Degen disease
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| Parkinson's Disease Etiology | Degen of SN dopa neurons, onset: >40; causes: pesticides(paraquat and dieldren), uknown, vascular, viral, metabolic, ^microglial(free rads) ^glutamic activity(^Ca++), causes of 2nd: meds(haldol), Drugs mptp(endstage), multi infarct, Post encephalitis
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| Parkinson's Disease Pathogenesis | dec. DA(striatum)=inc. ACH(GP)=inc GABA(Basil ganglia=inc inhbition of Thamlmus=inc inhib of MC= poverty of movement, nicotine protect, anti inflams, alpha-synuclei neuroprotect
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| Parkinson's Dx | clinical, gold std.=neuropathology, depig of SN, neuronal loss of SN, lewy bodies; r/o wilson's Disease
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| Parkinson's clinical manifestations | tremor, rigidity, akinesia, postural instability, festinant gait, slow start/stop, soft monot voice, Autonomic-orthohypo, urin reten, seborrhea, Depress, anxiety, dementia; lack of convergence, glabellar tap, blepharoclonus, head drop
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| Stages of Parkinson's | I: unilateral, II: bilateral, III: postural unsteadiness, IV: movement disabling, V: bed ridend
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| Selegine | MAO-BI(eldepral) Medical Tx of Parkinson's, blocks DA->DOPAC, 3-MT->HVA, NE-> epi(high tyrosine, hypertensive crisis)
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| Tasmer | COMT-I(tolcapine) Medical Tx of Parkinson's, blocks DA->3-MT, DOPAC->HVA, hepatotoxic
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| Congentin | anticholinergic(benzotropine) artane, used with haldol and thorizeine to treat parkinson like ss, ^HR, Gastroparesis, confusion in elderly
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| Parlodel | DA agonist(bromocriptine), SE: psychosis, hallucin, Permax(pergolide)increase valvular disease(removed)
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| Amantadine | DA reuptake inhibitor; usually for depression
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| Sinemet | L-dopa+carbidopa, SE: dyskinesia, n/v, on off phenom due to protein comp, wearing off re-emgergence of manigests, progresses disease quicker
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| Parkinson's tx >60 | use sinement, add DA agonist; avoid: selegine, amatadine, anticholinergic
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| Parkinson's tx 50-59 | use: selegiline, DA agonist, add sinemet, amantadine
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| Parkinson's tx <50 | use: selegeline, amantadine, anticholinergic
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| Parkinson's SX Tx | Palidotomy: GPi, Thalmotomy: unilateral ventral(bi= dysarthria); Thalamic stim/subthalamic stim deep, bilateral and reversible, unknown mech
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| Striatonigral Degen | Degen of BG, SN and Striatum, resistant to L-dopa, no lewy,(similar SS to parkins)
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| Shy drager syndrome | Degen of BG, associated with autonomic dysfunction and extrapyramidal manifestations
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| Oliviopontocerebellar atrophy | degen of BG, no 2 cases alike, eye movement abn, dysarthria
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| Huntington's Disease Etiology | auto dom disorder CHR 4 CAG repeats, degen GABA neurons, onset: 40y, common:N. euro, Juvenile: from Dad, 10-15 years life expectancy,
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| Huntington's disease pathogenesis | Dec GABA from the striatum(Gabaneurgic die from build up of gaba precusors no glutamic decarboxylase)=inc DA from SN= dec ACH on GP= dec GABA on thalmus= overexitement of the motor cortex
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| Huntigton's Disease clinical Manifestations | hyperkinesa, hypotonia, decrease in mentations
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| Huntington's Dx | Genetic Screening, MRI: flatening of the cuadate nucleous
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| Hunington's Tx | Haldol: Da antagonist, dec. movement disorder, Li+: decrease mood swings,
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| MS etio | CNS demyel, death of olgodendrocyte, scars, plaques, viral/genetic/ immune mech, in cold climates that are variable(virus)
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| MS patho | over active T cells=cytokines=B cell activation=abs to myelin basic protein, proteolysis, myelin oligodendroglycoprotein, activate Macro+complement, increase in HLA DR2 20x risk,corticospinal tract(most common), brainstem, cerebellar, cerebral
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| MS corticospinal manifests | weakness, hyper-reflexiam spasticity, bowel/bladder dysf(x)'
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| MS brainstem manifests | lateral gaze paralysis, nystagmus, vertigo, tinnitus, facial weakness, lhermitte's sign with spinal cord lesions
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| MS cerebellar manifests | ataxia, intent tremor, poor articulation, clumsiness, poor balance
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| MS cerbral manifests | optic neuritis: optic pallor(fuzzy-> blind), scotoma(dec. vision), intellestual changes, emotional changes(attnetion), sensory
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| MS course of Disease | progressive-relapse (steady decline superimposed attacks), secondary progressive (intial RR->progressive), primary progressive(steady increase w/o attacks), relapsing remiting(attacks followed by remiccion) RR-> 2nd progressive 85%start-> 50% progress
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| MS Dx | MRI: anm om 90%, CSF: >90% uave IgG high protein, invoked potentials
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| MS tx | Interferon B1a: avonenx daily, B1b: netageron 2/week; dec t vell prolif, decrease HLA express, dec. gamma interferon(inc. response); corticos(acute, baclofen(spasticity), bethanechol(choline agon, urinary reten), capaxone, competes with MBP for t cell
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| Ataxia telangiectasia | auto recessive chr 11, immunodeficient; dec IgA and IgE; increase of lymphom or leuk 1000x, wobbly 2 year old, telangiectasia spider vessels
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