apex components

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ophthalmic artery, ciliary ganglion and nerves, the fascicles of cranial nerves III, IV, VI, and the ophthalmic branch of cranial nerve V

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Damage to the fornix cause what S/Ss

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Papez circuit and results in loss of the ability to form new memories

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thalamus is supplied mainly 3A

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posterior cerebral, posterior communicating, and posterior choroidal

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name muscles trigeminal nerve supplies --the muscles of mastication

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temporalis, masseter, medial and lateral pterygoids, mylohyoid, anterior belly of the digastric, tensor veli palatini, and tensor tympani muscle

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suprachiasmatic nucleus of the hypothalamus

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dominant circadian pacemaker of the mammalian brain.

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anterior nucleus of the thalamus project to?

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Fibers from the anterior nucleus of the thalamus project to the cingulate gyrus. The anterior nucleus receives projections from the mamillary bodies

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Neural crest derivatives

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dorsal root ganglia (involved in transmission of pain) and sympathetic ganglia Schwann cells and melanocytes

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insular cortex in put

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receives visceral nociceptive input via the ventromedial posterior (VMP) thalamic nucleus

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The layers traversed during a lumbar puncture

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skin, subcutaneous fat, supraspinous ligament, intraspinous ligament, ligamentum flavum, epidural fat, subarachnoid space

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central cord lesion, most suggestive feature

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upper extremities greater than lower, Pro more affect than Dis decreased pinprick sensation with retained fine touch spare vibration and light touch

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Opioid intoxication, pupil?

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constrict

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Transient global amnesia

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temporary but almost total disruption of short-term memory with a range of problems accessing older memories

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Williams syndrome

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deletions on chromosome 7 excellent social and language skills Marked impairment is noted on tests of visual processing cardiac, hyperacusis, endocrine disorders, and hypercalcemia

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Klüver-Bucy syndrome

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bilateral temporal lesions involving amygdalae

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Define concussion

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Concussion is recognized as a clinical syndrome of biomechanically induced alteration of brain function, typically affecting memory and orientation, which may involve loss of consciousness (LOC).

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List 3 Diffuse astrocytomas ,WHO 2007

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WHO 2007 Diffuse astrocytomas 1, Low grade astrocytoma;(grade II) 2, anaplastic astrocytoma(III); 3, Glioblastoma (IV): mitotic figures prominent, vanscular proliferation and/or necrosis

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Pathological feature of low grade astrocytoma

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nuclear pleomorphism, mitotic figures rare

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Pathological feature anaplastic astrocytoma (grade III

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nuclear pleomorhism, mitotic figures prominent, NO vascular proliferation or necrosis

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Pathological feature Glioblastoma (IV)

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mitotic figures prominent, vanscular proliferation and/or necrosis

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Loss of Heterozygosity (LOH)

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occurs when a somatic cell contains only one copy of an allele due to non-disjunction during mitosis, segregation during recombination, or deletion of a chromosome segmen

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Primary gliobalstoma

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1,Glioblastoma arise de novo, without progression from lower grade glioma 2, IDH 1 mutation is rare 3, Loss of Heterozygosity 10q, EGFR amplification, p16 deletin and PTEN mutation

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Secondary glioblastoma

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1,Arise from lower grade glioma 2, Sequential acuisition of new mutation 3, IDH1 and P53 common 4, Temozolamide lead to hypermutation RB and Akt-mTOR pathway

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Diffuse gliomas favorable genetic signatures

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1p/19q co-deletion IDH mutation

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IDH mutation presents in what types of CNS tumors

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IDH1(R132H) prsent in 75% of grade II and III glioma Not present in primary glioblastomas Very rare in other types of primary CNS tumor

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prion disorders

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14-3-3 protein

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serotonin syndrome

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results from concomitant administration of medications that enhance serotonin transmission via decreased breakdown, or increased production

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clinical triad of abnormalities of serotonin syndrome

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Cognitive effects: headache, agitation, hypomania, mental confusion, hallucinations, coma Autonomic effects: shivering, sweating, hyperthermia, vasoconstriction, tachycardia, nausea, diarrhea. Somatic effects: myoclonus (muscle twitching), hyperreflexia (manifested by clonus), tremor.

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serotonin syndrome differentiated from neuroleptic malignant syndrome clinically

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serotonin syndrome has shiveringa and myoclonus

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serotonin syndrome how fast occur with medication changes

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within 6 to 8 hours of initiating or increasing serotonergic medications

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ideational apraxia

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the patient cannot correctly pantomime a multistep command

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ideomotor apraxia

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, in which the patient cannot perform on command simple actions such as waving good-bye or using a hammer

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Bromocriptine

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dopamine receptor agonist, which activates postsynaptic dopamine receptors. treatment of choice for neuroleptic malignant syndrome,

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Forced normalization

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psychosis occurring after achievement of good clinical seizure control, or resolution of interictal epileptiform discharges

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sarcoidosis CNS manifestatins

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motor and sensory deficits, progressive visual loss, and cranial nerve palsies. hypothalamus and basal forebrain resulting in hypopituitarism, hyperphagia, and hypersomnolence

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West Nile virus (WNV) encepahlitis

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older patients mental status change movement disorder flaccid paralytic syndrome

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Rabie virus

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temporal and limbic lobes-memory loss and personality changes, seizure and death

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medial dorsal nucleus of the thalamus connections (AOL)

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amygdala, orbitofrontal region, and the temporal lobe ---disinhibition, utilization behavior, mania, and memory loss

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why need check glucose before tPA

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Hypoglycemia can present with signs of focal neurologic dysfunction and mimic stroke.

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fourth nerve palsy, head posiion

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(1) head tilt to opposite shoulder, (2) head turn downward with chin depressed and eyes upward, and (3) face turn to opposite side

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Acute dystonic reactions

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Anticholinergic medication, including diphenhydramine, is the most appropriate treatment

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copper deficiency

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subacute syndrome of pyramidal tract and dorsal column impairment afterr gastric surgery, or after zinc overdose Alpha-tocopherol transfer protein gene mutation would present typically in childhood with gradual onset

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REM sleep behavior disorder (RBD assoicated with pathology

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synucleinopathies

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which virus can cause a necrotizing myelopathy

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Varicella zoster virus

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Oral hormone contraception and lamotrigine

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(OHC) induces this system and may result in a significant lowering of serum LTG levels. LTG has no effect on OHC metabolism and does not increase the risk of OHC failure.

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Schilder's disease

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a very rare, progressive, degenerative, demyelinating disorder of the central nervous system (CNS) that usually begins in childhood and young adulthood (mostly males between the ages of 7 and 12) larger and larger patches of demyelination

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Marburg Disease

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the fulminant form of multiple sclerosis, is a rare disease that typically kills within a year.

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Charcot-Marie-Tooth disease (CMT) 3 common types

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classic phenotype ;walking on time, and develop weakness or sensory loss during the first two decades CMT1A and men with CMT1X infantile-onset,do not begin walking until they are at least 15 months of age, req assistant of ambulation by 20yrs, CMT1B or CMT1E adult-onset, approximately 40 years of age, largely CMT1B, CMT1X

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Superior semicircular canal dehiscence

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perilymphatic fistula and is characterized by sound, pressure induced vertigo, and oscillopsia.

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Ménière disease,

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due to endolymphatic hydrops

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Mollaret meningitis

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recurrent, self-limiting, aseptic meningitis caused by the herpes simplex virus 2 and may benefit from treatment with acyclovir

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level of evidence from AAN

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class A, established, need 2 class I studies Class B, probable, need 1 class I and 2 class II studies class C, possible, need 1 Class II and 2 Class II studies Class U, inadequated, unapproved

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Metronidazole neuro- toxicity

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sensorimotor polyneuropathy affecting both large and small fibers recovery, 6 to 12 months

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neurogenic claudication

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due to chronic compression of the lumbosacral nerve roots of the cauda equina, within the thecal sac, due to lumbosacral central spinal stenosis resembles vascular claudication. pain is classically relieved by a change in position or flexion of the waist and not simply relieved by rest

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Down syndrome, neuro

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Down syndrome can be associated with atlantoaxial instability

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what neuropathy unique to Sjögren syndrome

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Dorsal root ganglion involvement leading to an ataxic sensory neuronopathy

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what neuropathy is a common neurologic feature of connective tissue diseases

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Trigeminal sensory neuropathy

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on bevacizumab, developed hypertension, a seizure, and cortical blindness.

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reversible posterior leukoencephalopathy

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Allelic loss (loss of heterozygosity) of chromosomes 1p and 19q are molecular signatures of which tumor

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grade III (anaplastic) oligodendrogliomas. increased responsiveness to chemotherapy and increased length of survival compared to grade III astrocytomas and grade IV gliomas

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trigeminal neuralgia (tic douloureux) DD

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vascular loop structural causes such as a meningioma, cerebellopontine angle neoplasm, and aneurysm. Multiple sclerosis stroke

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A late posttraumatic seizure

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defined as a seizure that occurs more than 1 week after a head injury

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progressive supranuclear palsy

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tauopathy, early falls, bradykinesia, and marked axial rigidity Supranuclear gaze palsy occurs with initial impairment of down gaze

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MRI characteristic of Creutzfeldt-Jakob disease (CJD)

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restricted diffusion in the cortical mantle and basal ganglia CSF-neuron-specific enolase and 14-3-3 protein

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FDA advises of MS pregancy

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The FDA advises discontinuation of disease-modifying agents, starting about 1 cycle prior to conception, under the justification of "do no harm". RRMS has not been associated with an increased risk of birth defects or pregnancy related complications. There is no known contraindication to corticosteroids during pregnancy.

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typically presention of cerebral amyloid angiopathy

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elderly persons with spontaneous lobar hemorrhage

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cerebral autosomal dominant arteriopathy, with subcortical infarcts and leukoencephalopathy (CADASIL)

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NOTCH3 gene on chromosome 19.

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heaadache and breast feeding

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amount of most medications excreted in breast milk is 1% to 2% of the maternal dose Aspirin is considered less safe than acetaminophen Triptans and tricyclic antidepressants are of unknown risk to the infant, while narcotics are safe.

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RLS is characterized by..

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1) an urge to move the legs, usually accompanied by uncomfortable or unpleasant leg sensations, 2) symptoms beginning or worsening during rest or inactivity, 3) symptoms occur only, or are worse, in the evening or night compared to the day, 4) symptoms are partially or totally relieved by movement or stretching for at least as long as the activity continues, and 5) symptoms are not solely accounted for as being primary to another condition, such as leg cramps

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POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes)

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serum vascular endothelial growth factor (VEGF) is one of the major diagnostic criteria supporting a diagnosis of POEMS

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Charles Bonnet syndrome

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complex visual hallucinations in the setting of significant visual deprivation

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Paroxysmal hemicrania Tx

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exquisitely sensitive to indomethacin

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described premotor features of idiopathic Parkinson disease

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Anosmia, REM sleep behavior disorder, constipation and depression

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Balint syndrome

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simultagnosia, oculomotor apraxia and optic ataxia

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the North American Antiepileptic Drug (AED) Pregnancy Registry findings

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lamotrigine is associated with the lowest risk of major congenital malformations during exposure in the first trimester compared to other the AEDs given. The approximate relative risks of the other AEDs listed compared to lamotrigine were 1.2 for levetiracetam; 1.5 for carbamazepine; 1.5 for phenytoin; 2.2 for topiramate; and 5.1 for valproic acid.

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acute onset of epidural spinal cord compression Tx

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surgery within 24 hours had significant improvement in their ability to walk and the length of time that they were able to continue to walk.

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Nitrous oxide toxicity

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cause a progressive myelopathy of the posterior and lateral columns that mimics that of vitamin B12 deficiency. Nitrous oxide irreversibly oxidizes the cobalt moiety of methylcobalamin and interferes with conversion of homocysteine to methionine

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Natalizumab-induced PML

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discontinuation of natalizumab and initiation of plasma exchange IRIS (immune reconstitution inflammatory syndrome) treatment for IRIS is high-dose glucocorticoids followed by a slow taper

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Cerebrovascular disease occurs in ??% of sickle cell disease

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25% of sickle cell disease Eighty percent of events occur at or before age 15 chronic transfusion therapy to keep hemoglobin S below 30%

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Hallmarks of Friedreich ataxia

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weakness, ataxia, absent tendon jerks, Babinski sign, and abnormal position sense. Romberg sign is the best indication of loss of position sense. Hammer toes, pes cavus deformities, and scoliosis are frequent findings.

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atlantoaxial instability

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Down syndrome Klippel-Feil ( abnormal fusion), Morquio (mucopolysaccharidosis IV), Larsen syndromes, achondroplasia previous cervical spinal surgery

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exposed to valproic acid in utero

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valproic acid in utero had significantly lower IQ scores than children of the same age who had in utero exposure to to carbamazepine, lamotrigine, and phenytoin

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Lesch-Nyhan syndrome

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dystonia typically prior to 12 months self-injurious behavior, between 2 and 4 years of age hypoxanthine-guanine phosphoribosyltransferase

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Bartonella henselae (catscratch disease) signs on O/E

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enlarged lymph node in the arm axilla or neck

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Niemann-Pick disease type C

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autosomal recessive inheritance hepatosplenomegaly, progressive neurodegeneration; vertical supranuclear (usually downgaze) palsy, ataxia, dystonia, seizures, gelastic cataplexy, and dementia

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Metachromatic leukodystrophy

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autosomal recessive disorder periventricular and deep white matter with relative sparing of the U-fibers (long tract signs alone are common Neurocognitive symptoms can be the presenting

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Pelizaeus- Merzbacher Diseasem MRI

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white matter in the centrum semi-ovale is isodense with the cortical ribbon on T1- weighted imaging

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l-asparaginase

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cause significant hypercoagulability, particularly secondary anti-thrombin III deficiency, which this patient had. Venous sinus thromboses, with infarctions in a venous distribution, are the result.

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Joubert syndrome

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hypoplasia of the cerebellar vermis most common features of this syndrome include hyperpnea, hypotonia, oculomotor apraxia, ataxia, and mental retardation

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molar tooth sign

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thickening and horizontalization of the superior cerebellar peduncle and a deep interpeduncular fossa, Joubert syndrome Dekaban-Arima syndrome, Senior-Loken syndrome, COACH (cerebellar vermis hypoplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis).

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subdural hemorrhages in the absence of inflicted trauma in infants

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Menkes disease, type 1 osteogenesis imperfecta, and glutaric aciduria type 1

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bacterial meningitis long term complication

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hearing loss found in as many as 15% to 30% of cases in various series, permanent motor deficits or learning disabilities are found in in 5% to 15%; epilepsy or hydrocephalus in less than 5% of cases.

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Mutations of filamin A linked to ??

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bilateral periventricular heterotopias, enlarged cistern magna thinning or agenesis of the corpus callosum. aortic aneurysm

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diagnosis of spasmus nutans

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dysconjugate, highly variable nystagmus, head nodding head tilt, without ophthalmologic abnormalities, and with normal neuroimaging

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Congenital nystagmus

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conjugate and suppressed by convergence (in contrast to spasmus nutans,which is typically increased by convergence)

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Opsoclonus

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chaotic, conjugate saccades, seen classically in the paraneoplastic syndrome associated with neuroblastoma.

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evidence of treatment of Bell palsy

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There is Class 1 evidence supporting treatment with steroids to improve recovery. There is weak evidence for antiviral treatment. There is no benefit to combination therapy

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sensitivity

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number of true positives over true positives plus false negatives

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Specificity

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is the number of true negatives over true negatives plus false positives

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Myoclonic epilepsy with ragged red fibres.

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mitochondrial DNA (mtDNA) gene MT-TK encoding tRNA lysine (tRNALys) is the gene most commonly associated with MERRF (found in 80% of patients).

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Neurogenic pattern

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Denervation atrophy with small angulated fibers - grouped atrophy Fiber-type grouping if there is reinnervation (loss of the checker pattern) Acute denervation causes small angulated fibers and groups of atrophied fibers target cell-denervation

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Myopathic pattern

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Necrosis, myophagocytosis Fiber splitting, Fiber size variation (hypertrophy and splitting) Central nuclei, Basophilia (degeneration), Non-grouped atrophy Fibrosis / lipid deposition

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Retinitis Pigmentosa

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NARP, MNGIE, Refsum's, Abetalipoproteinemia, KS

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Tay-Sach

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Hexosamindase A

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Sandhoff

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Hexosamindase A & B

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Fragile X Premutation Syndrome MRI

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MRI show hyperintensity in middle cerebellar peduncles

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Fragile X Premutation Syndrome

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50(55)-200: Premuation FX tremor/ataxia syndrome (FXTAS)

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Maple Syrup Urine Disease MRI

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characteristic edema affecting deep cerebellar WM, dorsal brainstem, cerebral peduncles and internal capsule

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Maple Syrup Urine Disease-Tiral of thiamine

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- there is a thiamine responsive variant

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Anticipation

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Expanded repeats usually enlarge when passed to successive generations, producing more severe disease at earlier age of onset

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imprinted genes

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imprinted genes are only active from one parental allele

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Harding's Essential Diagnostic Criteria for Friedreich Ataxia

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When symptoms have been present for less than 5 years: (1) Onset of symptoms before 25 years of age (2) Progressive ataxia of all limbs and gait (3) Absent knee and ankle tendon reflexes (4) Babinski response present (5) Motor nerve conduction velocity greater than 40 m per second in the upper extremity with small or absent sensory responses

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DNA repair defect

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• Ataxia telangiectasia • Ataxia with oculomotor apraxia 1 (AOA 1) • Ataxia with oculomotor apraxia 2 ( AOA 2) • Ataxia-telangiectasia -like disorder (ATLD) • Spinocerebellar ataxia with axonal neuropathy (SCAN 1) • Xeroderma pigementosum (XP)

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Ataxia with vitamin E deficiency

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Treatment: Vitamin E supplementation

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Refsum's disease

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Dietary reduction of phytanic acid

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Cerebrotendinous xanthomatosis

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Cholic acid and chenodeoxycholic acid: compensate for the deficient bile acids in the intrahepatic pool and decrease cholestanol synthesis and levels o Simvastatin or lovastatin: decrease cholesterol synthesis and cholesterol levels

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Episodic ataxia type 1 & 2

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Acetazolamide (500-700 mg daily) often completely eliminates the attacks (better and more predictable response in type 2 than type 1)

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Abetalipoproteinemia

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Restriction of dietary fat and supplementation of vitamins ADEK (often massive doses)

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Wilson Disease

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D-penicillamine

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FRAXA

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X-linked FMR1, on the X-chromosome CGG

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FXTAS (Fragile Xassociated tremor/ ataxia syndrome)

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X-linked FMR1, on the X-chromosome CGG

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FRAXE (Fragile XE mental retardation)

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X-linked AFF2 or FMR2, on the X-chromosome GCC

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FRDA (Friedreich's ataxia)

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AR FXN or X25, (frataxin) GAA

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DM (Myotonic dystrophy)

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AD, DMPK CTG

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SCA8 (Spinocerebellar ataxia Type 8)

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AD OSCA or SCA8 CTG

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SCA12 (Spinocerebellar ataxia Type 12)

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AD PPP2R2B or SCA12 CAG

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Metachromatic LD

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arylsulfatase A

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Fabry

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-galactosidase A

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Tangiers

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-lipoproteinemia (ABC transporter deficiency) (ABCA1 Gene)

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Gaucher

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β-glucocerebrosidase

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Niemann-Pick

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Sphingomyelin phosphodiesterase 1

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Canavan

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Aspartoacylase - U fiber involvement

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Alexander

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GFAP (frontotemporal)

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Adrenoleukodystrophy

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ABCD1 (VLCFA accumulation, posterior)

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Pelizaeus-Merzbacher

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Proteolipid protein 1 (PLP1)

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Vanishing White Matter

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eIF2B5

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What is the most prominent efferent pathway from the amygdaloid

answer

stria terminalis. Most of the fibers arise from the corticomedial part of the nuclear complex. They arch along the medial border of the caudate near its junction with the thalamus. The largest termination is the nucleus of the stria terminalis. Other terminations include the anterior hypothalamic nuclei and the medial preoptic area.

question

where is the frontal eye field in humans

answer

The frontal eye field in humans is located in the caudal part of the middle frontal gyrus. Efferents from this region project to the paramedian pontine reticular formation (PPRF).

question

medial medullary syndrome

answer

infarction in the anterior spinal artery distribution at the level of the medulla. ipsilateral CNXII paralysis (fascicle of CNXII), contralateral hemiparesis (pyramid), and contralateral loss of position and vibratory sensation (medial lemniscus). A bilateral lesion in this vascular territory will result in quadriparesis, bilateral loss of proprioception and vibration, and complete paralysis of the tongue

question

Innervation of the detrusor muscle

answer

parasympathetic (S2-S4) fibers. The internal urethral sphincter at the neck of the bladder receives its innervation via sympathetic fibers. Somatic fibers via the pudendal nerve innervate the external sphincter.

question

baroreceptor reflex, which nerve

answer

baroreceptor reflex helps to regulate blood pressure by detecting changes via baroreceptors mostly located in the carotid sinus and aortic arch. Information from the carotid sinus travels via the glossopharyngeal nerve while that from the aortic arch is carried by the vagus.

question

thalamus Blood Supply

answer

The thalamoperforating branches of the posterior cerebral arteries perfuse the medial and anterior regions of the thalamus. The thalamogeniculate branches of the posterior cerebral arteries perfuse the pulvinar and lateral nuclei. The inferior thalamic arteries arise from the posterior communicating arteries and perfuse the inferior portions of the thalamus. The medial posterior choroidal artery supplies the superior and medial portions of the thalamus.

question

the order of Myelination

answer

Myelination begins in the peripheral nervous system. The motor roots myelinate before the sensory roots. Before birth, myelin appears in the central nervous system in components of major sensory systems and components of major motor systems. Myelination in the commissures continues to occur well after birth. Subcortical U fibers myelinate around 8 months of life, and the myelin pattern is essentially mature by 18 months.

question

cervical central spinal cord syndrome

answer

lower motor findings in his hands, upper motor neuron hyperreflexia in his legs and a classical sensory disturbance in which secondary order neurons subserving pain and thermal sense are interrupted as they decussate in the central spinal cord. The primary order neuronal axons in the dorsal columns subserving fine touch and proprioception are spared. The central spinal cord syndrome most commonly results from syringomyelia or intrinsic tumors.

question

Asomatognosia

answer

patient denies ownership of a limb contralateral to a lesion of the supramarginal gyrus of the parietal lobe (usually nondominant).

question

zone of Lissauer (dorsolateral fasciculus)

answer

The zone of Lissauer (dorsolateral fasciculus) is adjacent to lamina I in the spinal cord. It is composed of fibers that originated in the dorsal roots

question

connection between GPi and thalamus

answer

The internal segment of the globus pallidus sends outputs to the thalamus through the ansa lenticularis and the lenticular fasciculus

question

"one-and-a-half" syndrome

answer

ipsilateral eye has no horizontal movements and the contralateral eye is only able to abduct. The clinical findings are a combination of a left intranuclear ophthalmoplegia (which prevents the left eye from adducting on rightward gaze with end-gaze nystagmus of the abducting right eye) and a left abducens nuclear palsy (which produces an ipsilateral gaze palsy preventing the patient from looking left). The only horizontal eye movement still possible, then, is abduction of the right eye on rightward gaze. This lesion must involve the left medial longitudinal fasciculus and left abducens nucleus.

question

masseter

answer

trigeminal nerve

question

levator palpebrae

answer

oculomotor nerve

question

genioglossus

answer

hypoglossal nerve

question

stylopharyngeus

answer

glossopharyngeal nerve

question

dorsal nucleus of Clarke

answer

extends from C8 to L2 or L3. It receives proprioceptive input from axons traveling in the dorsal columns and gives rise to the dorsal spinocerebellar tract. Input from the upper extremities synapse in the external cuneate nucleus which gives rise to the cuneocerebellar tract. Both the dorsal spinocerebellar tract and the cuneocerebellar tract ascend to the cerebellum by way of the ipsilateral cerebellar peduncle and convey unconscious proprioception to that structure.

question

where rubrospinal tract runs

answer

The rubrospinal tract runs lateral to the lateral corticospinal tract in the dorsal funiculus of the spinal cord and is the most lateral of the five descending pathways listed.

question

straight (rectus) sinus drainage

answer

straight (rectus) sinus receives venous blood directly from the inferior sagittal sinus. The basal vein of Rosenthal drains into the great cerebral vein (vein of Galen), which subsequently drains into the straight sinus

question

nucleus accumbens involved in which neurological/psychiatric condition

answer

The nucleus accumbens, a component of the basal ganglia, receives extensive input from the limbic system and the orbitofrontal cortex. It is involved in anticipating rewards and is therefore implicated in substance abuse and addiction.

question

Neuro embrology, what is BASAL AND ALAR PLATES

answer

representing the motor and sensory areas, respectively. THE MEDULLA, like opening a textbook, the roof plate stretches and becomes a single layer of cells covering an enlarged central cavity called the fourth ventricle. In myelencephalon- the hypoglossal (XII) nucleus. In the metencephalon and mesencephalon, -the abducens (VI), trochlear (IV), and oculomotor (III) cranial nerves. A lateral general visceral efferent group contains the neurons whose axons grow out as preganglionic fibers. In the myelencephalon- dorsal nucleus of the vagus (X) and the inferior salivatory nucleus cranial nerve IX (glossopharyngeal)

question

The major function of amygdala, caudate, nucleus accumbens and hippocampus

answer

The amygdala lies in the anterior pole of the temporal lobe just deep to the uncus and is an important structure in processing the emotional significance of stimuli, including pain. The caudate is the principal basal ganglia nucleus involved in processing oculomotor and prefrontal information. The nucleus accumbens is involved in anticipating reward and habit formation rather than the emotional component of pain. The hippocampus is part of the limbic system and is an essential component of memory formation. The pulvinar is an association nucleus in the thalamus involved in visual processing

question

orbitofrontal syndrome

answer

The orbitofrontal syndrome consists of socially inappropriate behaviors as well as poor impulse control with disinhibition.

question

lateral convexity syndrome

answer

lateral convexity syndrome is associated with dysexecutive symptoms, such as planning, abstract thinking, flexibility and behavioural control. Symptoms of DES fall into three broad categories: cognitive, emotional and behavioural.

question

mesial frontal syndrome

answer

The mesial frontal syndrome is associated with amotivation. The anterior cingulate is the origin of the anterior cingulate-subcortical circuit. Damage to these circuits causes apathy or abulia (a severe form of apathy). Acute bilateral lesions in the medial frontal area can cause akinetic mutism, in which the individual is awake and has self awareness but does not initiate behaviors

question

which spinal segment is most vulnerable to ischemic insult

answer

The upper thoracic (T1-T4) segments are in the vascular boundary zone between ascending and descending sources of the blood supply to the spinal cord and are therefore the areas of the cord most vulnerable to ischemic insult. The largest medullary artery, the great anterior artery of Adamkiewicz, arises between T9 and L2 and supplies the lumbar enlargement

question

symptoms of lesion of mediodorsal nucleus of the thalamus

answer

The dorsomedial (or mediodorsal) nucleus of the thalamus provides innervation to the whole prefrontal cortex, which consists of a paralimbic orbitofrontal region involved in control of affect and behavior, and an associative dorsolateral region involved in executive functions. Lesions in this area may result in frontal systems impairments, including disinhibition and poor executive function. Isolated lesions of the anterior nucleus may result in amnesia but do not result in disinhibition and poor executive function.

question

anterior choroidal artery

answer

The anterior choroidal artery usually arises from the internal carotid artery and feeds portions of the globus pallidus, putamen, thalamus, and the posterior limb of the internal capsule

question

what is dentate gyrus

answer

In the hippocampus, granule cells of the dentate gyrus form distinctive unmyelinated axons that project along the mossy fiber pathway to the Cornu Ammonis area 3,(CA3) .Granule cell synapses tend to be glutamatergic.

question

Pathway of Primary nociceptive afferents

answer

Primary nociceptive afferents, from the dorsal root ganglion, traverse the zone of Lissauer prior to synapsing in the posteromarginal nucleus (lamina I). Cells in lamina I respond specifically to noxious and thermal stimuli and contribute fibers to the contralateral spinothalamic tract.

question

Weber's syndrome

answer

A lesion at the base of the midbrain (cerebral peduncle) will produce an ipsilateral third nerve palsy and contralateral hemiparesis (Weber's syndrome).

question

muscle supplied by CN V

answer

The trigeminal nerve supplies the muscles of mastication-the temporalis, masseter, medial and lateral pterygoids, mylohyoid,anterior belly of the digastric, tensor veli palatini, and tensor tympani muscles.

question

muscle supplied by CN VII

answer

The facial nerve supplies the frontalis, corrugator, orbicularis oculi, nasalis, buccinator, orbicularis oris, mentalis, and platysmas.

question

ocular tilt reaction.

answer

A peripheral or brainstem lesion that affects the otolithic (gravitational) pathways can result in a skew deviation, ocular torsion, and a head tilt. When all three are present, it is called the ocular tilt reaction

question

eye movements

answer

Visual fixation Holds the image of a stationary object on the fovea Vestibulo-ocular reflex Holds images of the seen world steady on the retina during brief head rotations Optokinetic Holds images of the seen world steady on the retina during sustained and low-frequency head rotations Smooth pursuit Holds the image of a moving target close to the fovea Nystagmus quick phases Reset the eyes during prolonged rotation; direct gaze toward the oncoming visual scene Saccades Bring images of eccentrically located objects of interest onto the fovea Vergence Moves the eyes in opposite directions so that images of a single object are placed simultaneously on both foveas during gaze shifts in depth

question

suprachiasmatic nucleus

answer

The suprachiasmatic nucleus inhibits the paraventricular nucleus of the hypothalamus, which decreases paraventricular driving of the superior cervical ganglion, thereby decreasing sympathetic drive onto the pineal gland

question

end organs involved in perception of vibration

answer

Pacinian corpuscles found in subcutaneous connective tissue and in periosteum.

question

insular cortex receive what input

answer

The insular cortex receives visceral nociceptive input via the ventromedial posterior (VMP) thalamic nucleus.

question

Mesolimbic and mesocortical projections

answer

Mesolimbic and mesocortical projections arise from the ventral tegmental area of the midbrain. The fibers are dopaminergic, and travel to cerebral cortex and limbic structures, such as the amygdala.

question

fornix

answer

The fornix is the principal output pathway of the hippocampus is divided into two fiber bundles as it passes the anterior commissure (pre- and postcommissural fibers). The postcommissural fibers innervate the mammillary bodies while the precommissural fibers innervate the septal area and anterior hypothalamus

question

common gait disorders

answer

spastic gait-stiff legged, circumduction, scissoring ataxia gait-wide based, unsteady, staggering side to side, falling towards side of worse pathology, drunk walk--toxin or cerebellar vermis vertiginous gait-like ataxia, vestibular nuclei or semicircular canals frontal gait--slow, shuffling, narrow or wide based, magnetic, unsteady, gait apraxia,--- frontal lobe/subcortical WM Parkinsonion --substantia nigra dyskinetic-subthalamic nucleus Tabetic- posterior columns or sensory nerver-high-stepping, foot-flapping gait, diff in dark paretic-peripheral N antalgic-pain orthopedic gait functional

question

Which of the sensory pathways projects outside the thalamus

answer

olfactory pathway.

question

What are the 2nd order neurons of the olfactory pathways?

answer

mitral and tufted cells.

question

Where is the primary olfactory cortex located?

answer

temporal lobe.

question

The axons of _______ cells in the retina collectively form the optic disc.

answer

ganglion cells of retina

question

The optic tract extends from the dorsolateral aspect of the _______ to the ________.

answer

optic chiasma lateral geniculate body

question

Thalamic nucleus that is a relay station for all axons of retinal ganglion cells subserving vision:

answer

lateral geniculate body (L for light)

question

Lateral geniculate nucleus is laminated into structure containing how many layers?

answer

question

Cochlear nerve extends from ______.

answer

organ of Corti

question

The spiral ganglion is related to:

answer

auditory pathway.

question

The 3rd order neurons of the auditory pathway which end in the medial geniculate body, originate from:

answer

inferior colliculus.

question

The ascending pathway used for propioception of the legs:

answer

dorsal spinocerebellar tract.

question

Vibration sensation from forelimbs and hindlimbs is transmitted via the _______ and the _______.

answer

dorsal column and spinocervical thalamic tract.

question

Proprioception sensation from forelimbs project through:

answer

dorsal columns

question

Proprioception sensation from hindlimbs project through:

answer

gracile tract upto the level of dorsal nucleus of Clarke and from there they leave the gracile tract and synapse with the dorsal nucleus of Clark and ascend via the dorsal spinocerebellar tract.

question

Long thoracic nerve (C5, C6, C) innervates

answer

serratus anterior muscle

question

Suprascapular nerve innervates

answer

supraspinatus and infraspinatus muscles

question

Dorsal scapular nerve innervates

answer

rhomboids and levator scapulae muscles.

question

Axillary nerve arises from ______ cord.

answer

posterior

question

Root innervation of axillary nerve

answer

C5, C6.

question

Axillary nerve innervates

answer

deltoid and teres minor muscles

question

Lateral brachial cutaneous nerve is a branch of _____ nerve, and supplies the skin over the outers surface of the arm, mainly _____ region.

answer

deltoid

question

The first _____ degrees of arm abduction from trunk is performed by supraspinatus muscle

answer

question

Musculocutaneous nerve arises from _____ cord.

answer

lateral

question

Nerve root innervation of musculocutaneous nerve

answer

C5, C6, C7

question

What are does the musculocutaneous nerve innervate?

answer

BBC. Biceps, brachialis, and coracobrachialis.

question

Lateral cutaneous nerve of the forearm is a continuation of _______ nerve.

answer

musculocutaneous.

question

Muscle that supinates forearm, especially if the elbow is flexed at 90 degrees.

answer

biceps.

question

Lesion of which nerve results in weakness of elbow flexion against resistance in a fully supinated hand, possible weakness in arm elevation, arm pain, and radial forearm paresthesia.

answer

musculocutaneous.

question

Compression site of the radial nerve.

answer

spiral groove in the posterior aspect of the humerus.

question

Radial nerve arises fromm _____ cord.

answer

posterior

question

To differentiate between a lesion of upper brachial plexus and one of the lateral cord, the most useful muscle to test is the:

answer

teres minor

question

To differentiate between a lesion of lower trunk of brachial plexus and one of the medial cord, the most useful muscle to test is the:

answer

extensor indicis proprius

question

To differentiate between a lesion of middle trunk of brachial plexus and one of the posterior cord, the most useful muscle to test is the:

answer

pronator teres

question

In the sciatic nerve, before it divides, the medial trunk of the nerve comprises fibers of the _____ and of the _____ nerves.

answer

tibial and peroneal

question

The only thigh muscle innervated by the lateral peroneal division of sciatic nerve.

answer

short head of biceps

question

Adductor magnus is supplied by two nerves.

answer

obturator and sciatic nerves.

question

Hamstrings are innervated by ______ nerve.

answer

sciatic.

question

Sartorius muscle is innervated by ______ nerve.

answer

femoral

question

Carpal tunnel contains how many structures?

answer

question

What are the structures in the carpal tunnel?

answer

4 tendons of flexor digitorum superficialis 4 tendons of flexor digitorum profundus median nerve 1 tendon of flexor pollicis longus

question

What bones make up the Guyon's canal?

answer

Hamate and pisiform bones.

question

What is the Guyon's canal related to?

answer

compression site of the ulnar nerve at wrist.

question

Genitofemoral nerve divides near the _______ into the external genital branch and the medial femoral branch

answer

inguinal ligament

question

Name the nerve innervating the cremaster muscle.

answer

external genital branch of the genitofemoral nerve.

question

Name the nerve innervating the skin over the femoral triangle.

answer

medial femoral branch of the genitofemoral nerve.

question

Superior gluteal nerve innervates _____ and _____ muscles.

answer

gluteus medius and gluteus minimis.

question

Gluteus maximus is innervated by

answer

inferior gluteal nerve.

question

The root fibers of the _____ nerve course in the medulla oblangata lateral to the medial leminiscus and emerge on the ventral surface of the medulla between the _____ and _____.

answer

hypoglossal nerve. pyramid and inferior olive

question

The gaze-holding neural integrator for vertical gaze is located in _________.

answer

interstitial nucleus of Cajal.

question

Vertical and torsional saccade is controlled by

answer

cortical pathways descending to the rostral interstitial nucleus of the medial longitudinal fasiculus at the junction between the midbrain and thalamus.

question

Horizontar gaze center

answer

PPRF and abducens nucleus

question

The gaze-holding neural integrator for horizontal gaze

answer

horizontal gaze cener, medial vestibular nucleus, and nucleus prepositus hypoglossi.

question

Which of the cranial nerves exits the brainstem from its dorsal aspect?

answer

Trochlear nerve

question

The fibers of _____ nucleus cross to the other side of the brainstem just prior to exiting the pons.

answer

trochlear

question

Superior oblique muscle of eye is innervated by

answer

trochlear nerve.

question

Perihypoglossal nuclei are involved in

answer

extraocular movement.

question

Ventral motor nucleus of vagus

answer

nucleus ambiguus

question

Nucleus ambiguus

answer

SVE to branchiomeric muscles of pharynx and larynx. Efferent fibers to CN 9 and CN 11.

question

Taste sensation and pain from the epiglottis and posterior third of tongue is received by

answer

nucleus tractus solitarius (rostral and lateral zone)

question

Nuclei involved in the cardiovascular control

answer

nucleus tractus solitarius, dorsal motor nucleus of vagus, caudal and ventrolateral medulla, which receive projections from sensorimotor cortex.

question

Taste sensation is conveyed by

answer

facial, glossopharyngeal, and vagus nerves.

question

Taste sensation from anterior two-thirds of tongue

answer

facial nerve

question

Taste sensation from posterior third of tongue

answer

glossopharyngeal nerve

question

Taste sensation from epiglottis

answer

vagus nerve

question

Anosognosia for hemiplegia is seen in lesions of

answer

nondominant parietal lobe. pontine reticular nucleus in the tegmentum and corticospinal fibers in the basis pontis.

question

Pituitary adenomas where

answer

arise from within the sella

question

Chordomas where

answer

heterogenous tumors consisting of notochord remnants which most often occur in the clivus and sacrococcygeal regions.

question

Craniopharyngiomas where

answer

centered in the suprasellar (rather than intrasellar) compartment and contain cysts and calcification

question

Giant cell (temporal) arteritis

answer

granulomatous vasculitis affecting chiefly the extracranial arteries of the head (superficial temporal, ophthalmic, carotid and vertebral); usually sparing intracranial vessels. It may also affect the aorta and its branches

question

Hypertension CNS hemorrhage

answer

deep grey matter pons and rarely cerebellum

question

Neurosarcoid

answer

basal cranial meninges; the spinal meninges cranial neuropathies (50-75%). The facial nerve is the most frequent cranial nerve involved Pituitary stalk diencephalon Aseptic meningitis, nodular dural masses, and hydrocephalus

question

Kufs disease

answer

adult form of neuronal ceroid lipofuscinosis

question

typical microscopic findings of hemangioblastoma

answer

vacuolated stromal cells and variably sized vascular structures

question

Dandy-Walker malformation

answer

vermis may be either absent entirely or hypoplastic enlarged posterior fossa brainstem microscopic developmental abnormalities

question

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

answer

Notch 3 gene on chromosome 19.

question

CADASIL

answer

HTRA1 gene

question

neuromyelitis optica Pathology

answer

The presence of necrosis in spinal cord virtually defines the entity

question

monoamine oxidase (MAO) and cheese

answer

prevents dietary pressor amines, typically tyramine, from entering the tissues, a marked hypertensive response (the "cheese reaction") can occur

question

Selegiline

answer

(a preferential MAO-B inhibitor) has no MAO-A inhibition at 10 mg/d or less, but at 20 mg/D or above it has

question

Fingolimod

answer

sphingosine-1 phosphate-receptor reduces lymphocyte recirculation from the lymph nodes resulting in functional immunosuppression.

question

cardiac effects of fingolimod

answer

maximal after the first dose but persist for about 14 days

question

tramadol and Sz

answer

Daily doses of 400 mg or more of tramadol may provoke seizure activity

question

Oxcarbazepine and Na

answer

produce hyponatremia due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH)

question

Fabry's disease

answer

X-linked defect in alpha-galactosidase painful peripheral neuropathy with autonomic manifestations, a typical rash

question

Topiramate

answer

increases the risk for calcium kidney stones easier for calcium to bind with phosphate (forming calcium phosphate stones Common calcium kidney stones is calcium oxalate stones There is no contraindication to administer topiramate to a patient with history of calcium oxalate stones

question

Causes of a sensory neuronopathy

answer

Sjögren syndrome, paraneoplastic syndrome usually with an anti-HU antibody, pyridoxine intoxication (at least 200 mg per day), a variant of Guillain-Bare syndrome, and with platinum-based chemotherapies

question

pathophysiology of migraine

answer

1,vasoactive peptides (calcitonin gene-related peptide, neurokinin A, substance P) from trigeminal afferents supplying dural blood vessels. 2,vasodilation and sterile inflammation in dural vessels, leading to activation of first-order trigeminal afferents 3, trigeminothalamic and thalamocortical neurons become activated

question

Anti-Yo

answer

the anti-Purkinje cell antibody and reacts only with Purkinje cells. anti-Yo have a gynecological cancer, ovarian or breast 90% of the time

question

analgesic effects of pregabalin

answer

decrease in central sensitization and nociceptive transmission to the action on the alpha-2-delta subunit of the calcium channel

question

manganese

answer

disposal of manganese in the body is biliary excretion characterized by parkinsonism and dystonia, which do not respond to levodopa. T1-weighted MRI shows hyperintensity in the globus pallidus, striatum, and midbrain. The primary site of damage is the globus pallidus

question

REM sleep behavior disorder (RBD) tx

answer

Clonazepam is efficacious and well tolerated by the majority of patients. melatonin may be a good alternative to clonazepam

question

Meds avoided in patients with RBD.

answer

Monoamine oxidase inhibitors, tricyclic antidepressants, serotonergic synaptic reuptake inhibitors noradrenergic antagonists

question

clozapine

answer

Up to 1% percent of patients for several months experience bone marrow suppression

question

Dalfampridine (4-AP)

answer

potassium-channel blocker that improves nerve conduction along centrally demyelinated axons 0.2% risk of seizure sassociated with toxic blood levels

question

risk of Stevens-Johnson syndrome

answer

The HLA-B*1502 allele increases the risk of Stevens-Johnson syndrome after carbamazepine exposure by 10 times

question

Teriflunomide (TF)--relapsing-remitting multiple sclerosis

answer

reversibly inhibits dihydroorotate dehydrogenase, a key mitochondrial enzyme involved in new pyrimidine synthesis for DNA replication

question

pedunculopontine nucleus (PPN)

answer

inputs from the globus pallidus and subthalamic nucleus, and projects to the substantia nigra, thalamus, and medullary reticulospinal neurons

question

Tardive dyskinesia

answer

dopaminergic D2 receptor blockers classical highpotency neuroleptics, but also antiemetic agents such as prochlorperazine or metoclopramide.

question

Blockade of histamine (H-1 and H-2) receptors can cause what clinical signs

answer

sedation, drowsiness; potentiation of central depressant drugs; weight gain.

question

Blockade of muscarinic acetylcholine receptors can cause what clinical signs

answer

dry mouth, blurred vision, sinus tachycardia, constipation, urinary retention, memory impairment

question

Blockade of norepinephrine uptake can cause what clinical signs

answer

tremors, jitteriness, tachycardia, and diaphoresis, erectile and ejaculatory dysfunction

question

Blockade of serotonin uptake can cause what clinical signs

answer

sexual dysfunction, nausea, vomiting, diarrhea, anorexia, increase or decrease in anxiety (dose-dependent), asthenia (tiredness), insomnia, and extrapyramidal side effects.

question

Blockade of serotonin-2 (5-HT2) receptors

answer

ejaculatory dysfunction, hypotension, alleviation of migraine headaches, decrease in anxiety, and decrease motor restlessness

question

Blockade of a1-adrenergic receptor can cause what clinical signs

answer

postural hypotension, dizziness which predisposes to falls possibly resulting in broken bones and subdural hematomas

question

Blockade of a2-adrenergic receptors can cause what clinical signs

answer

priapism

question

drugs of choice of of treatment of severe hypertension in neurological catastrophes

answer

labetalol, esmolol, and enalapril

question

key role of CoQ-10

answer

an electron shuttle between complexes I or II and complex III in the respiratory chain

question

Primary CoQ-10 deficiency

answer

myopathy or progressive cerebellar degeneration

question

Charcot-Marie-Tooth disease 1B

answer

myelin protein zero

question

Charcot-Marie-Tooth disease type 1A

answer

Point mutations of the peripheral myelin protein-22 gene in chromosome 17p

question

Tramadol,

answer

centrally acting analgesic structurally related to codeine and morphine, consists of two enantiomers

question

Lamotrigine

answer

metabolized in the liver predominantly by glucuronic acid conjugation

question

Carbamazepine, phenytoin, and phenobarbital induce glucuronic acid conjugation of lamotrigine resulting in a ??% reduction of the lamotrigine level

answer

40%

question

Valproic acid inhibits glucuronic acid conjugation of lamotrigine ____ the lamotrigine level

answer

doubling

question

mexiletine

answer

reduce the associated hyperexcitability of the sarcolemma by interfering with sodium channels located on it.

question

narcolepsy is associated with human leucocyte antigen (HLA)??

answer

Over 90% of patients with narcolepsycataplexy carry HLA-DQB1*0602 probably have an autoimmune disorder

question

VPA side effect mechanism

answer

increases levels of gamma-aminobutyric acid and prolongs the recovery of inactivated sodium channels---cause CNS depressant impairments in fatty-acid metabolism and disrupt the urea cycle, leading to hyperammonemia fatty-acid metabolism, impairs beta-oxidation (a mitochondrial process), and disrupts the urea cycle

question

Midodrine

answer

treatment of orthostatic hypotension unresponsive to fludrocortisone

question

Codeine,

answer

metabolized by glucuronidation

question

Paralytic shell-fish poison (saxitoxin)

answer

resembles tetrodotoxin in its ability to reversibly bind to membrane voltage-gated sodium channels

question

Fosphenytoin

answer

phosphate ester prodrug of phenytoin Advantages: convenient and rapid intravenous administration, availability IM, d low potential for adverse local reactions at injection sites. Drawbacks: transient paraesthesias and pruritus at rapid infusion rates, and cost. highly bound (93-98%) to plasma proteins half-life for conversion of fosphenytoin to phenytoin ranges from 7-15 minutes rapid achievement of effective concentrations

question

intramuscular administration Fosphenytoin

answer

therapeutic phenytoin plasma concentrations are observed within 30 minutes and maximum plasma concentrations occur at approximately 30 minutes

question

Single fiber EMG (SFEMG)

answer

most sensitive diagnostic test for myasthenia gravis increased jitter in more than 90% of cases Slow repetititive stimulation of a distal muscle is approximately 50% sensitive Repetitive stimulation of a proximal muscle has a higher yield (approximately 75%).

question

Repetitive stimulation >10Hz

answer

Rapid repetitive stimulation (more than 10 hz) is used in presynaptic disorders of neuromuscular transmission

question

Somatosensory evoked potentials

answer

record activity in the dorsal column/medial lemniscal sensory pathways

question

Andersen-Tawil syndrome

answer

is caused by a potassium channel mutation and includes attacks of weakness, cardiac arrhythmias and dysmorphic features

question

Hypokalemic periodic paralysis

answer

is caused by a calcium channel mutation, does not demonstrate myotonic and is characterized by attacks of weakness

question

14 and 6 phantom spikes

answer

bilaterally synchronous and diffuse fashion. The bursts are brief and last 1 to 2 seconds. At times the 14 Hz or, more commonly, the 6 Hz pattern may be seen in isolation. normal variant but can easily be confused with epileptiform discharges.

question

Hypsarrhythmia

answer

high-voltage and irregular slow waves with multiple sharp and spike discharges

question

Subacute sclerosing panencephalitis -EEG

answer

generalized periodic long-interval diffuse discharges

question

Spongiform encephalopathies-EEG

answer

PEDs, but intervals are typically shorter than those of SSPE.

question

HSV-EEG

answer

lateralized periodic epileptiform discharges

question

Tay Sachs disease-EEG

answer

slow background with or without multifocal epileptiform transients

question

Alzheimer's disease-EEG

answer

normal EEGs or sometimes having excessive theta activities

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facial nerve compound muscle action potential (CMAP)

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lower the CMAP amplitude, the more axonal degeneration and the poorer the prognosis

question

hereditary neuropathy with liability to pressure palsies (HNPP).

answer

This disorder is caused by a deletion or point mutation in the PMP22 gene

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question

Presenilin 2 (Chromosome)

answer

Chromosome 1

question

Paternal Transmission of Chromosome 15

answer

Prader-Willi

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Voltage gated Sodium Channel Myotonia of eyelids, face, and hands

answer

Paramyotonia Congenita

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Motor Regression, cherry red spot, optic atrophy to blindness, severe retardation, seizures Decreased hexosaminidase A Chromosome 5/15

answer

Tay-Sachs (GM2 Gangliosidosis)

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Recurrent infections with B cell neoplasia Chromosome 11 affected

answer

Ataxia-Teleangectasia

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Birefringent Ca Oxalate and oxalic acid in urine

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Ethylene Glycol toxicity

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Pseudoperiodic high voltage sharp and slow waves

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EEG findings with CJD

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Reduces the function of B12

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NO Toxicty

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High-voltage polyphasic sharp and slow wave complexes of 0.5 to 2 second duration that recur every 4-15 seconds

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EEG fingins with SSPE

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Mechanism of Tetrodotoxin

answer

Blocks Sodium Channels

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Malabsorption, peripheral neurolopathy, pigmentary retinopathy Treated with Vitamin E

answer

Bassen-Kornzweig

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Palatal Neuropathy 10% Develop defuse sensorimotor polyneuropathy 8-12 weeks after infection Increase protein in CSF

answer

Diptheira

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Dopamine refractory parkinsonism and dystonia, associated with liver disease Globus Pallidus and midbrain increased on T1

answer

Manganese Intoxication

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Astrocytic Tumor (Chromosome/gene)

answer

Chromosome 17 P53 gene

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Mechanism of Lathyrus toxicity (alpha-amino-beta-oxalaminoproprionic acid)

answer

Glutamate Toxicity

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Retinoblastoma (Chromosome/gene)

answer

Chromosome 13 pRB gene

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Latrotoxin (Source/mechanism)

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Black Widow Venom Releases acetylcholine

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Dopamine Responsive Dystonia (Chromosome)

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Chromosome 14

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Kugelberg-Welander (SMA type/Chromosome)

answer

SMA Type III Chromosome 5

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Charcot Marie Tooth Disease 1 (Chromosome/gene) Hereditory motor and sensory neuropathy 1 (Chromosome/gene)

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Chromosome 17/PMP-22 Chromosome 1/ P0

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Familial Hemiplegic Migraine (Channel/Chromosome)

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P/Q Calcium Channel Chromosome 19

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Pyramidal signs, peripheral neuropathy Autosomal dominant 6 CAG trinucleotide repeat

answer

Spinocerebellar Degeneration Type I

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Associated wiht Meningioma, schwanoma, glioma Chromosome 22

answer

NF 2

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Hemangioblastoma of CNS (cerebellum and retina) Associated with pheochromocytoma and renal cancer

answer

Von Hipple-Lindau

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"Rod bodies" Static muscle weakness, congenital hypotonia Autosomal dominate when on Chromosome 1 (most common) Autosomal Recessive when on Chromosome 2

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Nemaline Myopathy

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Infantile Onset SMA Type 1 SMN Gene Chromosome 5

answer

Werdnig-Hoffman

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Progressive external opthalmoplegia pathology

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Mitochondrial Ragged Red Fibers

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Voltage Gated sodium channel Autosomal Dominant on Chromosome 17 Treatement with Carbonic anhydrase inhibitors

answer

Hyperkalemic periodic paralysis

question

answer

Leigh's Syndrome

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Phosphofructokinase deficiency Decreased myophosphorylase Chromosome 11 Cramps with exercise, mild weakness Subsarcolemmal Blebs

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McArdle's Disease

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Dihydropyridine receptor mutation (Voltage cated calcium channel) Autosomal Dominant Chromosome 1 Treatment with Potassium and carbonic anyhydrase inhibitors

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Hypokalemic Periodic Paralysis

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"Happy Puppet" Maternal transmission of chromosome 15

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Angelman Syndrome

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Spreading limpness on stiffness, hand myokymia, continuous motor unit activity on EMG Potassium channel gene Chromosome 12 Treatment with Acetazolamide or Phenytoin

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Episodic ataxia - 1

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Delayed motor development, involuntary movements, compulsive self mutilation Decreased hypoxantine guanine phosphorribosyltransferase Increased uric acid Chromosome X

answer

Lesch-Nyhan

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Ataxia, retinitis pegmentosa, icthyosis Decreased phantic acid oxidase

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Refsum's Disease

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Ataxia, spasticity, optic atrophy, dementia, decreased MSR Lysosomal enzyme disorder Decreased aylsulftase A Chromosome 10

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Metachromatic Leukodystrophy

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Muscle stiffness (painless and worsened by cold) and hypertrophy Chloride channel Chromosome 7 Treatement with mexiletine, phenytoin, carbamazepine

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Myotonia congenita

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Peripheral involvement Van Recklinghausen Chromosome 17

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NF 1

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A form of spinocerebellar ataxia 6 Chromosome 19 half have migraines P/Q Calcium channel Treatment with acetazolamide

answer

Episodic Ataxia - 2

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answer

Krabbe's Leukodystrophy

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Acute infantile form Cherry red macula, hepatosplenomegaly, mental deterioration (psychomotor regression), postural hypotonia Decreased sphingomyelinase Vacuolated lymphocytes

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Niemann-Pick Type A

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Myotonia, facial and distal weakness, multiple endocrinopathies, temporal, masseter, and SCM wasting, distrubed GI motility 19 CTG trinucleotide repeat

answer

Myotonic Dystrophy

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Eponyms for Neuronal Ceroid Lipofuscinosis

answer

Santavuori-Haltia - 6-12 months on Chromosome 1 Jansky-Bielschowksy - 2-3 years on Chromosome 11 Batten or Speilmeyer-Vogt - 4-12 years on Chromosome 16

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Ataxia, dysarthia, decrease MSR, + babinski, decreased vibration sense, hypertrophic cardiomyopathy Frataxin 9 GAA trinucleotide repeat

answer

Friedreich's Ataxia

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Progressive dementia, hypotonia, ataxia, seizures (myclonus) Abnormal VEP and later blindness Lipofuscin strored in neurons

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Neuronal ceroid lipofuscinosis

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Dystonia, athetosis, rigidity, LMN signs and ataxia 14 CAG trinucleotide repeat

answer

Spinocerebellar Atrophy Type 3 (Machado-Joseph)

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Tuberous Sclerosis (Chromosome/Product)

answer

Chromosome 9, 16 Hemartin

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Narcolepsy (Chromosome)

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Chromosome 6

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Early onset primary dystonia DYT1 (Chromosome)

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Chromosome 9

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Amyloid Precursor Protein (Chromosome)

answer

Chromosome 21

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Apoplipoprotein E (Chromosome)

answer

Chromosome 19

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Alzheimer's - Late, familial (Chromosome)

answer

Chromosome 19

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Alzheimer's - Early, familial (Chromosome)

answer

Chromosome 14, 21

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Spastic Paraplegia (Chromosome)

answer

Chromosome X

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William's Syndrome (Chromosome)

answer

Chromosome 7

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Frontotemporal Dementia (Chromosome)

answer

Chromosome 17

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Wilson's Disease (Chromosome)

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Chromosome 13

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Acute Intermittent Porphyria (Chromosome)

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Chromosome 11

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CJD - Familial Gerstman-Schenker-Strauss (Chromosome)

answer

Chromosome 20

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Familial ALS (Chromosome)

answer

Chromosome 21

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medial medullary syndrome

answer

ipsilateral CNXII paralysis (fascicle of CNXII), contralateral hemiparesis (pyramid), and contralateral loss of position and vibratory sensation (medial lemniscus) anterior spinal artery distribution

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orbital apex components

answer

ophthalmic artery, ciliary ganglion and nerves, the fascicles of cranial nerves III, IV, VI, and the ophthalmic branch of cranial nerve V

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Damage to the fornix cause what S/Ss

answer

Papez circuit and results in loss of the ability to form new memories

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thalamus is supplied mainly 3A

answer

posterior cerebral, posterior communicating, and posterior choroidal

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name muscles trigeminal nerve supplies --the muscles of mastication

answer

temporalis, masseter, medial and lateral pterygoids, mylohyoid, anterior belly of the digastric, tensor veli palatini, and tensor tympani muscle

question

suprachiasmatic nucleus of the hypothalamus

answer

dominant circadian pacemaker of the mammalian brain.

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anterior nucleus of the thalamus project to?

answer

Fibers from the anterior nucleus of the thalamus project to the cingulate gyrus. The anterior nucleus receives projections from the mamillary bodies

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Neural crest derivatives

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dorsal root ganglia (involved in transmission of pain) and sympathetic ganglia Schwann cells and melanocytes

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insular cortex in put

answer

receives visceral nociceptive input via the ventromedial posterior (VMP) thalamic nucleus

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The layers traversed during a lumbar puncture

answer

skin, subcutaneous fat, supraspinous ligament, intraspinous ligament, ligamentum flavum, epidural fat, subarachnoid space

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central cord lesion, most suggestive feature

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upper extremities greater than lower, Pro more affect than Dis decreased pinprick sensation with retained fine touch spare vibration and light touch

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Opioid intoxication, pupil?

answer

constrict

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Transient global amnesia

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temporary but almost total disruption of short-term memory with a range of problems accessing older memories

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Williams syndrome

answer

deletions on chromosome 7 excellent social and language skills Marked impairment is noted on tests of visual processing cardiac, hyperacusis, endocrine disorders, and hypercalcemia

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Klüver-Bucy syndrome

answer

bilateral temporal lesions involving amygdalae

question

Define concussion

answer

Concussion is recognized as a clinical syndrome of biomechanically induced alteration of brain function, typically affecting memory and orientation, which may involve loss of consciousness (LOC).

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List 3 Diffuse astrocytomas ,WHO 2007

answer

WHO 2007 Diffuse astrocytomas 1, Low grade astrocytoma;(grade II) 2, anaplastic astrocytoma(III); 3, Glioblastoma (IV): mitotic figures prominent, vanscular proliferation and/or necrosis

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Pathological feature of low grade astrocytoma

answer

nuclear pleomorphism, mitotic figures rare

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Pathological feature anaplastic astrocytoma (grade III

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nuclear pleomorhism, mitotic figures prominent, NO vascular proliferation or necrosis

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Pathological feature Glioblastoma (IV)

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mitotic figures prominent, vanscular proliferation and/or necrosis

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Loss of Heterozygosity (LOH)

answer

occurs when a somatic cell contains only one copy of an allele due to non-disjunction during mitosis, segregation during recombination, or deletion of a chromosome segmen

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Primary gliobalstoma

answer

1,Glioblastoma arise de novo, without progression from lower grade glioma 2, IDH 1 mutation is rare 3, Loss of Heterozygosity 10q, EGFR amplification, p16 deletin and PTEN mutation

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Secondary glioblastoma

answer

1,Arise from lower grade glioma 2, Sequential acuisition of new mutation 3, IDH1 and P53 common 4, Temozolamide lead to hypermutation RB and Akt-mTOR pathway

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Diffuse gliomas favorable genetic signatures

answer

1p/19q co-deletion IDH mutation

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IDH mutation presents in what types of CNS tumors

answer

IDH1(R132H) prsent in 75% of grade II and III glioma Not present in primary glioblastomas Very rare in other types of primary CNS tumor

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prion disorders

answer

14-3-3 protein

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serotonin syndrome

answer

results from concomitant administration of medications that enhance serotonin transmission via decreased breakdown, or increased production

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clinical triad of abnormalities of serotonin syndrome

answer

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serotonin syndrome differentiated from neuroleptic malignant syndrome clinically

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serotonin syndrome has shiveringa and myoclonus

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serotonin syndrome how fast occur with medication changes

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within 6 to 8 hours of initiating or increasing serotonergic medications

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ideational apraxia

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the patient cannot correctly pantomime a multistep command

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ideomotor apraxia

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, in which the patient cannot perform on command simple actions such as waving good-bye or using a hammer

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Bromocriptine

answer

dopamine receptor agonist, which activates postsynaptic dopamine receptors. treatment of choice for neuroleptic malignant syndrome,

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Forced normalization

answer

psychosis occurring after achievement of good clinical seizure control, or resolution of interictal epileptiform discharges

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sarcoidosis CNS manifestatins

answer

motor and sensory deficits, progressive visual loss, and cranial nerve palsies. hypothalamus and basal forebrain resulting in hypopituitarism, hyperphagia, and hypersomnolence

question

West Nile virus (WNV) encepahlitis

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older patients mental status change movement disorder flaccid paralytic syndrome

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Rabie virus

answer

temporal and limbic lobes-memory loss and personality changes, seizure and death

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medial dorsal nucleus of the thalamus connections (AOL)

answer

amygdala, orbitofrontal region, and the temporal lobe ---disinhibition, utilization behavior, mania, and memory loss

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why need check glucose before tPA

answer

Hypoglycemia can present with signs of focal neurologic dysfunction and mimic stroke.

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fourth nerve palsy, head posiion

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(1) head tilt to opposite shoulder, (2) head turn downward with chin depressed and eyes upward, and (3) face turn to opposite side

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Acute dystonic reactions

answer

Anticholinergic medication, including diphenhydramine, is the most appropriate treatment

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copper deficiency

answer

question

REM sleep behavior disorder (RBD assoicated with pathology

answer

synucleinopathies

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which virus can cause a necrotizing myelopathy

answer

Varicella zoster virus

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Oral hormone contraception and lamotrigine

answer

(OHC) induces this system and may result in a significant lowering of serum LTG levels. LTG has no effect on OHC metabolism and does not increase the risk of OHC failure.

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Schilder's disease

answer

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Marburg Disease

answer

the fulminant form of multiple sclerosis, is a rare disease that typically kills within a year.

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Charcot-Marie-Tooth disease (CMT) 3 common types

answer

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Superior semicircular canal dehiscence

answer

perilymphatic fistula and is characterized by sound, pressure induced vertigo, and oscillopsia.

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Ménière disease,

answer

due to endolymphatic hydrops

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Mollaret meningitis

answer

recurrent, self-limiting, aseptic meningitis caused by the herpes simplex virus 2 and may benefit from treatment with acyclovir

question

level of evidence from AAN

answer

class A, established, need 2 class I studies Class B, probable, need 1 class I and 2 class II studies class C, possible, need 1 Class II and 2 Class II studies Class U, inadequated, unapproved

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Metronidazole neuro- toxicity

answer

sensorimotor polyneuropathy affecting both large and small fibers recovery, 6 to 12 months

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neurogenic claudication

answer

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Down syndrome, neuro

answer

Down syndrome can be associated with atlantoaxial instability

question

what neuropathy unique to Sjögren syndrome

answer

Dorsal root ganglion involvement leading to an ataxic sensory neuronopathy

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what neuropathy is a common neurologic feature of connective tissue diseases

answer

Trigeminal sensory neuropathy

question

on bevacizumab, developed hypertension, a seizure, and cortical blindness.

answer

reversible posterior leukoencephalopathy

question

Allelic loss (loss of heterozygosity) of chromosomes 1p and 19q are molecular signatures of which tumor

answer

grade III (anaplastic) oligodendrogliomas. increased responsiveness to chemotherapy and increased length of survival compared to grade III astrocytomas and grade IV gliomas

question

trigeminal neuralgia (tic douloureux) DD

answer

vascular loop structural causes such as a meningioma, cerebellopontine angle neoplasm, and aneurysm. Multiple sclerosis stroke

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A late posttraumatic seizure

answer

defined as a seizure that occurs more than 1 week after a head injury

question

progressive supranuclear palsy

answer

tauopathy, early falls, bradykinesia, and marked axial rigidity Supranuclear gaze palsy occurs with initial impairment of down gaze

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MRI characteristic of Creutzfeldt-Jakob disease (CJD)

answer

restricted diffusion in the cortical mantle and basal ganglia CSF-neuron-specific enolase and 14-3-3 protein

question

FDA advises of MS pregancy

answer

question

typically presention of cerebral amyloid angiopathy

answer

elderly persons with spontaneous lobar hemorrhage

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cerebral autosomal dominant arteriopathy, with subcortical infarcts and leukoencephalopathy (CADASIL)

answer

NOTCH3 gene on chromosome 19.

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heaadache and breast feeding

answer

question

RLS is characterized by..

answer

question

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes)

answer

serum vascular endothelial growth factor (VEGF) is one of the major diagnostic criteria supporting a diagnosis of POEMS

question

Charles Bonnet syndrome

answer

complex visual hallucinations in the setting of significant visual deprivation

question

Paroxysmal hemicrania Tx

answer

exquisitely sensitive to indomethacin

question

described premotor features of idiopathic Parkinson disease

answer

Anosmia, REM sleep behavior disorder, constipation and depression

question

Balint syndrome

answer

simultagnosia, oculomotor apraxia and optic ataxia

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the North American Antiepileptic Drug (AED) Pregnancy Registry findings

answer

question

acute onset of epidural spinal cord compression Tx

answer

surgery within 24 hours had significant improvement in their ability to walk and the length of time that they were able to continue to walk.

question

Nitrous oxide toxicity

answer

question

Natalizumab-induced PML

answer

discontinuation of natalizumab and initiation of plasma exchange IRIS (immune reconstitution inflammatory syndrome) treatment for IRIS is high-dose glucocorticoids followed by a slow taper

question

Cerebrovascular disease occurs in ??% of sickle cell disease

answer

25% of sickle cell disease Eighty percent of events occur at or before age 15 chronic transfusion therapy to keep hemoglobin S below 30%

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Hallmarks of Friedreich ataxia

answer

question

atlantoaxial instability

answer

Down syndrome Klippel-Feil ( abnormal fusion), Morquio (mucopolysaccharidosis IV), Larsen syndromes, achondroplasia previous cervical spinal surgery

question

exposed to valproic acid in utero

answer

valproic acid in utero had significantly lower IQ scores than children of the same age who had in utero exposure to to carbamazepine, lamotrigine, and phenytoin

question

Lesch-Nyhan syndrome

answer

dystonia typically prior to 12 months self-injurious behavior, between 2 and 4 years of age hypoxanthine-guanine phosphoribosyltransferase

question

Bartonella henselae (catscratch disease) signs on O/E

answer

enlarged lymph node in the arm axilla or neck

question

Niemann-Pick disease type C

answer

autosomal recessive inheritance hepatosplenomegaly, progressive neurodegeneration; vertical supranuclear (usually downgaze) palsy, ataxia, dystonia, seizures, gelastic cataplexy, and dementia

question

Metachromatic leukodystrophy

answer

autosomal recessive disorder periventricular and deep white matter with relative sparing of the U-fibers (long tract signs alone are common Neurocognitive symptoms can be the presenting

question

Pelizaeus- Merzbacher Diseasem MRI

answer

white matter in the centrum semi-ovale is isodense with the cortical ribbon on T1- weighted imaging

question

l-asparaginase

answer

cause significant hypercoagulability, particularly secondary anti-thrombin III deficiency, which this patient had. Venous sinus thromboses, with infarctions in a venous distribution, are the result.

question

Joubert syndrome

answer

hypoplasia of the cerebellar vermis most common features of this syndrome include hyperpnea, hypotonia, oculomotor apraxia, ataxia, and mental retardation

question

molar tooth sign

answer

question

subdural hemorrhages in the absence of inflicted trauma in infants

answer

Menkes disease, type 1 osteogenesis imperfecta, and glutaric aciduria type 1

question

bacterial meningitis long term complication

answer

question

Mutations of filamin A linked to ??

answer

bilateral periventricular heterotopias, enlarged cistern magna thinning or agenesis of the corpus callosum. aortic aneurysm

question

diagnosis of spasmus nutans

answer

dysconjugate, highly variable nystagmus, head nodding head tilt, without ophthalmologic abnormalities, and with normal neuroimaging

question

Congenital nystagmus

answer

conjugate and suppressed by convergence (in contrast to spasmus nutans,which is typically increased by convergence)

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Opsoclonus

answer

chaotic, conjugate saccades, seen classically in the paraneoplastic syndrome associated with neuroblastoma.

question

evidence of treatment of Bell palsy

answer

There is Class 1 evidence supporting treatment with steroids to improve recovery. There is weak evidence for antiviral treatment. There is no benefit to combination therapy

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sensitivity

answer

number of true positives over true positives plus false negatives

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Specificity

answer

is the number of true negatives over true negatives plus false positives

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Myoclonic epilepsy with ragged red fibres.

answer

mitochondrial DNA (mtDNA) gene MT-TK encoding tRNA lysine (tRNALys) is the gene most commonly associated with MERRF (found in 80% of patients).

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Neurogenic pattern

answer

question

Myopathic pattern

answer

Necrosis, myophagocytosis Fiber splitting, Fiber size variation (hypertrophy and splitting) Central nuclei, Basophilia (degeneration), Non-grouped atrophy Fibrosis / lipid deposition

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Retinitis Pigmentosa

answer

NARP, MNGIE, Refsum's, Abetalipoproteinemia, KS

question

Tay-Sach

answer

Hexosamindase A

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Sandhoff

answer

Hexosamindase A & B

question

Fragile X Premutation Syndrome MRI

answer

MRI show hyperintensity in middle cerebellar peduncles

question

Fragile X Premutation Syndrome

answer

50(55)-200: Premuation FX tremor/ataxia syndrome (FXTAS)

question

Maple Syrup Urine Disease MRI

answer

characteristic edema affecting deep cerebellar WM, dorsal brainstem, cerebral peduncles and internal capsule

question

Maple Syrup Urine Disease-Tiral of thiamine

answer

- there is a thiamine responsive variant

question

Anticipation

answer

Expanded repeats usually enlarge when passed to successive generations, producing more severe disease at earlier age of onset

question

imprinted genes

answer

imprinted genes are only active from one parental allele

question

Harding's Essential Diagnostic Criteria for Friedreich Ataxia

answer

question

DNA repair defect

answer

question

Ataxia with vitamin E deficiency

answer

Treatment: Vitamin E supplementation

question

Refsum's disease

answer

Dietary reduction of phytanic acid

question

Cerebrotendinous xanthomatosis

answer

question

Episodic ataxia type 1 & 2

answer

Acetazolamide (500-700 mg daily) often completely eliminates the attacks (better and more predictable response in type 2 than type 1)

question

Abetalipoproteinemia

answer

Restriction of dietary fat and supplementation of vitamins ADEK (often massive doses)

question

Wilson Disease

answer

D-penicillamine

question

FRAXA

answer

X-linked FMR1, on the X-chromosome CGG

question

FXTAS (Fragile Xassociated tremor/ ataxia syndrome)

answer

X-linked FMR1, on the X-chromosome CGG

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FRAXE (Fragile XE mental retardation)

answer

X-linked AFF2 or FMR2, on the X-chromosome GCC

question

FRDA (Friedreich's ataxia)

answer

AR FXN or X25, (frataxin) GAA

question

DM (Myotonic dystrophy)

answer

AD, DMPK CTG

question

SCA8 (Spinocerebellar ataxia Type 8)

answer

AD OSCA or SCA8 CTG

question

SCA12 (Spinocerebellar ataxia Type 12)

answer

AD PPP2R2B or SCA12 CAG

question

Metachromatic LD

answer

arylsulfatase A

question

Fabry

answer

-galactosidase A

question

Tangiers

answer

-lipoproteinemia (ABC transporter deficiency) (ABCA1 Gene)

question

Gaucher

answer

β-glucocerebrosidase

question

Niemann-Pick

answer

Sphingomyelin phosphodiesterase 1

question

Canavan

answer

Aspartoacylase - U fiber involvement

question

Alexander

answer

GFAP (frontotemporal)

question

Adrenoleukodystrophy

answer

ABCD1 (VLCFA accumulation, posterior)

question

Pelizaeus-Merzbacher

answer

Proteolipid protein 1 (PLP1)

question

Vanishing White Matter

answer

eIF2B5

question

What is the most prominent efferent pathway from the amygdaloid

answer

question

where is the frontal eye field in humans

answer

The frontal eye field in humans is located in the caudal part of the middle frontal gyrus. Efferents from this region project to the paramedian pontine reticular formation (PPRF).

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medial medullary syndrome

answer

question

Innervation of the detrusor muscle

answer

question

baroreceptor reflex, which nerve

answer

question

thalamus Blood Supply

answer

question

the order of Myelination

answer

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cervical central spinal cord syndrome

answer

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Asomatognosia

answer

patient denies ownership of a limb contralateral to a lesion of the supramarginal gyrus of the parietal lobe (usually nondominant).

question

zone of Lissauer (dorsolateral fasciculus)

answer

The zone of Lissauer (dorsolateral fasciculus) is adjacent to lamina I in the spinal cord. It is composed of fibers that originated in the dorsal roots

question

connection between GPi and thalamus

answer

The internal segment of the globus pallidus sends outputs to the thalamus through the ansa lenticularis and the lenticular fasciculus

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"one-and-a-half" syndrome

answer

question

masseter

answer

trigeminal nerve

question

levator palpebrae

answer

oculomotor nerve

question

genioglossus

answer

hypoglossal nerve

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stylopharyngeus

answer

glossopharyngeal nerve

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dorsal nucleus of Clarke

answer

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where rubrospinal tract runs

answer

The rubrospinal tract runs lateral to the lateral corticospinal tract in the dorsal funiculus of the spinal cord and is the most lateral of the five descending pathways listed.

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straight (rectus) sinus drainage

answer

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nucleus accumbens involved in which neurological/psychiatric condition

answer

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Neuro embrology, what is BASAL AND ALAR PLATES

answer

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The major function of amygdala, caudate, nucleus accumbens and hippocampus

answer

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orbitofrontal syndrome

answer

The orbitofrontal syndrome consists of socially inappropriate behaviors as well as poor impulse control with disinhibition.

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lateral convexity syndrome

answer

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mesial frontal syndrome

answer

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which spinal segment is most vulnerable to ischemic insult

answer

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symptoms of lesion of mediodorsal nucleus of the thalamus

answer

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anterior choroidal artery

answer

The anterior choroidal artery usually arises from the internal carotid artery and feeds portions of the globus pallidus, putamen, thalamus, and the posterior limb of the internal capsule

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what is dentate gyrus

answer

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Pathway of Primary nociceptive afferents

answer

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Weber's syndrome

answer

A lesion at the base of the midbrain (cerebral peduncle) will produce an ipsilateral third nerve palsy and contralateral hemiparesis (Weber's syndrome).

question

muscle supplied by CN V

answer

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muscle supplied by CN VII

answer

The facial nerve supplies the frontalis, corrugator, orbicularis oculi, nasalis, buccinator, orbicularis oris, mentalis, and platysmas.

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ocular tilt reaction.

answer

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eye movements

answer

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suprachiasmatic nucleus

answer

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end organs involved in perception of vibration

answer

Pacinian corpuscles found in subcutaneous connective tissue and in periosteum.

question

insular cortex receive what input

answer

The insular cortex receives visceral nociceptive input via the ventromedial posterior (VMP) thalamic nucleus.

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Mesolimbic and mesocortical projections

answer

Mesolimbic and mesocortical projections arise from the ventral tegmental area of the midbrain. The fibers are dopaminergic, and travel to cerebral cortex and limbic structures, such as the amygdala.

question

fornix

answer

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common gait disorders

answer

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Which of the sensory pathways projects outside the thalamus

answer

olfactory pathway.

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What are the 2nd order neurons of the olfactory pathways?

answer

mitral and tufted cells.

question

Where is the primary olfactory cortex located?

answer

temporal lobe.

question

The axons of _______ cells in the retina collectively form the optic disc.

answer

ganglion cells of retina

question

The optic tract extends from the dorsolateral aspect of the _______ to the ________.

answer

optic chiasma lateral geniculate body

question

Thalamic nucleus that is a relay station for all axons of retinal ganglion cells subserving vision:

answer

lateral geniculate body (L for light)

question

Lateral geniculate nucleus is laminated into structure containing how many layers?

answer

question

Cochlear nerve extends from ______.

answer

organ of Corti

question

The spiral ganglion is related to:

answer

auditory pathway.

question

The 3rd order neurons of the auditory pathway which end in the medial geniculate body, originate from:

answer

inferior colliculus.

question

The ascending pathway used for propioception of the legs:

answer

dorsal spinocerebellar tract.

question

Vibration sensation from forelimbs and hindlimbs is transmitted via the _______ and the _______.

answer

dorsal column and spinocervical thalamic tract.

question

Proprioception sensation from forelimbs project through:

answer

dorsal columns

question

Proprioception sensation from hindlimbs project through:

answer

gracile tract upto the level of dorsal nucleus of Clarke and from there they leave the gracile tract and synapse with the dorsal nucleus of Clark and ascend via the dorsal spinocerebellar tract.

question

Long thoracic nerve (C5, C6, C) innervates

answer

serratus anterior muscle

question

Suprascapular nerve innervates

answer

supraspinatus and infraspinatus muscles

question

Dorsal scapular nerve innervates

answer

rhomboids and levator scapulae muscles.

question

Axillary nerve arises from ______ cord.

answer

posterior

question

Root innervation of axillary nerve

answer

C5, C6.

question

Axillary nerve innervates

answer

deltoid and teres minor muscles

question

Lateral brachial cutaneous nerve is a branch of _____ nerve, and supplies the skin over the outers surface of the arm, mainly _____ region.

answer

deltoid

question

The first _____ degrees of arm abduction from trunk is performed by supraspinatus muscle

answer

question

Musculocutaneous nerve arises from _____ cord.

answer

lateral

question

Nerve root innervation of musculocutaneous nerve

answer

C5, C6, C7

question

What are does the musculocutaneous nerve innervate?

answer

BBC. Biceps, brachialis, and coracobrachialis.

question

Lateral cutaneous nerve of the forearm is a continuation of _______ nerve.

answer

musculocutaneous.

question

Muscle that supinates forearm, especially if the elbow is flexed at 90 degrees.

answer

biceps.

question

Lesion of which nerve results in weakness of elbow flexion against resistance in a fully supinated hand, possible weakness in arm elevation, arm pain, and radial forearm paresthesia.

answer

musculocutaneous.

question

Compression site of the radial nerve.

answer

spiral groove in the posterior aspect of the humerus.

question

Radial nerve arises fromm _____ cord.

answer

posterior

question

To differentiate between a lesion of upper brachial plexus and one of the lateral cord, the most useful muscle to test is the:

answer

teres minor

question

To differentiate between a lesion of lower trunk of brachial plexus and one of the medial cord, the most useful muscle to test is the:

answer

extensor indicis proprius

question

To differentiate between a lesion of middle trunk of brachial plexus and one of the posterior cord, the most useful muscle to test is the:

answer

pronator teres

question

In the sciatic nerve, before it divides, the medial trunk of the nerve comprises fibers of the _____ and of the _____ nerves.

answer

tibial and peroneal

question

The only thigh muscle innervated by the lateral peroneal division of sciatic nerve.

answer

short head of biceps

question

Adductor magnus is supplied by two nerves.

answer

obturator and sciatic nerves.

question

Hamstrings are innervated by ______ nerve.

answer

sciatic.

question

Sartorius muscle is innervated by ______ nerve.

answer

femoral

question

Carpal tunnel contains how many structures?

answer

question

What are the structures in the carpal tunnel?

answer

4 tendons of flexor digitorum superficialis 4 tendons of flexor digitorum profundus median nerve 1 tendon of flexor pollicis longus

question

What bones make up the Guyon's canal?

answer

Hamate and pisiform bones.

question

What is the Guyon's canal related to?

answer

compression site of the ulnar nerve at wrist.

question

Genitofemoral nerve divides near the _______ into the external genital branch and the medial femoral branch

answer

inguinal ligament

question

Name the nerve innervating the cremaster muscle.

answer

external genital branch of the genitofemoral nerve.

question

Name the nerve innervating the skin over the femoral triangle.

answer

medial femoral branch of the genitofemoral nerve.

question

Superior gluteal nerve innervates _____ and _____ muscles.

answer

gluteus medius and gluteus minimis.

question

Gluteus maximus is innervated by

answer

inferior gluteal nerve.

question

The root fibers of the _____ nerve course in the medulla oblangata lateral to the medial leminiscus and emerge on the ventral surface of the medulla between the _____ and _____.

answer

hypoglossal nerve. pyramid and inferior olive

question

The gaze-holding neural integrator for vertical gaze is located in _________.

answer

interstitial nucleus of Cajal.

question

Vertical and torsional saccade is controlled by

answer

cortical pathways descending to the rostral interstitial nucleus of the medial longitudinal fasiculus at the junction between the midbrain and thalamus.

question

Horizontar gaze center

answer

PPRF and abducens nucleus

question

The gaze-holding neural integrator for horizontal gaze

answer

horizontal gaze cener, medial vestibular nucleus, and nucleus prepositus hypoglossi.

question

Which of the cranial nerves exits the brainstem from its dorsal aspect?

answer

Trochlear nerve

question

The fibers of _____ nucleus cross to the other side of the brainstem just prior to exiting the pons.

answer

trochlear

question

Superior oblique muscle of eye is innervated by

answer

trochlear nerve.

question

Perihypoglossal nuclei are involved in

answer

extraocular movement.

question

Ventral motor nucleus of vagus

answer

nucleus ambiguus

question

Nucleus ambiguus

answer

SVE to branchiomeric muscles of pharynx and larynx. Efferent fibers to CN 9 and CN 11.

question

Taste sensation and pain from the epiglottis and posterior third of tongue is received by

answer

nucleus tractus solitarius (rostral and lateral zone)

question

Nuclei involved in the cardiovascular control

answer

nucleus tractus solitarius, dorsal motor nucleus of vagus, caudal and ventrolateral medulla, which receive projections from sensorimotor cortex.

question

Taste sensation is conveyed by

answer

facial, glossopharyngeal, and vagus nerves.

question

Taste sensation from anterior two-thirds of tongue

answer

facial nerve

question

Taste sensation from posterior third of tongue

answer

glossopharyngeal nerve

question

Taste sensation from epiglottis

answer

vagus nerve

question

Anosognosia for hemiplegia is seen in lesions of

answer

nondominant parietal lobe. pontine reticular nucleus in the tegmentum and corticospinal fibers in the basis pontis.

question

Pituitary adenomas where

answer

arise from within the sella

question

Chordomas where

answer

heterogenous tumors consisting of notochord remnants which most often occur in the clivus and sacrococcygeal regions.

question

Craniopharyngiomas where

answer

centered in the suprasellar (rather than intrasellar) compartment and contain cysts and calcification

question

Giant cell (temporal) arteritis

answer

question

Hypertension CNS hemorrhage

answer

deep grey matter pons and rarely cerebellum

question

Neurosarcoid

answer

question

Kufs disease

answer

adult form of neuronal ceroid lipofuscinosis

question

typical microscopic findings of hemangioblastoma

answer

vacuolated stromal cells and variably sized vascular structures

question

Dandy-Walker malformation

answer

vermis may be either absent entirely or hypoplastic enlarged posterior fossa brainstem microscopic developmental abnormalities

question

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

answer

Notch 3 gene on chromosome 19.

question

CADASIL

answer

HTRA1 gene

question

neuromyelitis optica Pathology

answer

The presence of necrosis in spinal cord virtually defines the entity

question

monoamine oxidase (MAO) and cheese

answer

prevents dietary pressor amines, typically tyramine, from entering the tissues, a marked hypertensive response (the "cheese reaction") can occur

question

Selegiline

answer

(a preferential MAO-B inhibitor) has no MAO-A inhibition at 10 mg/d or less, but at 20 mg/D or above it has

question

Fingolimod

answer

sphingosine-1 phosphate-receptor reduces lymphocyte recirculation from the lymph nodes resulting in functional immunosuppression.

question

cardiac effects of fingolimod

answer

maximal after the first dose but persist for about 14 days

question

tramadol and Sz

answer

Daily doses of 400 mg or more of tramadol may provoke seizure activity

question

Oxcarbazepine and Na

answer

produce hyponatremia due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH)

question

Fabry's disease

answer

X-linked defect in alpha-galactosidase painful peripheral neuropathy with autonomic manifestations, a typical rash

question

Topiramate

answer

question

Causes of a sensory neuronopathy

answer

question

pathophysiology of migraine

answer

question

Anti-Yo

answer

the anti-Purkinje cell antibody and reacts only with Purkinje cells. anti-Yo have a gynecological cancer, ovarian or breast 90% of the time

question

analgesic effects of pregabalin

answer

decrease in central sensitization and nociceptive transmission to the action on the alpha-2-delta subunit of the calcium channel

question

manganese

answer

question

REM sleep behavior disorder (RBD) tx

answer

Clonazepam is efficacious and well tolerated by the majority of patients. melatonin may be a good alternative to clonazepam

question

Meds avoided in patients with RBD.

answer

Monoamine oxidase inhibitors, tricyclic antidepressants, serotonergic synaptic reuptake inhibitors noradrenergic antagonists

question

clozapine

answer

Up to 1% percent of patients for several months experience bone marrow suppression

question

Dalfampridine (4-AP)

answer

potassium-channel blocker that improves nerve conduction along centrally demyelinated axons 0.2% risk of seizure sassociated with toxic blood levels

question

risk of Stevens-Johnson syndrome

answer

The HLA-B*1502 allele increases the risk of Stevens-Johnson syndrome after carbamazepine exposure by 10 times

question

Teriflunomide (TF)--relapsing-remitting multiple sclerosis

answer

reversibly inhibits dihydroorotate dehydrogenase, a key mitochondrial enzyme involved in new pyrimidine synthesis for DNA replication

question

pedunculopontine nucleus (PPN)

answer

inputs from the globus pallidus and subthalamic nucleus, and projects to the substantia nigra, thalamus, and medullary reticulospinal neurons

question

Tardive dyskinesia

answer

dopaminergic D2 receptor blockers classical highpotency neuroleptics, but also antiemetic agents such as prochlorperazine or metoclopramide.

question

Blockade of histamine (H-1 and H-2) receptors can cause what clinical signs

answer

sedation, drowsiness; potentiation of central depressant drugs; weight gain.

question

Blockade of muscarinic acetylcholine receptors can cause what clinical signs

answer

dry mouth, blurred vision, sinus tachycardia, constipation, urinary retention, memory impairment

question

Blockade of norepinephrine uptake can cause what clinical signs

answer

tremors, jitteriness, tachycardia, and diaphoresis, erectile and ejaculatory dysfunction

question

Blockade of serotonin uptake can cause what clinical signs

answer

sexual dysfunction, nausea, vomiting, diarrhea, anorexia, increase or decrease in anxiety (dose-dependent), asthenia (tiredness), insomnia, and extrapyramidal side effects.

question

Blockade of serotonin-2 (5-HT2) receptors

answer

ejaculatory dysfunction, hypotension, alleviation of migraine headaches, decrease in anxiety, and decrease motor restlessness

question

Blockade of a1-adrenergic receptor can cause what clinical signs

answer

postural hypotension, dizziness which predisposes to falls possibly resulting in broken bones and subdural hematomas

question

Blockade of a2-adrenergic receptors can cause what clinical signs

answer

priapism

question

drugs of choice of of treatment of severe hypertension in neurological catastrophes

answer

labetalol, esmolol, and enalapril

question

key role of CoQ-10

answer

an electron shuttle between complexes I or II and complex III in the respiratory chain

question

Primary CoQ-10 deficiency

answer

myopathy or progressive cerebellar degeneration

question

Charcot-Marie-Tooth disease 1B

answer

myelin protein zero

question

Charcot-Marie-Tooth disease type 1A

answer

Point mutations of the peripheral myelin protein-22 gene in chromosome 17p

question

Tramadol,

answer

centrally acting analgesic structurally related to codeine and morphine, consists of two enantiomers

question

Lamotrigine

answer

metabolized in the liver predominantly by glucuronic acid conjugation

question

Carbamazepine, phenytoin, and phenobarbital induce glucuronic acid conjugation of lamotrigine resulting in a ??% reduction of the lamotrigine level

answer

40%

question

Valproic acid inhibits glucuronic acid conjugation of lamotrigine ____ the lamotrigine level

answer

doubling

question

mexiletine

answer

reduce the associated hyperexcitability of the sarcolemma by interfering with sodium channels located on it.

question

narcolepsy is associated with human leucocyte antigen (HLA)??

answer

Over 90% of patients with narcolepsycataplexy carry HLA-DQB1*0602 probably have an autoimmune disorder

question

VPA side effect mechanism

answer

question

Midodrine

answer

treatment of orthostatic hypotension unresponsive to fludrocortisone

question

Codeine,

answer

metabolized by glucuronidation

question

Paralytic shell-fish poison (saxitoxin)

answer

resembles tetrodotoxin in its ability to reversibly bind to membrane voltage-gated sodium channels

question

Fosphenytoin

answer

question

intramuscular administration Fosphenytoin

answer

therapeutic phenytoin plasma concentrations are observed within 30 minutes and maximum plasma concentrations occur at approximately 30 minutes

question

Single fiber EMG (SFEMG)

answer

question

Repetitive stimulation >10Hz

answer

Rapid repetitive stimulation (more than 10 hz) is used in presynaptic disorders of neuromuscular transmission

question

Somatosensory evoked potentials

answer

record activity in the dorsal column/medial lemniscal sensory pathways

question

Andersen-Tawil syndrome

answer

is caused by a potassium channel mutation and includes attacks of weakness, cardiac arrhythmias and dysmorphic features

question

Hypokalemic periodic paralysis

answer

is caused by a calcium channel mutation, does not demonstrate myotonic and is characterized by attacks of weakness

question

14 and 6 phantom spikes

answer

question

Hypsarrhythmia

answer

high-voltage and irregular slow waves with multiple sharp and spike discharges

question

Subacute sclerosing panencephalitis -EEG

answer

generalized periodic long-interval diffuse discharges

question

Spongiform encephalopathies-EEG

answer

PEDs, but intervals are typically shorter than those of SSPE.

question

HSV-EEG

answer

lateralized periodic epileptiform discharges

question

Tay Sachs disease-EEG

answer

slow background with or without multifocal epileptiform transients

question

Alzheimer's disease-EEG

answer

normal EEGs or sometimes having excessive theta activities

question

facial nerve compound muscle action potential (CMAP)

answer

lower the CMAP amplitude, the more axonal degeneration and the poorer the prognosis

question

hereditary neuropathy with liability to pressure palsies (HNPP).

answer

This disorder is caused by a deletion or point mutation in the PMP22 gene

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