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Neurology

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Action tremor:   Most common; arise when attempting to maintain a fixed position; frequency is 7-13 Hz; Essential or physiologic  
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Essential tremor =   resembles slower frequency physiologic tremor; essential or familial (auto dom); common in later life; extremities, head (titubation), or voice  
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Drug causes of physiologic tremor   Amphetamines, theophylline, lithium and valproate.  
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Essential tremor Tx   Firstline: BB: propranolol 60-240mg/day (CI in COPD; asthma; DM; bradycardia; AV conduction probs); Primidone 50/day - 125 TID (effective but sedating); Alprazolam for anxiety related; EtOH: temporary suppression  
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Ataxic (intention) tremor   Absent at rest/start of movement; dysmetria  
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Ataxic tremor: Causes:   Dz of cerebellum / connections (MS; tumors; infarct; ethanol-induced cerebellar degeneration)  
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Ataxic tremor: Tx:   Meds usu ineffective; Weights; surgical lesions of ventrolateral thalamus may be effective in severe cases  
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Athetosis:   slow writhing purposeless movements usu involving hands, tongue & face; usu in kids w/ cerebral palsy or result of kernicterus or hypoxia (poss later in life d/t hypoxia)  
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Chorea: Sx   Involuntary, irregular jerky movements; can cause continuous movements.  
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Chorea: may be due to:   untreated strep infxn (Sydenham chorea), PG (chorea gravidarum) or Huntington dz  
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Tics   onset 2-13 yrs; tx Haldol/pimozide  
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Hemiballismus   Violent flinging movements; Unilateral hemiballismus usu d/t infarct in contralateral subthalamic nucleus of Luys  
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Myoclonus =   Shock-like contraction of a group of mx; irreg in rhythm/amplitude; may involve restricted grp mx or be generalized.  
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Myoclonus may result from:   anoxic damage, spinal cord injury, uremia, hepatic encephalopathy or other rare neuro syndromes  
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Myoclonus Tx   Clonazepam, Valproate (Both have limited efficacy)  
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Dystonia:   Maintenance of a persistent extreme posture in one or more joints.  
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Generalized dystonia:   dystonia musculorum deformans; a rare hereditary dystonia  
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Focal dystonias:   torticollis (dystonia of the neck), writer's cramp & blepharospasm (dystonia of the mx’s involving eye closure).  
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Dystonia: Tx   Botox injxn increasingly common & helpful; Surgical tx (torticollis) also available; Medical tx (benzos, anticholinergics, neuroleptics) generally unsatisfactory  
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Parkinson dz: epidemiology   500,000 in US; onset 40-70 yo; M=F  
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Parkinson dz: Sx   Unilateral onset. Rest tremor/3-6 hz; pill rolling; cogwheeling; rigidity; bradykinesia; difficulty initiating movement. Masked facies; stooped posture, shuffling or festinating gait. Diminished postural reflexes & eye blinking rate. Micrographia  
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Parkinson dz: Pathology   Degeneration & loss of pigmented cells in nigrostriatal pathway, raphe nuclei, locus ceruleus & motor nucleus of vagus; dramatic loss of DA-containing neurons  
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L-DOPA   80% of pts improve; Sinemet treats akinesia & is less effective in treating tremor.  
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L-DOPA MOA   precursor of DA (which cannot cross BBB; L-DOPA can)  
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Dopamine agonists (Parlodel, Mirapex, Requip) MOA   Act like DA; direct DA effect on striatal neurons; may allow for reduction in dose of Sinemet required & may decrease on-off phenomena  
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Amantidine MOA   May increase DA release from presynaptic storage site at nerve terminals; weakly effective  
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Anticholinergic drugs (Artane, Cogentin) MOA:   May be more effective for tx of tremor; AE = confusion, constipation, dry mouth, urinary retention  
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Selegiline MOA   inhibits monoamine oxidase type B  
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Cabergoline MOA   inhibits COMT  
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Huntington Dz presentation   AD, onset 30-50 yo; Sx often begin w/ psych disorder (immaturity, impulsivity, depression); later apathy & dementia; Chorea, Athetosis; butterfly ventricles (caudate atrophy)  
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Huntington chorea due to:   Decreased activity of acetylcholine & GABA neurons; increased activity of dopaminergic neurons  
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Huntington Tx   For sx reduction only. Tetrabenazine or amantadine 25 TID for chorea. DA receptor blockers (haloperidol) for behaviors/dyskinesias; clozapine  
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Wilson dz   Auto rec; onset 2nd-3rd decades; first sx: hepatic dysfn/cirrhosis; tremor/ bradykinesia, dysarthria, dysphagia; limbs rigid, facial mx fixed empty smile; arms: wing-beating tremor; Kayser-Fleischer (deep cornea)  
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Wilson: labs   Serum ceruloplasmin (Cu carrying pro) very low; urine Cu high  
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Wilson: patho logy   CNS changes: brownish discoloration of some basal ganglia; proliferation of protoplasmic astrocytes (Alzheimer Type II cells)  
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Wilson: Tx   Should begin before neuro Sx onset; early tx prevents neuro sequela devt; eat low Cu foods; sulfurated potash with meals (prevent Cu absorption); chelator (d-penicillamine) to remove absorbed Cu  
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Wilson dz: Tx: foods to avoid   liver; chocolate; mushrooms; shellfish; nuts  
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Tardive dyskinesia: cause   Iatrogenic d/t LT neuroleptic tx (esp Haldol; also atypicals, poss metoclopramide, amphetamines, L-dopa); may be result of DA receptor supersensitivity  
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Tardive dyskinesia: Sx   Abnormal BLM (tongue thrusting & chewing); head movements (turning & bobbing); poss also abnormal limb movements; Sx can fluctuate & may take mos-yrs to resolve (sometimes permanent)  
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Tardive dyskinesia: Tx   stop the offending drug; many tx tried w/ marginal success  
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Tardive dyskinesia: most successful tx:   achieved with DA-depleting agents (tetrabenazine); also Vitamin E?  
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Excess DA results in:   dyskinesia and chorea  
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Dopamine deficiencies cause:   Parkinsonian-like symptoms  
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Action of acetylcholine & DA:   Act in opposite directions; adding DA is equivalent to blocking acetylcholine  
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CJD Sx   usu later in life; rapidly progressive dementia, myoclonus, ataxia & somnolence; epileptiform pattern on EEG  
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MS epidemiology   F>M; 350K in US (1M world); onset 20-40 yo; most present w/relapsing form; prevalence increases with distance from equator  
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MS S/S   Optic (retrobulbar) neuritis; Transverse myelitis; Paresthesias; Ataxia; Weakness or incoordination; Spasticity; Cognitive impairment  
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MS: MRI findings   multiple characteristic lesions or plaques: periventricular or subcortical U-fibers, corpus callosum lesions  
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MS: CSF findings   evidence of oligoclonal bands or increased IgG index  
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MS: types of dx criteria   Schumacher; Poser: Macdonald  
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MS: Goals of tx   Tx whole dz; slow accumulation of disability; Reduce relapse rate & CNS inflam (lesions) & progression of brain atrophy (shrinkage); improve pt QOL (including cognitive)  
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MS Tx options   Beta-interferon; SQ glatiramer; poss immunosupp adjuncts for aggressive/ breakthrough; corticosteroids (methylprednisolone) for relapse  
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Forms of MS:   relapsing-remitting, primary progressive, secondary progressive  
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Tourette etiology / pathology   Auto dominent; can be presentation of Wilson dz. Onset usually 2-15 yo  
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Recurrent episodes of vision change, diplopia, weakness & tingling in extremities that resolve   MS  
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CJD S/S   usu later in life; rapidly progressive dementia, myoclonus, ataxia & somnolence; epileptiform pattern on EEG  
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MS S/S   Optic neuritis; Transverse myelitis; Paresthesias; focal neuralgia; Ataxia; Weakness/ incoordination; Spasticity; Cognitive impairment  
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Lhermitte’s sign =   electrical sensation down body w/ neck flexion; seen in MS  
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MS pathophys   immuno d/o assoc w/CNS Ig prodn & T-lymph alteration; poss viral etio  
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MS pattern of sxs   affect multiple areas over time (if they don't, prob not MS)  
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Ataxic (intention) tremor   Absent at rest/start of movement; dysmetria; Dz of cerebellum / connections (MS; tumors; infarct; ethanol-induced cerebellar degeneration)  
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Dystonia:   Maintenance of a persistent extreme posture in one or more joints.  
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Focal dystonias:   torticollis, writer's cramp, blepharospasm; tx Botox / surg  
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Huntington Dz presentation   AD, varying age of onset; Sx often begin w/ psych disorder (immaturity, impulsivity, depression); later apathy & dementia; Chorea, Athetosis; butterfly ventricles (caudate atrophy)  
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Huntington chorea due to:   DA excess state  
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Wilson dz   Auto rec; onset 2nd-3rd decades; first sx: hepatic dysfn/cirrhosis; tremor/ bradykinesia, dysarthria, dysphagia; limbs rigid, facial mx fixed empty smile; arms: wing-beating tremor; Kayser-Fleischer (deep cornea)  
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Wilson: patho   CNS changes: brownish discoloration of some basal ganglia; proliferation of protoplasmic astrocytes (Alzheimer Type II cells)  
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Tardive dyskinesia: Sx   Abnormal BLM (tongue thrusting & chewing); head movements (turning & bobbing); poss also abnormal limb movements; Sx can fluctuate & may take mos-yrs to resolve (sometimes permanent)  
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Tardive dyskinesia: most successful tx:   achieved with DA-depleting agents (tetrabenazine); also Vitamin E?  
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Action of acetylcholine & DA:   Act in opposite directions; adding DA is equivalent to blocking acetylcholine  
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RLS can be primary, or secondary to:   periph neuropathy, uremia, PG, Fe def  
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ALS: Dx criteria   UMN & LMN sx in 3 regions; UMN: clinical; LMN: EMG; CK nml or high  
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Primary lateral sclerosis: path   degeneration of lateral corticospinal tract  
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Primary lateral sclerosis clin findings   usu legs before arms; leg weak/stiff; spasticity; ave duration >8 yrs; many pts develop LMN sx & transition to ALS  
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progressive muscular atrophy   pure LMN dz; slower progress than ALS; often spares bulbar mx; CK very high  
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Wilson manifestations:   hepatolenticular degen (impaired ceruloplasmin synth); presents in teen years (hepatitis); tremor, dysarthria, slow, hoarse, chorea  
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Parkinson: TRAP =   Tremor, Rigidity, Akinesia, Postural instability  
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Most common genetic form of Parkinson:   PARK8  
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Progressive demyelinating; brain, sp cord, optic n.; viral; F>M, peak 20-40yo   MS  
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Entacapone is tx for:   Parkinson dz  
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Entacapone side effect   brown urine  
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Damaged areas in ALS   corticospinal tracts, anterior horn cells, bulbar motor nuclei  
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MS testing   MRI, VER, BAER, SSEP, CSF oligoclonal banding, spinal fluid IgG  
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MS S/S   15-50 yo; optic neuritis; fatigue; Lhermitte sx; Uhthoff's phenomenon  
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MS tx   methylprednisolone/ IVIg for acute; glatiramer & interferon for relapsing- remitting sx  
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