Neurodegenerative Diseases

Question 1

Alzheimer’s Disease

Answer 1

Early memory and visuospacial problems

Path: amyloid plaques and Neurofibrilary tangles, typically in cortex and hippocampus with sparing of deeper structures. Grossly, this looks like diffuse atrophy

Acetylcholine deficit

Question 2

Frontotemporal dementia

Answer 2

Early Behavioral, executive, and language problems

Path: varied, many phenotypes, including Neurofibrilary tangles, ubiquitin inclusions, tau, etc.

Question 3

Parkinson’s Disease

Answer 3

Tremor, rigidity, and bradykinesia

Path: Lewy bodies with synuclein protein

Dopamine deficit

Question 4

Lewy

Answer 4

Early Parkinsonian features, psychosis, fluctuating consciousness

Path: Lewy bodies with synuclein protein

Dopamine AND acetylcholine deficit

Question 5

Progressive supra nuclear palsy

Answer 5

Bradykinesia, rigidity, falls, [abnormal vertical eye movements]

Question 6

Amyotrophic lateral sclerosis

Answer 6

Weakness and atrophy, farcical actions, and the combination of both upper and lower motor neuron signs

Question 7

Huntington’s Disease

Answer 7

Dementia, depression, and chorea

Path: Atrophy of head of caudate

CAG repeats on chromosome 4, Autosomal dominant

Question 8

Creutzfeldt-Jacob disease

Answer 8

Rapidly progressive dementia with myoclonus

Compare to Gerstmann-Straussler-Scheinker: a familial prion disease with cerebelli signs including clumsiness, in coordination, and ataxia.

Also compare Fatal Familial Insomnia, Autosomal dominant, with progressive insomnia, memory loss, confusion, and hallucinations