question

orbital pain after exercise+ horner syndrome-->?

answer

carotid a. dissection

question

after Bell's palsy, every blink of eye will associated with twitching at the corner of mouth , why?

answer

Aberrant regeneration of VII n. (異常的) happens only if nerve cell bodies survive the injury and produce axons that find their way to neuromuscular junctions.

question

sudden onset severe headache-->subarachnoid hemorrhage--> do CT, normal--> what is next step?

answer

LP 10% case will show normal CT, should do LP to exclude. 1.elevated RBC 2.xanthochromia 3.increased opening pressure (60-110mmH2O)

question

suspect subarachnoid hemorrhage, if CT,CSF normal-->next step?

answer

do cerebral angiogram and call a neurosurgical consult.(it may be an aneurysm,not ruptured)

question

CT: what is hyperdense with or without contrast?

answer

with contrast: highly vascular lesion without contrast: calcification

question

EEG sleep frequency

answer

delta 13

question

hyperostosis of skull

answer

meningioma

question

pineal tumor account for 1% of brain tumor' 1/3 is germ cell tumours , including?

answer

germinoma choriocarcinoma

question

iv prochlorperazine

answer

for status migrainosus

question

metastatic brain tumor

answer

lung> skin>kidney>breast>colon by: 1. blood stream 2. Batson's plexus

question

which metastatic cancer has shortest life expectancy?

answer

1. malignant melanoma (cause intracranial hemorrhage) 2. choriocarcinoma (bleeding too)

question

gelastic seizure (歇斯底里的笑)

answer

seizure preceded by episodes of uncontrollable laughter seen in Hypothalamic hamartoma

question

區別: 1. hypothalamic hamartoma 2.craniopharyngioma

answer

hypothalamic hamartoma 1. neuroendocrine function( precocious puberty, acromegaly, GHRH) 2.gelastic seizure: paroxysms of laughter craniopharyngioma 1. hypopituitatism 2.visial field disturbance

question

Parinaud syndrome

answer

loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergency-retraction nystagmus, in which the eye appear to jerk back into the orbit on attempted up gaze

question

Parinaud syndrome lesion? associated disease

answer

dorsal midbrain in the region of the superior colliculus 1. pineocytoma (benign) 2.germinoma 3.teratoma,glioma 4.malignant pineal tumor: pineoblastoma

question

paraproteinemic neuropathy

answer

multiple myeloma

question

which is for controlling seizure right now?

answer

Phenytoin (immediately) lamotrigine ( must be slowly titrated over many weeks when first started because the risk of severe rash)

question

iv drug user, HIV, CD4 normal, CT show hemorrhagic lesion, or with contrast see other lesion in another location EEG: focal slowing over the left parietal lobe aortic valve disease CSF: xanthochromic Dx?

answer

mycotic aneurysm (arterial wall injury in the CNS) sex intercourse may lead to rupture of the aneurysms

question

mycotic aneurysm

answer

low virulence organism->subacute bacterial endocarditis->infected embolus originating on the diseased valves lodging in arterial wall of CNS->mycotic aneurysm. multifocal High virulent organism->meningitis

question

MCC of lobar haemorrhage in elderly patients without HTN

answer

CAA cerebral amyloid angiopathy 1. >70y, with or without dementia 2. multiple cortical hemorrhage 3. additional hemorrhage may be seen only on special techniques: Gradient echo MRI (magnifies the effects of hemosiderin in regions of prior haemorrhage )

question

nucleus ambiguus

answer

ventrolateral medulla CN IX, X hoarseness and dysphagia

question

wallenberg =lateral medullary syndrome

answer

1. V-> 同側facial pain,T,corneal reflex 2.nuleus ambiguus: vagus, glossopharyngeal n. ->dysphagia, dysphonia 3.spinothalamic tract->對側pain,T on limbs and trunk 4.inferior cerebellar peduncle->同側ataxia 5.decending sympathetic fibers->同側horner syn

question

Dejerine-Roussy syndrome

answer

= thalamic pain syndrome=paradoxical pain condition developed after a thalamic stroke,Weeks to months later, numbness can develop into dysaesthesia or allodynia. Tx: analgesics:opiates, anti-depressants,anti-convulsants

question

Allodynia

answer

Allodynia is pain from a stimulus that would normally not cause pain

question

Dysaesthesia

answer

Dysaesthesia is defined as pain due to thalamic lesioning. This form of neuropathic pain can be any combination of itching, tingling, burning, or searing experienced spontaneously or from stimuli

question

todd paralysis

answer

weakness after seizure may due to glucose deplete

question

post truamatic seizure (25%patient after head truama) MC: generalized convlsions

answer

early: 1week Tx: antiepileptic drug is good for early epilepsy, not reduce the late epilepsy

question

sturge-weber syndrome (encephalofacial angiomatosis)

answer

1. facial cutaneous angioma(port wine nevus): over the sensory distribution of the first division of the CN V 2.leptomeningeal angioma 3. hemiparesis or hemiatrophy on the opposite side of port wine nevus 4.mental retardation

question

charcot bouchard aneurysms

answer

perforating a. of brain in chronic HTN patients

question

transient attacks of amaurosis fugax

answer

monocular blindness in carotid a. disease embolism of the central retinal a. or one of its branches !Retinal vein thrombosis: 1.rapidly progressive loss of vision 2.hemorrhages in retina 3. no transient attack of amaurosis fugax

question

lung cancer + muscle weakness

answer

1.LEMD 2.dermatomyositis (paraneoplastic syndrome. when >40y, 40% in F, 60% in man)

question

Duchenne dystrophy carrier, CK? mental retardation?

answer

1.may be increase may also have abnormalities in limb girdle muscle on biopsy 2. normal for carrier, slightly decreased in affected male

question

Duchenne incidency?

answer

1 in 3,000 male infant other: spontaneous mutation rate for the gene is presumed to be high for what reason? men with duchenne dystrophy do not reproductive (all the mutation comes from spontaneous mutation in ovaries in women)

question

myotonic dystrophy

answer

1. can not relaxing grip, hypersomnolence, premature baldness, testicular atrophy, cataracts 2.EMG: dive bomber pattern (sound stimulus) repetitive discharges with minor stimulation Tx: pacemaker implantation to avoid sudden death

question

polymyogia rhumatica

answer

elevated ESR, anemia, weight loss, malaise, >60y normal CK

question

EMG of ALS?

answer

fibrillations, normal conduction time, abnormal pattern

question

denervation atrophy looks like?

answer

small muscle fibers intermingled with hypertrophied fibers. mosaic of muscle fiber types, no inflammatory infiltrate.

question

signs indicate poor survival beyond 1 year in ALS?

answer

brainstem disease: 1. diaphragmatic weakness 2.facial fasiculations

question

1. lhermitte sign 2. uhthoff's sign

answer

MS: 1. electrical sensation radiating down the spine when the neck is passively flexed 2. heat sensitivity, get weaker when in hot water or after exercise.

question

MS ,IgG: k-light chain

answer

IgG elevated enen between acute exacerbations total CSF protein is not elevated very much

question

MS patient, use cortical steroids, need to think about adding which drug?

answer

PPI or ranitidine to prevent steroids induced ulcer

question

canavan disease

answer

1.infant regression at about 6month 2.extensor posturing and rigidity 3.defect in N acetylspartic acid metabolism 4.widespread white matter change->spongiform appearance 5.macrocephaly(increase brain volume and weight)

question

Neuromyelitis optica

answer

1. bilateral potic neuritis 2. transverse myelitis: (inflammatory demyelinating lesion that transects much of the spinal cord, may have necrotizing process) paraparesis, bladder and bowel dysfunction, sensorydeficit

question

Leber optic atrophy

answer

1.centrocecal scotoma: blind spot extends into central vision 2. mutaion in mitochondria DNA 區別:MS:很少會只有中間那一點blind spot

question

Adrenoleukodystrophy

answer

1. adrenal dysfunction 2.progressive degenerative disease of the white matter 3.X-linkedd, survival of 3 ys. 4.機制:defect of ATP binding transporter in peroxisomal system responsible for ling chain fatty acid metabolism->LCFA accumulate in adrenal cortical and other cells.

question

Adrenomyeloneuropathy

answer

1.damage to the spinal cord and peripheral N. also brain. 2.routinely develop spastic paraparesis 3.may also develop in heterozygous women

question

Pelizaeus-Merzbacher disease = sudanophilic leukodystrophies (autopsy stain)

answer

1. demyelinating disorder 2. symptoms during first month of life, survival to third decade 3. male

question

HIV and CMV brain change 共同點

answer

microglial nodules 1.HIV: around blood vessel throughout the brain 2. CMV: subpial and subependymal

question

amebic meningoencephalopathy

answer

from fresh water swimming

question

MCC of encephalitis

answer

virus: HSV MCC of meningitis: bacterial

question

JC virus =PML CJ disease =prion J=jakob C=creutzfeldt

answer

Tx for JC virus in HIV patient: HAART therapy transmitted by respiratory secretions.

question

rim enhanced brain lesion in AIDS

answer

1. PCNSL primary CNS lymphoma: check CSF EBV PCR test is highly sensitive 2. Toxoplasma gondii 3. absecss: bacterial or fungal

question

recurrent meningitis

answer

CSF leak (head truam) 1. through nose: rhinorrhea 2. through ear: otorrhea

question

brain polycystic lesion (large cyst contains numerous daughter cyst)

answer

echinococcus granulosa 區別: teania solium (lava form):neurocysticercosis.

question

fluctuating but slowly progressive bilateral lower limbs weakness where is the ova of parasite deposit?

answer

spinal cord S. manosni: deposits eggs in the valveless veins of Batson->granuloma around the ova lodge in the spinal cord.

question

sarcoid lymphadenopathy involve which CN?

answer

VII half facial muscle weakness without sensation loss

question

economo's encephalitis =encephalitis lethargica

answer

1. cause: viral influnza infection 2.transient fever, lethargy, headache 3. disturbed eye movement, chora, athetosis, dystonia, myoclonus 4. MCC sequela: unremitting Parkinsonism 5.oculogyric crises: episode of eye deviate to one side or upward

question

B. burgdorferi (lyme disease) Tx

answer

iv high dose penicillin or ceftriaxone 10-14day if allergic to iv treatment: tetracycline qid for 30day

question

aquired CJD 傳播途徑

answer

1. growth hormone treatment:GH preparation from cadaver pituitary 2.dura mater graft 3.Kuru: eating human brain tissue

question

Rhombencephalitis = brainstem encephalitis. 1.infectious 2.autoimmune:Behçet disease 3.Paraneoplastic:anti-neuronal antibodies empiricTx: (ampicillin+gentamycine) and (acyclovir)

answer

1#: Listeria: -flu-like syndrome followed by brainstem dysfunction.abnormal brain MRI scan -CSF pleocytosis 2#:Enterovirus 71 :Asian-Pacific region 3#:HSV 4#:EBV,HHV6

question

Triphasic wave=periodic sharp wave

answer

1. JCD 2. hepatic encephalitis

question

MC symptoms of brain abscess

answer

1. headache: 75% 2.seizure or focal deficit:30% 3.papilledema: 25%

question

AIDS: MCC of brain abscess

answer

1. toxoplasma gondii 2.fungi: Aspergillus,cryptococcus, candida, mucor,

question

AIDS: MCC of fungal malignant external otitis

answer

Aspergillus

question

JC virus pathology? MRI?

answer

1. inclusion bodies in the oligodendrocte nuclei 2.multiple focal well defined whiter matter lesions that do not enhance or have mass effect.

question

miller fisher variant (Guillain barre syn)

answer

triad : ophthalmoplegia, ataxia, and areflexia Anti-GQ1b antibodies CSF dissociation (increase protein,normal WBC)

question

SSPE subacute sclerosing panencephalitis

answer

1. children have measles before 2years old. 2.6-8y develop SSPE, 1-3y die. 3. CSF: similar to MS: gamma globulin fraction high 4. eosinophilic inclusion seen in cytoplasma and nuclei of neurons and glial cell

question

Pick disease lewy body disease

answer

Pick: 1.frontal-tempral dementia 2.personality change Lewy: 1.fluctuating cognitive impairment 2. visual hallucination 3. parkinsonism is seen, but poor response to dopaminergic agonist drug

question

infantal seizure

answer

1.lennox gastaut syndrome 2.landau-kleffner syndrome 3.juvenile myoclonic epilepsy 4.febrile seizure

question

lennox gastaut syndrome

answer

1. mental dysfunction 2. multiple seizure types,1-2Hz general lized spike wave discharge 3. with history of Infantile spasms( west syndrome): paroxysmal flexion of the body, waist or nexk and have profoundly disorganized EEG pattern called hypsarrhythmia

question

landau-kleffner syndrome

answer

1. loss of language function 2.abnormal EEG during sleep

question

juvenile myoclonic epilepsy=myoclonic seizure

answer

relatively benign epilepsy with onset in late adolescence or early adulthood may happen when person wakes up

question

alcohol withdraw

answer

1-2day: seizure 2-4 day: delirium tremens

question

clinically Dx simple partial seizure (notice one hand shaking, and then loss control of movement of that arm) next step?

answer

Do brain MRI because after clinical Dx, no need to do EEG, you need to find out the etiology of the focal change. dont discharge unless do the CT or MRI

question

Drug for seizure: dose and rout and side effect when injection to fast

answer

1.phenytoin:iv <50mg/min adult or 1mg/kg-min children it takes 20min to iv 1000mg for status epilepticus too fast cause cardiac arrhythmia, hypotension 2.Fosphenytoin: given up to 150mg/min, can intramuscular, less side effect 3.carbamazepin: no iv at all 4. phenobarbital:hypotension, respiratory arrest 5.BZ: hypotension diazepam:rapid onset,rapid clearance, need other drug for maintain lorazrpam: rapid onset, slow clearance.

question

MCC of intractable complex partial seizure in adults

answer

mesial temporal sclerosis MTS (MRI: sclerotic hippocampus) Tx: surgical resection of the temporal lobe (medication is poor) but may lead to superior auadrantanopsia

question

jacksonian march = sequential seizure

answer

patient develop focal seizure that is primarily motor and spreads (finger to forearm to face)

question

levetiracetam

answer

complex partial seizure

question

west syndrome Tx (infantel spasm)

answer

ACTH hormone

question

drug: Divalproex ethosuximide

answer

for absent seizure Divaplroex better: less effect, also good for absent with generalized tonic clonic seizure

question

classic migraine common migraine

answer

1. common migraine: Migraine without aura, 2. classic migraine:Migraine with aura with accompanying motor weakness(first 2 type) - familial hemiplegic migraine - sporadic hemiplegic migraine - basilar-type migraine:headache and aura (may complete blindness )are accompanied by difficulty speaking, world spinning, ringing in ears, or a number of other brainstem-related symptoms, but NO motor weakness

question

symptomatic trigeminal neuralgia cause: CN V compression

answer

1.MS 2.basilar a. aneurysms 3.acoustic schwannoma 4. post. fossa meningioma

question

tolosa-hunt syndrome 位置: superior orbital fissure or the cavernous sinus pathology

answer

1. inflammatory disorder 2.ophthalmoplegia + headache 3. loss sensation over forhead 4. pupillary function is normal

question

區別: atypical facial pain trigeminal neuralgia

answer

facial pain: constant, deep pain, bilateral or unilateral

question

thunderclap headache

answer

worst headache 1.CT: exclude subarachnoid hemorrhage 5%false negative 2.LP: make sure 3.if normal: maybe sudden change in wall of aneurysm provoke the pain (after intercourse) 4.do angiogram to confirm 5.if develop to migraine,then the pain is simply the initial presentation.

question

best treatment for spinal cord injury

answer

iv methylprednisolone 30mg/kg, followed by 5.4mg/kg-hr for 23hrs 其他都沒太多效果或是已經證明 (surgery decompression, correction bone displacement or removal of hemorrhage)

question

MC place in head trauma

answer

1# Coup injury: temporal lobe and inferior frontal lobes. -uncal herniation, memory, language. -wenicke area is sufficiently posterior to tmporal lobe to escape injury # Contrecoup injury: occipital lobe ( rebound movement)

question

complication of acoustic trauma (working in noisy place, hearing loss)

answer

timmitus

question

claw hand

answer

ulnar n. damage -ulnar lumbrical m. weakness, the extensor sheaths of the digits are not properly positioned.

question

n.damage, leave patient with easily provoked pain

answer

causalgia 灼痛 a disturbance in sensory perception characterized by hypersthesia, dysesthesia, and allodynia.

question

Friedreich disease 位置

answer

primarily in spinal cord ( spinocerebellar tract, dorsal column, corticospinal tract)

question

MCC of mono neuropathy multiplex

answer

1# DM other: rheumatoid disease, sarcoidosis

question

toxin 1.lead: wristdrop, (painless neuropathy, radial n.) 2.mercury: gait ataxia, "mad hatter", irritability, tremor, paresthesia (sensory neuropath) 3.cyanide: optic neuropathy 4.thallium: hair loss, stupor, GI distress, seizure, headache, painfull symmetric sensory neuropathy. 5.manganese:(inhalation by miners, similar to wilson disease)parkinsoinism

answer

6.Arsenic: white line on nails. tonic clonic seizure, hemolysis, circulatory collapse, mucosa irritation, polyneuropathy 7.ergot:potnet vasoconstriction, from rye fungus. degeneration of the posterior columns and dorsal roots(sensory neuropathy, ataxia, seizure, fasciculation) 8. ciguatoxin:eat grouper魚, reef fish. peculiar sensory phenomenon of T reversal 冷的東西覺得很燙

question

lyme neuropathy = noncompressive siatica (case: meningitis and facial weakness, not respond to rifampin, isoniazid and steroids, later develop pain radiating down the back of leg and cant dorsiflexing foot)

answer

- irritation of spinal roots to the sciatic nerve produce the foot drop that may occure with lyme radiculopathy -also painful radiculopathy and peripheral neuropathy 區別: TB: response to steroids

question

friedreich ataxia (spinocerebellar degeneration) 1.AR, chromosome 9, frataxin mutation 2.progressive damage to the nervous system 3.Symp typically begin at ages of 5 to15, Late Onset FA may occur in the 20s or 30 4.Symptoms 後面隨意組合

answer

1.Muscle weakness in the arms and legs 2. Loss of coordination 3. Vision impairment, Hearing impairment 4. Slurred speech 5. Curvature of the spine (scoliosis) 6. High plantar arches (pes cavus deformity of the foot) 7. Diabetes (about 20% of people with Friedreich's ataxia develop carbohydrate intolerance and 10% develop diabetes mellitus) 8.Heart disorders (e.g., AF, tachycardia and hypertrophic cardiomyopathy) 9.peripheral neuropathy: loss of cells in the dorsal root ganglia, substantia gelatinosa(lissauer tract) of posterior horn and dorsal roots themselves

question

parsonage turner syndrome (brachial plexopathy=brachial neuritis=neuralgic amyotophy)

answer

1. acute onset of pain in the nexk, shoulder, or upper arm. sensory loss is also occours 2. In familial case: AD

question

Riley-day disease = familial dysautonomia

answer

1.AR, Jewish children 2. sympathetic and parasympathetic ganglia affected, also small fiber neuropathy 3.Sym:loss of tear, corneal ulceration, absence pupillary reactivity, poor T regulation, excessive perspiration出汗, orthostatic hypotension vomiting, GI dilation. absence of papillae of tongue.

question

Tx acute intermittent porphyria

answer

iv hematin

question

Thiamine or B1 deficiency

answer

painful sensory neuropathy

question

nitrous oxide intoxication (anesthetic agents) similar to B12 deficiency: interference with B12 dependent conversio of homocysteine to methionine

answer

1. megaloblastic anemia 2.peripheral neuropathy: numbness, paresthesia, limb spasticity ,ataxia, also central n.damage

question

parkinson high frequency stimulation place

answer

1. globus pallidus: inhibit thalamus 2.subthalamic nucleus:stimulate globus pallidus 3.thalamus: decrease over inhibition

question

MPTP side effect 1-methyl-4-phenyl-1236-tetrahydropytidine

answer

progressive damage to the substantia nigra --> mimic parkinson disase

question

levo dopa--> unmask huntington symptoms ( writhing and jerking movement of limbs)

answer

antipshychotic -->suppress huntington symptoms

question

influnza virus-->encephalitis lethargica also cause?

answer

postencephalitic parkinsonism

question

chorea gravidarum

answer

1.involuntary movement disorder that occurs during pregnancy (or taking estrogen) 2.relatively rapid and luid, but not rhythmic, limb and trunk movement 比較: huntington: progressive, uniformly fataal hereditary disease

question

tourette syn Tx (AD)

answer

haloperidol pimozide trifluoperazine, fluphenazine

question

parkinson biopsy

answer

lewy body : intracytoplasmic inclusion

question

Meige syndrome

answer

1.a form of focal dystonia 2.blepharospasm臉痙攣, forceful jaw opening lip retraction, neck contractions, tongue thrusting伸 3. Tx: Botulinum toxin

question

toxin: TOCP (triorthocresyl phosphate)

answer

1.來源: rat poison, roach powder, insecticides 2.damage: both upper and lover motor neurons, lead to severe motor polyneropathy 3.cause: headache, vomiting, ab cramps, sweating ,wheezing, twitching

question

toxin: Lead

answer

1.low dose: ataxia, tremor 2.chronic: psychomotor impairment, retardation 3.infant: brain edema, herniation

question

drug cause parkinsonism

answer

phenothiazine butyrophenone metoclopramide

question

botulism Dx

answer

EMG: decremental response of muscle to repetitive stimulation of the nerve at a low frequency(2-5Hz) and incremental response to repetitive stimulation at high frequency(20-50Hz)

question

區別: ADEM acute disseminated encephalomyelitis 與 MS

answer

ADEM: monophasic (childhood exanthemas疹 are likely to precipitate ADEM: smallpox and rabies immunizatiotn)

question

congenital horner syndrome

answer

heterochromia iridis -failure of one eye to develop normal iris color. because sympathetic innervatio of the iris is required for the change in the color of iris to occur after birth and infancy

question

GCS

answer

14-15 mild injury 9-13 moderate injury <=8 severe injury

question

basilar skull fracture

answer

racoon eye: periorbital ecchymosis battle sign: ecchymosis over the mastoid region hemotypanum:blood behind eardrum CSF rhinorrhea or otorrhea

question

MCC of coma in head injured patient without an intracranial mass lesion

answer

diffuse axonal injury 1. diffusely spread axonal swellin affecting the white matter, corpus callosum and upper brainstem 2. the goci are usually hemorrhagic

question

recommended to improve the outcome of traumatic head injury

answer

hypothermia ( packing the body in ice or using iv cooling system)

question

risk of develop MS after develop optic neuritis

answer

women: 75% men: 34% after 15years

question

Drug acyclovir

answer

1. for shingle: oral 2.for herpes encephalopathy: iv 2-3week

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