Degenerative Diseases--Krafts

Question 1

Clinical and morphological findings of Alzheimer’s

Answer 1

• Major degenerative disease of the cortex
• Main symptom: dementia
• Gross: atrophy
• Microscopic: plaques and tangles
• Prognosis: 3-20 years

Question 2

Clinical and morphological findings of Pick disease

Answer 2

• Rare, distinctive signs (locations), progressive dementia
• Frontal lobe signs (personality changes) and temporal lobe signs (language disturbances)
• Severe (“knife-edge”) atrophy of frontal and temporal lobes only
• Neuronal loss, swollen neurons (“Pick cells”), cytoplasmic neuronal inclusions (“Pick bodies”)
• Pick bodies contain tau protein

Question 3

Clinical and morphological findings of Parkinson’s disease

Answer 3

• Degeneration of neurons in substantia nigra
• Main symptoms: tremor, rigidity, bradykinesia
• Gross: atrophy of substantia nigra
• Microscopic: Lewy bodies
• Prognosis: slightly shortened life expectancy

Question 4

Clinical and morphological findings of amyotrophic lateral sclerosis

Answer 4

• Degeneration of motor neurons (Only LMNs!!)
• Rapidly progressive weakness, spasticity, dysphagia
• Sensory and cognitive function are unaffected
• Death within 2-3 years due to respiratory compromise

Question 5

Alzheimer Disease Morphology

Answer 5

Neuritic plaques: outside neurons, contain ß-amyloid

Neurofibrillary tangles: within neuron cytoplams, composed of tau, MAP2, ubiquitin (these destroy the neurons!!!)

Question 6

Parkinsonism symptomes

Answer 6

• Diminished facial expression
• Stooped posture
• Slowness of voluntary movement
• Festinating gait (small steps initially, then faster and faster)
• Rigidity
• “Pill-rolling” tremor

Question 7

Parkinson Disease Morphology

Answer 7

• Pallor of substantia nigra
• Loss of pigmented neurons
• Lewy bodies in some of the remaining neurons
• May also have neuronal loss and Lewy bodies in basal nucleus of Meynert (associated with abnormal mental function)

Question 8

ALS clinical features

Answer 8

• Early: asymmetric hand weakness, arm/leg spasticity, twitching, slurred speech
• Then atrophy, fasciculations, creeping paralysis
• Eventually respiratory muscles involved (infection)
• “Progressive muscular atrophy” vs. “progressive bulbar palsy”