Lecture+21+Parkinsons - Definition of Parkinson Disease...

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1 Definition of Parkinson Disease Clinical syndrome: TRAP T remor R igidity A kinesia/bradykinesia P ostural instability Typical Neuropathology Symptoms and signs in PD Hypomimia: loss of facial expression (“masked facies”) Hypophonia: decreased voice volume Cogwheel rigidity: increased tone with “rachet” quality Bradykinesia: slow movement Festination: small, stuttering steps Freezing: arrests in movement
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2 Cognitive Function in PD PD patients are frequenly depressed Decreased performance on cognitive tests especially tests that track frontal lobe cognitive ability: planning, execution (why?) Wisconsin Card Sort, Stroop A smaller proportion (10-30%) become demented Coexists with Alzheimer’s disease (dementia with Lewy bodies) History First described by James Parkinson 1817 Dopaminergic deficit discovered in 1960 Oral levodopa trials 1960’s First dopamine agonist (bromocriptine) 1974 MPTP toxin model of PD 1980’s Deep brain stimulation developed 1990’s First genetic causes identified starting 1997 COMT inhibitors developed 1998
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3 Epidemiology 1 million people in US Male:female = 3:2 Mean age: 65; 10% before age 40 About 5 % of cases are familial Gross pathology Substantia nigra neuronal loss - gross depigmentation < 50% loss, no symptoms 70% loss causes PD symptoms >90% loss at death
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4 Pathological hallmark: Lewy body Intracytoplasmic inclusions found in substantia nigra Contain α -synuclein Lewy Body Neuromelanin
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5 Dementia With Lewy Bodies (DLB)
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Lecture+21+Parkinsons - Definition of Parkinson Disease...

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