Neurodegenerative disease Flashcards
What is characteristic of Alzheimer’s disease?
Early memory and visuospatial problems
Acetylcholine deficit
What is characteristic of frontotemporal dementia?
Early behavioral, executive, and/or language.
One mutation- C9 open reading frame 72- also have psychosis and ALS
What is characteristic of Parkinson’s disease?
Tremor, rigidity, bradykinesia
Lewy bodies with synuclein protein
Dopamine deficit
What is Lewy body dementia?
It has the same early parkinsonian features, but with psychosis and fluctuating level of consciousness
Lewy bodies with synuclein protein
Dopamine and acetylcholine deficit
What is progressive supranuclear palsy?
Abnormal vertical eye movements (volitional diminished, but reflex retained), bradykinesia, rigidity, falls- similar to Parkinsons
What is amyotrophic lateral sclerosis?
Weakness and atrophy, fasciculations, both upper and lower motor neuron signs
What is Huntington’s Disease?
dementia, depression, chorea. Largely behavioral problems.Autosomal dominant
Increased polyglutamine repeats (CAG) in Huntington gene on chromosome 4
Caudate atrophy
What is Creutzfeldt-Jakob disease?
Rapidly progressive dementia, myoclonus- prion disease. Early symptoms are variable and based on where the disease starts. Onset usually age 55-65.
What is the protein involved in prion disease?
PrP- normally present, but misfolded in disease
What is kuru?
Prion disease due to cannibalism in Papua New Guinea. Early tremor and ataxia
What is GSS?
Autosomal dominant. Presence of dementia and severity varies. Cerebellar findings- clumsiness, gait issues, lack of coordination.
What is fatal familial insomnia?
AD, 35-60 years. Progressive insomnia, memory loss, confusion, hallucinations.
What are the predominant neurotransmitter deficits in neurodegenerative illnesses?
Acetylcholine:
Alzheimer’s, Lewy body disease
Dopamine:
Parkinson’s, Lewy body disease
Serotonin:
FTDWhat are the histological findings in frontotemporal dementia?
Neurofibrillary tangles (tau protein deposition)
Tau-reactive intraneuronal inclusions = Pick bodies
Dipeptide with C9ORF72
FUS or TDP-43
Dementia lacking distinct histological features
What are clinical findings associated with FTD?
aphasia, behavioral issues
