Neurodegenerative Diseases Flashcards
Alzheimer’s disease
Amyloid plaques + neurofibrillary tangles (caused by protein misfolding)
Acetylcholine deficit
Early memory and visuospatial problems
Associated with Trisomy 21
Frontotemporal dementia
Many different pathologies with a common phenotype
Serotonin deficit
Early behavioral, executive, apathy and language problems
-motor is totally fine
Parkinson’s disease
Lewy bodies with synuclein protein Lewy bodies are located in the substantia nigra in PD Dopamine deficit Tremor, rigidity, and bradykinesia -stiff and slow movement
Lewy Body Dementia
Lewy bodies with synuclein protein
Lewy bodies more diffusely distributed in the brain in LBD
Dopamine AND acetylcholine deficit
Early parkinsonian features, psychosis, fluctuating conciousness
-delusional, hallucinations, paranoid
Progressive supranuclear palsy
Caused by protein misfolding
Bradykinesia, rigidity, falls, abnormal vertical eye movements
-falls are prominent
Amyotrophic lateral sclerosis
Caused by cell death of motor neurons in the ventral spinal cord and pre-central gyrus
Weakness and atrophy, fasciculations, and the combination of both upper and lower motor neuron signs
-weak and twitchy, mix of UMN and LMN signs
Huntington’s disease
Autosomal dominant
Increased polyglutamine (trinucleotide) repeats (CAG) in Huntington gene on chromosome 4
Caudate nucleus atrophy
Chorea (fast, twitchy movements), depression, dementia
-acquired “sociopathy”, stealing at work, cheating on spouse, DUIs, etc.
Creutzfeldt -Jacob disease
Prion disease, abnormal prions causing normal prions to undergo abnormal conformational change
Movement disorder rapidly progressive dementia with myoclonus
-high velocity twitching disease (myoclonus)
Kuru
Prion disease
Transmitted by ritual cannibalism in Papua New Guinea
Gerstmann-Straussler-Scheinker Syndrome
Autosomal dominant prion disease
The presence of dementia, and its severity, varies among affected families and individuals within the same family
