MKSAP 16 Neurology – Flashcards

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most common aura in migraine
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flashing light or enlarging blind spot rimmed with shimmering edge or jagged lines in peripheral vision
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how is a "typical" aura defined?
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any combination of visual, hemisensory or language abnormalities developing over >5 mins and lasting max of 60 mins
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when should auras be investigated (in migraines)
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>60mins, involve any focal motor weakness, display brainstem symptoms
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two types of migraines
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episodic migraine (<15d/month) or chronic (15 least 15d/month x more than 3 months)
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risk factors for transformation of episodic to chronic migraine
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age, stress, female sex, low educ/socioeconomic status, head/neck trauma, obesity, comorbid pain and sleep or psych disorders, high caffeine or nicotine intake, overtreatment with acute headache meds
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overtreatment with acute headache medication resuls in
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medication overuse headache
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acute headache meds that lead to chronic migraine; and their duration of use
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opiates (8d/mo), barbiturates (5d/month) and NSAIDs and triptans (10-14d/month)
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treatments for acute migraine that are evidence-based
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NSAIDs, dihydroergotamine, triptans
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contraindications to triptans
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vasospastic or ischemic vascular disorders, uncontrolled HTN, hemiplegia or basilar migraine subtypes
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goal of migraine treatment
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cmoplete resolution within 1-2 hours and return to normal function within 24 hours
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define status migrainosus
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single migraine attack extending >72 hours
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the cornerstone of inpatient management of refractory migraine
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IV dihydroergotamine is extremely effective for patients who have not take triptans or ergots within 24 hours
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in-patient meds for migraine
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IV ketorolac, sodium valproic acid; dexa lowers recurrence; IV dopamine agonists with IV diphenhydramine
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six situations where migraine prophylaxis is indicated
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>2d/wk or 8d/month; use of acute meds >2d/wk; disabling symptoms despite intervention; prolonged (>1h) or complex aura; C/I to acute therapies, patient preference
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major classes of agents available for migraine prevention
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NSAIDS, B blockers, calcium blockers, antidepressants, anticonvulsants, serotonin receptor antagonists
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agents with strongest evidence for prevention of episodic migraines
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propranolol, timolol, amitriptyline, divalproex sodium, topiramate
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only 2 agents for chronic migraine
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topiramate PO and injectable onabotulinumtoxinA
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contraindications to topiramate
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kidney stones
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how long should you give prophylactic agents in migraine?
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6-12month maintenance phase after response (50% reduction in headache frequency); followed by a tapering phase
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define menstrually-related migraine vs. pure menstrual migraines
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menstrually-related migraines occur during (D2-3) and outside menses; pure menstrual migraine occurs exclusively during the menstrual period
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treatment for pregnant woman with migraine
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acetaminophen, metoclopramide, certain opiates and NSAIDs; avoid NSAIDs after week 32; other agents include prednisone; triptans are category C use only when benefit outweighs risks
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duration to consider generalized convulsive status epilepticus
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lasting longer than 30 minutes
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first line of management for status epilepticus
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IV ativan ffd by phenytoin
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why is fosphenytoin favored over phenytoin?
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adminstered faster and does not carry risk of thrombophlebitis or skin necrosis associated with phenytoin extravasation
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all immunocompromised patients with first seizure episode should have these two tests
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MRI and lumbar puncture
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why ask about the "aura" in seizure history?
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provides a clue to the cortical origin of the seizure
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seizure that involves the cortex diffusely
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generalized seizure
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seizure that has its onset in a limited region of the cortex
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partial seizure
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focal seizure that does not impair awareness
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simple partial seizure
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a focal seizure that involves alteration of consciouness
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complex partial seizure
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a simple partial motor seizure characterized by thythmic contraction of a group of muscles (fingers) ffd by spread to arm and then ipsilateral face
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classic jacksonian march
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simple partial seizures which cause purely subjective symptoms
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epileptic auras
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limb weakness contralateral to the site of seizure onset
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Todd paralysis
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causes of acute ataxia
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intoxicant, benzodiazepine, phenytoin or vascular event (cerebellar or brainstem hemorrhage or ischemic stroke)
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causes of subacute ataxia
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paraneoplastic syndrome (s.a. paraneoplastic cerebellar degeneration) or prion disease
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causes of chronic ataxia
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cerebellar degeneration
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most common adult-onset progressive ataxia syndrome
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multiple system atrophy cerebellar type (olivopontocerebellar atrophy) and spinocerebellar atrophy disordres including Machado-Joseph disease (spinocerebellar ataxia tyupe 3)
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drugs that cause or exacerbate tremor
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beta agonists, stimulants, cyclosporine, IFN, valproic acid, lithium, steroids, serotonin reuptake inhibitors
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treatment of essential tremor
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propranolol and primidone (oldest and most effective), gabapentin, methazolamide, topiramate
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treatment of medication-refractory essential tremor
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deep brain stimulation of ventral intermediate thalamic nucleus (VITN)
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tremor + ataxia, dysarthria, parkinsonism, dementia
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consider Fragile X tremor ataxia syndrome
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dystonia always involves this brain structure
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basal ganglia
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treatment of focal dystonia
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botulinum A or B injections
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first line drugs for treatment of generalized dystonia
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high-dose anticholinergic agents and baclofen
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treatment of tardive dystonia
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may respond to dopamine-depleting agents reserpine and tetrabenzine
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treatment of refractory dystonia
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bilateral continuous electrical stimulation of globus pallidus internal nuclei
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what is mild cognitive impairment
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loss of cognitive ability greater than expected by age, but does not interfere significantly with ADLs
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risk factors for progression of MCI to dementia
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family history of Alzheimer's, memory deficit >1.5 SD below normal; apolipoprotein E4 allele, reduced hippocampal volumes on neuroimaging
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tests for evaluation of cognitive function
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Folstein MMSE, Montreal cognitive assessment
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What score indicates dementia on the screening tests?
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less than 22
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differentials for rapidly progressive dementia (weeks to months)
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suggests a paraneoplastic disorder such as limbic encephalitis or prion disease s.a. CJD
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tests that can be ordered (judiciously) as part of work-up for dementia
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CBC, CT head, MRI brain, ESR TFTs B12 VDRL
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when is LP indicated in the evaluation of dementia?
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rapid progression, age <60y; systemic cancer, infection or autoimmune disease; immunosuppressed, (+) syphilis serology
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most definitive diagnostic test for dementia
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brain biopsy
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mutation in this gene for tau protein metabolism accounts for a fraction of Alzheimer's dementia
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presenilin
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the key pathologic agent involved in Alzheimer's disease
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beta-amyloid protein
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What is Capgras syndrome?
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"family members are imposters"(misidentification delusion in Alzheimer's)
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diagnosis of Alzheimer's disease
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no biologic markers exist; diagnosis depends on exclusion of other causes of dementia
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pathologic features of Alzheimer's brain (gross and microscopic)
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grossly, diffuse brain atrophy, widening of sulci and enlargement of ventricles; histologically, "tangles + amyloid + degenerating neurson", neurofibrillary tangles, amyloid-containing neuritic plaques and granulovacuolar degeneration of neurons
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most severely affected brain regions in Alzheimer's dementia
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temperal lobe, hippocampus, Ach-containing basal nucleus of Meynert
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name the Alzheimer's disease medications
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Gallant Don Rivas Tacker (NMDA) - galantamine, donepezil, rivastigmine, tacrine, memantine
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MOA of alzheimer's meds
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acetylcholinesterase inhibitor; except for memantine which is an NMDA inhibitor; galantamine is also a nicotinic receptor agonist
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these agents may reduce the rates of institutionalization in patients with Alzheimer's disease
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selegiline and Vitamin E
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second most common cause of dementia
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dementia with Lewy bodies
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pathologic hallmark of dementia with Lewy bodies
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widespread presence of Lewy bodies (eosinophilic intracytoplasmic accumulations of alpha-synuclein reveiled by ubiquitin and alpha-synuclein immunohistochemical staining techniques)
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treatment for Lewy body dementia
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same as for Alzheimer's dementia; levodopa to treat motor symptoms (often develop parkinsonism)
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which antipsychotic agent should be used with Lewy body dementia?
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agents that do not block dopamine receptors, s.a. clozapine
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What is the cause of frontotemporal dementia?
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mutations in tau protein --> become hyperphosphorylated --> aggregates in neurons or glia
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original name of frontotemperal dementia
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Pick disease (mutation in tau protein on Chromosome 17)
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triad of normal pressure hydrocephalus
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DUMB WALKER URINATES = cognitive decline, gait abnormalities, urinary incontinence in the setting of normal CSF pressure (usually <150mmH20)
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first diagnostic step in patient with suspected NPH
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MRI brain - ventricles expand without sulcal atrophy or obstruction
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best predictor for shunt-responsive hydrocephalus
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clinical improvement in gait after continuous lumbar drainage procedure over 2-3 days at a rate of 10ml/hr
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treatment of NPH
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shunt placement with programmable valve
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signs and symptoms of hemorrhagic stroke
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HA, vomiting, diastolic >110; meningisimus, seizures at onset, coma
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this study can reveal hyperintensity within minutes of acute cerebral infarction
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diffusion-weighted MRI
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gold standard for evaluating cerebral vasculature
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catheter-based angiography
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other tests to evaluate cerebral vasculature that is less invasive
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MRA without contrast; CT angio, doppler-flow ultrasonography
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new definition of TIA as per AHA/American Stroke Association?
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transient neurologic deficit resulting from focal brain, SC or retinal ischemia without acute infarction
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Which TIA patients get admitted?
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ABCD2 score identifies TIA patients with highest risk of subsequent stroke - all within 72 hours of initial symptoms with score >/= 3 should be admitted (transient monocular blindness should also be admitted)
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ABCD2 stands for
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age >/=60, BP>140/90, clinical presentation, duration of symptoms and presence of DM
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ICA disease and race - is there a connection?
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ICA disease most common in Europeans; intracranial atherosclerosis most common in Asians and Africans
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causes of lacunar infarcts
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LIPOHYALINOSIS of small penetrating arteries that feed off the MCA, ACA, PCA and vertebrobasilar arteries
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two types of intracerebral hemorrhage syndromes
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DEEP hemorrhage (basal ganglia or cerebellum) likely due to HTN and LOBAR hemorrhage likely due to cerebral amyloid angiopathy
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important caveat about diagnostic imaging of intracerebral hemorrhages
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repeat imaging study 4-6 weeks after initial event because hemorrhage may obscure an underlying tumor
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MOA of tPA
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converts plasminogen to plasmin in the presence of fibrin to initiate fibrinolysis
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time frame for endovascular clot removal
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within 8 hours
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BP goal for stroke patients undergoing tPA
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185/110
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antihypertensive agent of choice for stroke patients undergoing tPA
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labetalol or nicardipine (avoid NTG or nitroprusside - lowers BP excessively)
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after rtPA infusion, BP target?
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<180/105
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BP goal for stroke patients not undergoing tPA
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permit BP up to 220/120
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BP target in ICH
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160/90 or MAP of 110
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when should ICH be removed surgically?
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cerebellar ICH >3cm in size, or with neurologic deterioration or superficial hemorrhage >30ml
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characteristic neurologic syndrome in cavernous sinus thrombosis
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lesions of CN 3, 4, 5 (V1 and V2) and sometimes 6
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CT scan finding in dural sinus venous thrombosis
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CT shows filling defects in the dural sinuses "empty delta sign" at the confluence
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treatment of dural sinus venous thrombosis
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not established, heparin ffd by warfarin x 6 mos even in the setting of ICH
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HA + neck pain --> partial Horner syndrome (ptosis, miosis)
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cervicocephalic dissection
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Imaging of choice for cervicocephalic dissection
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MRI of soft tissues of neck; MRA and CTA will only show narrowing.
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incidental finding of intracerebral aneurysm - when is an aneurysm at risk for rupture?
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anterior circulation >12 mm; post circulation >7 mm
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the only nonsurgical intervention known to reduce mortality in stroke
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admission to a stroke unit
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when should carotid revascularization be considered in ICA atherosclerosis
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symptomatic extracranial ICA stenosis >70%
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risks of angioplasty and stenting vs carotid endarterectomy
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ANGIO you get stroke, ENDARTERECTOMY you get MI = angioplasty + stenting higher risk of perioperative stroke; carotid endarterectomy has higher risk of perioperative MI
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when should anticoagulation be started after hemorrhagic stroke
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start by day 1 to 4 as long as source of bleeding identified and treated or there is no evidence of active intracranial bleeding
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s/p cardiothoracic surgery, patient develops stroke; most common cause of perioperative stroke?
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post-operative AF
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trauma-induced alteration in mental status that is sometimes associated with a loss of consciousness
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concussion
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2 hallmarks of concussion
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Confusion and amnesia
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AAN Grade 1 concussion
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no LOC, but only momentary confusion or changes in mental status
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AAN Grade 2 concussion
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no LOC, but with symptoms or signs of concussion or changes in MS > 15 minutes
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AAN Grade 3 concussion
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Any loss of consciousness, either brief (seconds) or prolonged
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return to play with multiple grade 3 concussions
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1 month or longer, based on the clinical determination of the evaluating physician
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return to play with grade-3 concussion with prolonged (minutes) loss of consciousness
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2 weeks
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return to play with Multiple grade-2 concussions
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2 weeks
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return to play with grade 2 concussions, single or multiple grade 1 concussions
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1 week
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who should undergo imaging in patients with TBI
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Sx of SDH, on anticoagu or with bleeding disorder; headache >72h; change in behavior, or alertness, neurologic SSx:level of behavior, consciousness, or alertness; or focal or lateralizing neurologic symptoms
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unilateral, low-frequency resting tremor
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Parkinson's disease
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bilateral, high-frequency, kinetic, and postural tremor (with both limb movement and sustained posture)
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essential tremor
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sustained contraction of agonist and antagonist muscles
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dystonia
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brief, irregular, nonstereotypical, nonrhythmic movements and can involve the extremities, head, trunk, and face
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chorea
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impaired initiation and coordination of movements and can involve speech, gait, and the extremities
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cerebellar ataxia
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abnormal lightning-like jerking extremity movements
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myoclonus
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refers to a set of clinical features that include rigidity, resting tremor, bradykinesia, and postural instability
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parkinsonism
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a specific clinical parkinsonian disorder characterized by its distinct clinical and pathologic features and by its robust therapeutic response to dopaminergic medication
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Parkinson disease
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recognized by a wide-based stance and lurching, irregular steps that are worse with turning
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cerebellar ataxia
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characterized by poor initiation of gait, freezing or complete cessation of gait, postural instability, and a slow, shuffling gait pattern
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akinetic / rigid parkinson gait
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Unilateral ataxia suggests
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structural cause due to stroke, tumor, or demyelinating disease
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truncal and leg ataxia, with the arms left unaffected
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Chronic alcohol abuse
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rapidly progressive ataxia in conjunction with signs of progressive cognitive dysfunction
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Creutzfeldt-Jakob disease or a paraneoplastic disorder
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parkinsonism or dysautonomia in a patient with chronic, progressive cerebellar ataxia
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multiple system atrophy
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primary inherited cerebellar ataxic disorders in adults
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spinocerebellar ataxia, Friedreich ataxia, and fragile X tremor ataxia syndrome
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Wernicke encephalopathy results in ataxia with these associated features
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ophthalmoplegia, confusion, peripheral neuropathy, seizures
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Patients with paraneoplastic ataxia often have these other neurologic manifestations
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peripheral neuropathy or encephalomyelitis
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the only treatable inherited ataxias
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Refsum disease, cerebrotendinous xanthomatosis, and ataxia with isolated vitamin E deficiency
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most common autosomal recessive ataxic disorder
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Friedreich ataxia
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how is Friedreich ataxia diagnosed?
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demonstrate trinucleotide (GAA) repeat in the X25 gene "fried rice"
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Diagnosis of Parkinson's disease
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REMAINS CLINICAL based on a cardinal set of clinical features -- resting tremor, bradykinesia, rigidity, and postural instability; the tremor, bradykinesia, and rigidity are asymmetric
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MOA for carvidopa
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inhibits peripheral conversion of levodopa to dopamine
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dopamine agonists
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ropinirole and pramipexole (DOPE ROPE PRAMI)
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choreiform and dystonic movements, which are involuntary and related to dopamine therapy
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dyskinesias
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treatment for disabling dyskinesias on levodopa monotherapy
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add a dopamine agonist
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first-line agents for treating depression in patients with Parkinson's disease
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SSRIs or SNRIs
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these findings help distinguish progressive supranuclear palsy from Parkinson disease
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symmetric bradykinesia and axial rigidity, lack of resting tremor, and restriction of vertical saccades
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first line agents for severe essential tremor
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propranolol and primidone; alternative agents include gabapentin, topiramate, alprazolam, and sotalol
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this demyelinating disease affects the optic nerves and spinal cord but spares the brain
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DEVIC disease or neuromyelitis optica
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how is MS differentiated from acute disseminated encephalomyelitis?
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ADEM = fevers + enceph, greater brain involvement on MRI; more CSF lymphocytosis
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temporary worsening of any MS symptom in the presence of elevations in body temperaturs
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Uhthoff phenomenon
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three patterns of MS
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relapsing-remitting, secondary progressive or primary progressive disease
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predictor of long-term progression rates in MS
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early disability
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features of primary progressive MS
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presents later in life, associated with fewer lesions on MRI
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how does pregnancy affect MS?
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"gets better until baby is born" - reduces relapse on 3rd trimester but increases risk of relapse in first 3 months after delivery
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how does smoking affect MS?
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increases risk of secondary progression threefold
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what is pseudorelapse in MS?
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transient worsening of underlying neurologic symptoms in the setting of infection or another systemic illness
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treatment of MS relapse
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methylpred 1g/d x 3-5d ffd by 10-14d oral prenisone taper (optional); if refractory, plasmapheresis
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effect of steroid treatment on MS relapse
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"faster recover, same disability long term" - does not affect long term disability sustained in relapse but substantially hastens rate of recoverty
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disease modifying therapies for MS
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F-I-N-G-M - IFNB1a and IFNB1b; glatiramer, natalizumab, fingolimod, mitoxantrone
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how does IFN beta work in MS?
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directs immune response away from autoimmunity and protects the BBB
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how does glatiramer work in MS?
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copolymer of 4 AAs that may bind MHC complex molecules and shifts immune response away from autoimmunity
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MOA of natalizumab
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monoclonal Ab that binds alpha4 integrin cellular adhesion molecules on activated T cells and inhibits ability to bind to vascular endothelium and prevents transmigration into CNS
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if natalizumab is highly effective, why is it not used as first line meds in MS? (cf IFN and glatiramer)
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increased risk of multifocal leukoencephalopathy - demyelinating disease casused by reactivation of JC virus
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the only oral disease-modifying MS agent
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"finger is oral" fingolimod
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MOA of fingolimod
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sphingosine-1-phosphate modulator restricts activated lymphocytes into LN and may have direct neuroprotective effects
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how does mitoxantrone work
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antrhracenedione chemo agent that reduces lymphocyte proliferation
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the only disease modifying MS agent that has shown benefit for secondary progressive MS
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mitoxantrone
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risks of mitoxantrone
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cardiac toxicity and secondary leukemia
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agents for primary progressive MS
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none
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treatment of MS-related fatigue
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"tired? give her a man, a mod" modafinil, amantadine
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pharmacologic treatment of gait impairment in MS
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dalfampridine (improves walking speed and leg strength)
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apnea test is considered positive if...
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pCO2 rises 20mm Hg without observed respirations
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differentiate concussion grades
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grade 1-2 no LOC, only amnesia or altered mental status; 1 is 15 mins. Grade 3 - has LOC; grade 1 can go back to game; 2 and 3 cannot go back to game; grade 3 needs to be asymptomatic x 1 week before going back to game; CT scan only if persistent symptoms
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AED of choice for women of reproductive age
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"L. O. C. K." - carbamazepine (also keppra, lamotrigine and oxcarbazepine)
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BP goal after tPA administration
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180/105 or less
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the three indications for acute anticoagulation for acute ischemic stroke
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mechanical valves, AF with small infarcts after cardiac surgery, cervicocephalic dissection
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the trial that demonstrated high dose statins reduce recurrent stroke risk in patients with LDL >100
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SPARCL - stroke prevention by aggressive reduction in cholesterol levels
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When is corpus callostomy indicated?
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palliative treatment for symptomatic generalized epilepsy with tonic or atonic seizures
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treatment of tension type headache
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acetaminophen and ibuprofen, no evidence for TCAs and cyclobenzaprine
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Effectiveness of glatiramer acetate in MS
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reduces risk of MS development in the first 3 years by 45%
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what is MCI?
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mild cognitive impairment - loss of cognitive ability that exceeds the expected age-related memory loss but does not interfere significantly with daily activities
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Woman of reproductive age, with seizure; wants to go on OCP, which meds need adjustments?
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lamotrigine needs to be increased if estrogen is used; if carbamazepine or oxcarbazepine or phenytoin or topiramate is used, OCPs are less effective
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hallmark features of frontotemporal dementia
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APHID - apathy, perseveration, hoarding and disinhibition
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features of Lewy body dementia
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"Loopy Body Dementia- sees things, enacts dreams" - visual hallucinations, dream enactment behavior, atremulous parkinsonism
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differentials for cluster headache
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chronic paroxysmal HEMICRANIA and SUNCT syndrome or short-lasting unilateral neuralgiform headache with conjunctival injection and tearing
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differentiate cluster from hemicrania and SUNCT
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hemicrania usually lasts for 15 minutes and occurs about 40x/day; cluster lasts 1-2 hours and occurs <8x/day; SUNCT lasts for seconds and occurs hundreds of times per day
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treatment of hemicrania
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responds dramatically to INDOMETHACIN
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treatment of dyskinesia in Parkinson's patient who has been on increasing doses of levodopa
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deep brain stimulation
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delusion that a relative is an impostor or that someone's home is a replica and not real
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Capgras syndrome
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opposite of Capgras syndrome (that hospital is his home)
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reduplicative paramnesia
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what is secondary headache?
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headache that is unstable or progressive in temporal pattern and usually raise a red flag
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diagnostic test of choice for cerebral vasculitis
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CTA
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limbic encephalitis associated with ovarian teratomas
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anti-NMDA receptor encephalitis
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paraneoplastic syndrome in small cell lung cancer where patients present with motor or sensory symptoms
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anti-Hu paraneoplastic encephalitis
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post-intracerebral bleed, stable, when to start DVT prophylaxis
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day 4
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treatment for MS-related fatigue
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amantadine or modafinil
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migraine prophylaxis drug that is contraindicated in patients with kidney stones
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TOPIRAMATE (mild CAI, increases calcium phosphate stones
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treatment of disruptive tics
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clonidine, pimozide,haldol
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differentiate akathisia from restless legs syndrome
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akathisia is a form of restlessness coupled with urge to move but not accompanied by dysesthesia of RLS or periodic limb movements of sleep
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treatment of restless legs syndrome
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opiates or dopamine agonists (pramiprexole or ropinirole)
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treatment of MS-related spasticity
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tizanidine (central alpha-2-adrenergic agonist), baclofen or cyclobenzaprine; if severe, trial of botulinum toxin injections or intrathecal baclofen pump
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most common causes of neuroleptic malignant syndrome
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haloperidol and fluphenazine
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differentiate NMS from malignant hyperthermia
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the drugs involved - haldol and fluphenazine for NMS; inhalational anesthetic agents and depolarizing muscle relaxants in malignant hyperthermia
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treatment for ICA dissection
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treat the stroke - STENTING not proven to improve outcomes
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AED of choice for older patients >60y/o
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lamotrigine
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lab test to monitor on patients with MS started on IFN beta 1b
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LFTs
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treatment of choice for dystonias, specifically torticollis
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botulinum toxin infections
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nutrient deficiencies that causes myelopathy of the posterior columns of the spinal cord
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B12, copper (syphilis is another infectious cause)
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