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Neuropath final

Degen disease

QuestionAnswer
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
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
Parkinson's Dx clinical, gold std.=neuropathology, depig of SN, neuronal loss of SN, lewy bodies; r/o wilson's Disease
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
Stages of Parkinson's I: unilateral, II: bilateral, III: postural unsteadiness, IV: movement disabling, V: bed ridend
Selegine MAO-BI(eldepral) Medical Tx of Parkinson's, blocks DA->DOPAC, 3-MT->HVA, NE-> epi(high tyrosine, hypertensive crisis)
Tasmer COMT-I(tolcapine) Medical Tx of Parkinson's, blocks DA->3-MT, DOPAC->HVA, hepatotoxic
Congentin anticholinergic(benzotropine) artane, used with haldol and thorizeine to treat parkinson like ss, ^HR, Gastroparesis, confusion in elderly
Parlodel DA agonist(bromocriptine), SE: psychosis, hallucin, Permax(pergolide)increase valvular disease(removed)
Amantadine DA reuptake inhibitor; usually for depression
Sinemet L-dopa+carbidopa, SE: dyskinesia, n/v, on off phenom due to protein comp, wearing off re-emgergence of manigests, progresses disease quicker
Parkinson's tx >60 use sinement, add DA agonist; avoid: selegine, amatadine, anticholinergic
Parkinson's tx 50-59 use: selegiline, DA agonist, add sinemet, amantadine
Parkinson's tx <50 use: selegeline, amantadine, anticholinergic
Parkinson's SX Tx Palidotomy: GPi, Thalmotomy: unilateral ventral(bi= dysarthria); Thalamic stim/subthalamic stim deep, bilateral and reversible, unknown mech
Striatonigral Degen Degen of BG, SN and Striatum, resistant to L-dopa, no lewy,(similar SS to parkins)
Shy drager syndrome Degen of BG, associated with autonomic dysfunction and extrapyramidal manifestations
Oliviopontocerebellar atrophy degen of BG, no 2 cases alike, eye movement abn, dysarthria
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,
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
Huntigton's Disease clinical Manifestations hyperkinesa, hypotonia, decrease in mentations
Huntington's Dx Genetic Screening, MRI: flatening of the cuadate nucleous
Hunington's Tx Haldol: Da antagonist, dec. movement disorder, Li+: decrease mood swings,
MS etio CNS demyel, death of olgodendrocyte, scars, plaques, viral/genetic/ immune mech, in cold climates that are variable(virus)
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
MS corticospinal manifests weakness, hyper-reflexiam spasticity, bowel/bladder dysf(x)'
MS brainstem manifests lateral gaze paralysis, nystagmus, vertigo, tinnitus, facial weakness, lhermitte's sign with spinal cord lesions
MS cerebellar manifests ataxia, intent tremor, poor articulation, clumsiness, poor balance
MS cerbral manifests optic neuritis: optic pallor(fuzzy-> blind), scotoma(dec. vision), intellestual changes, emotional changes(attnetion), sensory
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
MS Dx MRI: anm om 90%, CSF: >90% uave IgG high protein, invoked potentials
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
Ataxia telangiectasia auto recessive chr 11, immunodeficient; dec IgA and IgE; increase of lymphom or leuk 1000x, wobbly 2 year old, telangiectasia spider vessels
Created by: bmg4
 

 



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