USMLE – Neurology – Flashcards
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Klüver-Bucy syndrome
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In bilateral Amygdala lesions. Sx: Hyperorality, hypersexuality, disinhibited behavior.
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Wernicke-Korsakoff syndrome
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In bilateral mamillary bodies lesions. Path.: Alcoholism, B1 - Thiamine deficiency. Sx: Wernicke: Confusion, ophtalmoplegia, ataxia; Korsakoff: Memory loss, confabulation, personality loss.
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Cerebellar lesions (hemisphere vs. vermis)
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Hemisphere lesions: ipsilateral ataxia, intention tremor. Vermis: truncal ataxia, dysarthria.
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Lesion of subthalamic nucleus
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contralateral hemiballismus
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Lesion of hippocampus
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anterograde amnesia
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Lesions of PPRF
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Paramedian pontine reticular formation = Importent for horizontal gaze and saccades. When lesioned eyes look away from side of lesion
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Lesion of Frontal eye fields
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Area in premotorik cortex = Importent for volontary eye movements. When lesioned eyes look to side of lesion
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Central pontine myelinolysis
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Sx: Acute paralysis, dysarthria, dysphagia, diplopia. Path.: To fast correction of hyponatremia.
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Lesions of arcuate fasciculus
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Connection between Wernicke's and Broca's. Sx: Conduction aphasia = Poor repetition, fluent speech, intact comprehension
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Wallenberg's syndrome
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lateral medullary syndrome; PICA infarct. Sx: dissociated sensory loss: at body contralateral loss of pain and temperature, ipsilateral ataxia; at face ipsilateral symptoms: loss of pain and temperatur, Horner's, dysphagia (CN IX); hoarseness (CN X), vomiting
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Posterior communicating artery lesions
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common area of aneurysm; CN III palsy
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Anterior communicating artery lesions
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common area of aneruysm; visual field defects
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Lesions of watershed zones between anterior & middle cerebral artery; middle & posterior cerebral artery
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Sx: Proximal leg/arm weakness; defects in high-order visual processing
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Locked-in syndrome
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Global palsy; CN III often intact -> Communication via horizontal eye movements.
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Foramina of Luschka
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Lateral foramina from 4th ventricle into subarachnoid space
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Foramina of Magendie
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Medial foramina from 4th ventricle into subarachnoid space
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Normal pressure hydrocephalus
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aka. normal pressure hydrocephalus of the elderly. Path.: idiopathic; nocturnal elevated pressures. Sx: Triad of ataxia, dementia, urinary incontinence.
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Spinothalamic tract
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(= Tractus spinothalamicus anterior & lateralis). Temperature and pain from contralateral side. Legs lateral, arms medial. 1st synapse at segment -> 2nd neuron decussates at anterior white commissure -> ascends in contralateral spinothalamic tract -> VPL -> 3rd neuron in sensory cortex
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Lateral corticospinal tract
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(= Tractus corticospinalis lateralis; Pyramidenbahn). Motoric innervation for ipsilateral side. Legs lateral, arms medial. 1st neuron (UMN) in 1° motor cortex -> ipsilateral internal capsule -> decussates in caudal medulla (pyramidal decusssation) -> descends in contralateral lateral corticospinal tract -> 1st synapsis in anterior horn of gray matter -> 2nd neuron (LMN) leaves cord with spinal nerve -> 2nd synapsis is neuromuscular junction.
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Dorsal columns
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(= Fasciculus cuneatus, Fasciculus gracilis). Fine tactil information, vibration, conscious proprioception. Legs medial, arms lateral. 1st synapse at segment in ncl. cuneatus/gracilis -> 2nd neuron ascends ipsilateral and decussates in medulla -> ascends in contralateral medial lemniscus ->2nd synapse in VPL -> 3rd neuron in sensory cortex
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ALS aka Lou Gehrig's disease
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Path.: superoxide dismutase 1 defect; betel nut ingestion. Sx: Combined UMN and LMN defects. No sensory deficits.
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Tabes dorsalis
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= Neurosyphilis. Degeneration of dorsal roots and dorsal columns. Sx: Impaired proprioception, ataxia
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Syringiomyelia
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central spinal cord lesions (syringobulbia at brainstem). Sx: Degeneration of anterior horn of gray matter -> ipsilateral paresis. Defeneration of anterior white commissura -> ipsilateral impairment of pain and temperature sensation
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Friedreich's ataxia
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autosomal rec. (GAA repeat on 9q) -> reduced Frataxin production -> Demyelination of dorsal columns, lateral corticospinal tract, spinocerebellar tracts. Sx: Ataxia, hyperreflexia, impaired proprioceptiv sensation. DD: Vit B12 deficiency, Vit E deficiency
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Poliomyelitis and Werdnig-Hoffmann disease
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LMN affection by destruction of anterior horns of gray matter. Werdnig-Hoffmann is aut. rec. infantile spinal muscular atrophy ("floppy baby")
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Afferent Muscle spindle nerve type
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Ia; Efferent to extrafusal fibers: alpha-motoneuron; Efferent to intrafusal fibers: gamma motor neuron
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Afferent Golgi tendon organ nerve type
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Ib
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Moro reflex
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Primitive reflex (disappear in 1st year): abduction and adduction of extremities when startled
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Rooting reflex
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Primitive reflex (disappear in 1st year): turning of head, when cheek is touched ("nipple seeking")
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Sucking reflex
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Primitive reflex (disappear in 1st year): starting to suck, when mouth is touched
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Palmar and plantar reflex
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Primitive reflexes (disappear in 1st year): curling of toes/fngers, when palm or sole of foot are touched
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Babinski reflex
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Primitive reflex (disappear in 1st year): dorsiflexion of large toe, fanning of other toes with plantar stimulation
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Parinaud syndrome
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Lesion of superior colliculli. Sx: Paralysis of conjugate vertical gaze
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Ncl. solitarius
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Visceral sensory information: e.g. taste, baroreceptors, gut distention. CN VII, IX, X
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Ncl. ambigous
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Motor innervation of pharynx, larynx, prox. esophagus: e.g. swallowing, palate elevation. CN IX, X, XI
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Structures through Optic canal
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CN I, A. ophtalmica
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Structures through superior orbital fissure
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CN V1 (N. frontalis), CN III, IV, VI
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Structures through Foramen rotundum
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CN V2 (N. maxillaris)
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Structures through Foramen ovale
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CN V3 (N. mandibularis)
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Structures through Foramen spinosum
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Middle meningeal artery
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Structures through Internal auditory canal
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CN VII, VIII
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Structures through Jugular foramen
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CN. IX, X, XI, Jugular vein
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Structures through Hypoglossal canal
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CN XII
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Structures through Foramen magnum
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CN XI (spinal roots), brain stem, vertebral arteries
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Structures through Cavernous sinus
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CN III, IV, VI; CN V1, V2; Internal carotid artery
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Cavernous sinus syndrome
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Sx: Ophtalmoplegia; Sensory loss of CN V1, V2 area
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CN XII LMN lesion
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Ipsilateral palsy of M. genioglossus -> Tongue deviates toward lesioned side
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CN X lesion
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Ipsilateral Arcus palatopharyngeus collapses -> Uvula deviates away from lesioned side
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CN VII Lesion - UMN
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Contralateral paralysis of lower face. Upper face intact bec. of bilateral innervation by UMN.
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CN VII Lesion - LMN
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Ipsilateral paralysis of upper and lower face.
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Mastication muscles
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Closing jaw: Mm. masseter, temporalis, Medial pterygoid muscle. Opening jaw: Lateral pterygoid muscle (Lateral lowers)
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papilledema
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Elevated optic disk with blurred margins, bigger bild spot. Sign of incr. intracranial pressure.
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Strabismus vs. Amblyopia
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Strabismus = Misalignment of eyes. Amblyopia = Reduction of vision bec. of disuse in critical period (2nd to unequal refractive errors, strabismus)
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Marcus Gunn pupil
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Afferent defect in pupillary reflex. Sx: Decreased bilateral contraction when affected side is illuminated. Normal bilateral contraction, when healthy side is illuminated.
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ARMD
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Age related macular degeneration. Sx: Scotomas. Dry/atrophic ARMD: slow, due to fat deposits. Wet ARMD: rapid, due to neovascularization
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MLF syndrome
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Internuclear ophtalmoplegia. Lesion of the Medial Longitudinal Fasciculus (Connection between CN VI and Medial rectus part of CN III, needed for lateral gaze). Sx: When looking left: Medial rectus palsy at right eye, nystagmus at left eye. Convergence intact!
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Dementia Types
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Alzheimer's > vascular > Lewy body dementia > Pick's disease, CJD, Neurolues, HIV, Vit B12 deficiency, Wilson's disease
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Alzheimer dementia
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Most common cause in elderly. Path.: 10% familial; early onset (APP, presenilin-1, presenilin-2); late onset (ApoE4); ApoE2 is protective. Histology/gross findings: global cortical atrophy; senile plaques (extracellular beta-amyloid depositions); neurofibrillar tangles (intracellular tau-protein depositions)
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Pick's disease
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aka frontotemporal dementia. Sx: Dementia, parkinsonian aspects, aphasia, personality change. Histology/gross findings: frontal atrophy; Pick bodies (intracellular, aggregated tau-protein)
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Lewy body dementia
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alpha-synuclein defect. Sx: Parkinsonism with dementia, hallucinations. Histology/gross findings: Lewy bodies (intracellular, esp. in limbic system, temporal cortex and SN
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MS
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Autoimmune demyelination of CNS. Diverse Sx: e.g. Charcot's triad (Scanning speech, nystagmus, intention tremor); incontinence; internuclear opthalmoplegia. Diag.: Elev. IgG in CSF; Oligoclonal bands; MRI;
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Guillain-Barré syndrome
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aka acute inflammatory demyelinating polyradiculopathy. Path.: autoimmune demyelination of PNS neurons; associated with Campylobacter jejuni or HSV infections (molecular mimicry). Sx: Symmetric ascending weakness in distal legs; 50% facial paresis; autonomic dysfunctions (arrhythmia; blood pressure irregularities). Dx: albuminocytologic dissociation (high protein, low cell count in CSF); high protein -> papilledema. Tx: Plasmapheresis; IV immune globulins; respiratory support
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PML
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aka progressive multifocal leukencephalopathy. Path.: demyelination of CNS neurons; associated with activation of latent JC virus infection in AIDS; Rapidly progressive, usually fatal.
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Acute disseminated (postinfectious) encephalomyelitis
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multifocal inflammation/demyelination after infection (e.g. chichenpox, measles) or vaccinations (e.g. rabies, smallpox
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Metachromatic leukodystrophy
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lysosomal storage disease. autosomal rec. defect of arylsulfatase A -> Buildup of sulfatides -> impaired myelin synthesis.
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Charcot-Marie-Tooth disease
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a type of HMSN (Hereditary Motor and Sensory Neuropathy). Defective production of proteins involved in meylin-sheath buildup.
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Partial seizures
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= synchronized firing in one area of the brain, but can secondarily generalize. 1. Simple partial (consciousness intact); 2. Complex partial (impaired consciousness)
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Generalized seizures
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= global synchronized neuronal firing. 1. Absence (petit mal); 2. Myoclonic; 3. Tonic-clonic (grand mal); 4. Tonic; 5. Atonic
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Epilepsy
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Disorder of recurrent seizures. Febrile seizures are not epilepsy!. Causes: Congenital/genetic; infection; trauma; metabolism; trauma; tumor; stroke
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DD of headaches
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Migraine, tension headache, cluster headache, subarachnoid hemorrhage, meningitis, hydrocephalus, arteritis, neoplasia
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Migraine
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Sx: unilateral pulsating pain with nausea, photophobia, phonophobia, +/- aura. Tx: propranolol, NSAID, sumatriptane
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Cluster headache
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Sx: unilateral periorbital pain, rhinorrhea, lacrimation, Horner's syndrome. Tx: sumatriptane
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Tension headache
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Sx: bilateral headache > 30 Minutes steady pain, w/o further neurological symptoms; no aggracation by light/noise; no aura.
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DD of vertigo
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Periphereal vertigo: Inner ear etiology (semicircular canal debris; CN VIII infection; Ménière's disease) -> delayed horizontal nystagmus in positional testing; Central vertigo: Brain stem or cerebellar lesion -> immediate nystagmus in any direction in positional testing
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Glioblastoma multiforme (°4 astrocytoma)
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Most common primary adult brain tumor. Prog.: GFAP positive; central necrosis and hemorrhage bordered by "Pseudopallisading" pleomorphic tumor cells
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Meningeoma
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2nd most common primary adult brain tumor. Prog.: Resectable. Histology: arachnoid cell origin; spindle cells, psammoma bodies.
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Schwannoma
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3rd most common primary adult brain tumor. Sx: e.g. deafness, vertigo. Location: CN VIII (bilateral in neurofibromatosis type II) Prog.: Resectable. Histology: Schwann cell origin; S-100 positive
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Pituitary adenoma
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4th most common primary adult brain tumor. Most often prolactinoma Sx: bilateral hemianopsia, hormonal dysregulation.
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Pylocytic (low-grade) astrocytoma
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Most common primary brain tumor in children. Prog.: benign. Histology: Astrocyte origin -> GFAP positive; Rosenthal-fibers (eosinophilic, corkscrew fibers).
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Medulloblastoma
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2nd most common primary brain tumor in children. Form of PNET (primitive neuroectodermal tumor). Prog.: Highly malignant, radiosensitive. Histology: Rosettes, blue cells
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Ependymom
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3rd most common primary brain tumor in children. Sx: hydrocephalus (location most often in 4th ventricle) Prog.: poor. Histology: Perivascular pseudorosettes; rod-shaped blepharoblasts
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Hemangioblastoma
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4th most common primary brain tumor in children. Sx: when producing EPO secondary polycythemia. Prog.: Combination with retinal angiomas -> von Hippel-Lindau syndrome.
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Craniopharyngeoma
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Most common supratentorial tumor in children. Sx: bitemporal hemianopsia. Prog.: benign. Histology: Derived from Rathke's pouch remnants; calcifications.
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DD ring-enhancing brain lesions
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metastases, abscesses, toxoplasmosis, AIDS lymphoma
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DD uniformly-enhancing brain lesions
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lymphoma, meningeoma, (metastases)
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DD heterogenously-enhancing brain lesions
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glioblastoma multiforme
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Butorphanol
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partial mu, full kappa agonist -> effective in reducing pain, fewer respiratory depression. AE: causes withdrawal if on full agonists
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1st line status epilepticus (acute, prophylaxis)
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acute: diazepam / lorazepam (increased GABA action; AE: Sedation, tolerance, dependance); prophylaxis: phenytoin (Na channel blocker, IB antiarrhythmic; AE: CYP-450 induction, SLE-like syndrome, teratogenic effects, nystagmus, diplopia, sedation, hirsutismus, gingival hyperplasia, megaloblastic anemia, pseudolymphoma)
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1st line absence
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Ethosuximide (blocks thalamic Ca channels; AE: think EFGH = Ethosuximide, Fatigue, GI-distress, Headache; Urticaria & SJS; I: Only used in absences!)
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1st line tonic-clonic (3)
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Valproic acid = Ergenyl (TM) (AE: rare but fatal hepatotoxicity, weight gain, tremor, GI distress, fetal toxicity -> spina bifida); Carbamazepine (AE: CYP-450 induction, aplastic anemia, agranulocytosis; SJS; liver toxicity; teratogenic; SIADH; also used for partial seizures); Phenytoin. All do increase Na channel inactivation
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Short acting benzodiazepines
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Midazolam (Dormicum (TM) t1/2: 1.5-2.5h); Triazolam (Halcion (TM) t1/2: 2-5h); Oxazepam (Adumbran (TM) t1/2: 5-15h); Alprazolam (t1/2: <12h)
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Ketamine
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dissociative anesthetic. blocks NMDA-R. Cardiovascular stimulant, incr. cerebral blood flow. AE: bad dreams, hallucinations, disorientation
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Propofol
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Potentiates GABA-A effect
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Local anesthetics
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esters: procaine, cocaine, tetracaine; amides (2x I's in name): lidocaine, mepivacaine, bupivacaine. Order of nerve blockade: small, myelinated > small, unmyelinated > large, myelinated > large, unmyelinated. Order of sensation loss: pain > temperature > touch > pressure. I: If allergic to esters, give amides.
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Depolarizing neuromuscular blocking drugs
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Succinylcholine. Phase I - prolonged depolarization (Cholinesterase-inhibitors potentiate block!), phase II - repolarized but blocked (Cholinesterase-I act as antagonists. AE: Hypercalcemia, hyperkalemia
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Non-depolarizing neuromuscular blocking drugs
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Tubocurarine, atracurium, mivacurium, rocuronium, pancuronium, vecuronium. Competitive inhibition of N-ACh-R. Block reversible by Cholinesterase-I (Neostigmin, edrophonium)
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Parkinson's: Dopamine agonists
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Bromocriptin, ropinirole, pramipexole
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Parkinson's: Increase dopamine concentration
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Dopamin-precursor: Levodopa (can cross BBB). Inhibition of periphereal levodopa-breakdown (decarboxylation): carbidopa. Inhibition of L-dopa breakdown (COMT): entacapone Inhibition of dopamin breakdown (MAO-B): selegelin
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Parkinson's: Inhibition of excess ACh-effect
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Benztropin
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Alzheimer drugs
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NMDA-Antagonist: Memantine (inhibits excitotoxicity); ACh-esterase-I: Donepezil, galantamine, rivastigmin
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Huntington's drugs
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Amine-depleting: Reserpine + tetrabenazine Dopamine-R-antagonist: Haloperidol
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Sumatriptane
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5-HT (1B/1D) agonist -> vasoconstriction. I: acute migraine, cluster headache. PK: t1/2 < 2h. AE: vasospasm (CI in CAD, Prinzmetal's angina)
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Ataxia teleangiectasia
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autosomal rec. defect of ATM (Ataxia Teleangiectasia Mutated) gene, involved in DNA repair. Sx: ataxia, teleangiectasia, radiationsensibility, cell-mediated and humeral immunodeficiency (IgA deficiency -> sinopulmonary infections)
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Niemann-Pick disease
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autosomal rec. defect of sphingomyelinase -> accumulation of sphingomyelin and monocytic cells. Sx: hepatosplenomegaly, anemia, motor neuron degeneration with areflexia and hypotonia, "cherry red spot" of macula (DD Tay-Sachs disease
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Opiate withdrawal
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Sx: abdominal pain, nausea, vomiting, diarrhea, agitation, pupillary dilation, diaphoresis, fever
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Anorexia nervosa
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Excessive dieting +/- purging; BW < 85% of ideal weight; commonly coexists with depression.
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Bulimia nervosa
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Binge eating +/- purging; commonly followed by self-induced vomiting, lacatives, diuretics or emetics. Russel's sign: dorsal hand calluses from inducing vomiting.
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opsoclonus-myoclonus syndrome
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Paraneoplastic syndrome in neuroblastoma (Most common extracranial tumor in children. Age onset usuall catecholamine producing
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Vitamin B12 deficiency
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Anemia + neurological symptoms. Diagnostic: increased serum methylmalonic acid.
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Viral meningitis
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Mainly enteroviruses (echovirus, coxsackievierus, poliovirus and enterovirus). Dx: CSF with predominant lymphocytes, leukocytes < 500/µl, glucose slightly reduced, protein elevated but <150mg/dl
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Buspirine
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5-HT1A receptor agonist. I: Generalized anxiety disorder. Minimal hypnotic effects; < abuse potential than benzodiazepines
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Cyproheptadine
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5HT1 & 5HT2 receptor antagonist. I: as antidot in serotonin syndrome (occurs e.g. when SSRI ist combined with MAO-I or high Tryptophane intake)
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Subacute sclerosing panencephalitis
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Rare complication of measles, that remain in the CNS for several years after infection. Inflammation, demyelination and gliosis in many cerebral areas.
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Pickwickian syndrome
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Obesity hypoventilation syndrome [OHS]. Because of obesity expansion of thorax and lungs is difficult -> increased respiratory work -> decreased respiratory drive -> chronic underventilation with high PaCO2 and low PaO2.
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Beta-endorphin
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is derived from POMC [proopiomelanocortin]. POMC (via enzymatic cleavage) -> Beta-endorphin, ACTH, MSH
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Motion sickness (receptors, medications)
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H1 and M1 muscarinic-R -> nausea, vomiting; medications: H1 - receptor blocker (Meclizine, Dimenhydrinate; both with additional antimuscarinic, antiserotonergic and antiadrenergic properties); M1 - receptor blockers: scopolamin. AE: dry mouth, blurry vision, palpitations, constipation, urinary retention
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Postpartum mood disturbances
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2-10d postpartum: Maternity blues (80% self-limiting) 2w - 12m: Postpartum depression (Antidepressants) variable: Postpartum psychosis (Antipsychotics, Antidepr.)
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Cerebral amyloid angiopathy
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Amyloid depositions in cerebrall arterial walls due to age (not systemic amyloidosis). Sx: recurrent small hemorrhages of the cerebral hemispheres (vs. basal ganglia in hypertensive encephalopathy and Charcot-Bouchard aneurysm)
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Myotonic dystrophy
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aut. dom. CTG repeat in myotonia-protein kinase gene. Sx: abnormally slow relaxation (difficulty loosening one's grip), atrophy (Type I > II fibers), cataracts, frontal balding, hypogonadism.
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pentazocine
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partial opioid receptor agonist. antagonist for mu-receptors. I: pain. AE: withdrawal when administered to patient with long term opioid intake.
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Trigeminal neuralgia
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Ethiology unclear. Sx: sudden pain in trigeminal are induced by stimuly to CN V (tooth brushing, eating). Tx: Carbamazepine
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First line treatments epilepsy forms
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Only absence: Ethosuximide (Ca-blocker); only tonic-clonic : Phenytoin, Carbamazepine (Na-blocker); both absence and tonic-clonic: Valproic acid (Na-blocker, incr. GABA conc.); status: benzodiazepines, phenytoin
