Neurodegenerative Diseases Flashcards
General overview/underlying cause of neurodegenerative disorders
- often due to spontaneous failure w/in body
- includes spontaneous death of a neuronal population
- location of population ==> clinical presentation
Typical categories of classification for neurodegenerative diseases
- clinical presentation
- transmission
- neurochemistry
- neuropathology
Common dementia-related problems in neurodegenerative diseases
- memory
- language
- executive function
- visuospatial
- depression, apathy, sociopathy
Common movement disorder problems in neurodegenerative diseases
- bradykinesia
- rigidity
- tremor
- chorea = involuntary movement disorder
Common motor/muscular-related problems in neurodegenerative diseases
- muscle weakness
- muscle atrophy
Dementia could indicate…
- Alzheimer’s disease
- FTD
- Lewy body
- HD
- CJD
Motor problems could indicate…
- ALS
- ALS/FTD (frontotemporal dementia)
Movement disorders could indicate…
- Parkinson’s
- PSP (progressive supranuclear palsy)
- CJD
- Huntingtons
Alzheimer’s disease: Clinical Features & Transmission
- clinical = early memory and visuospatial problems
- transmission
- most = sporadic
- genetic
- presenilin 1 mutation
- trisomy 21
Frontotemporal dementia: Clinical Features & Transmission
- early behavioral, executive, and/or language problems
- transmitted both genetically and sporadically
Parkinson’s disease: Clinical Features & Transmission
- tremor, rigidity, bradykinesia
- leads to slow speech and movement
- genetic and sporadic transmission
Lewy Body Dementia: Clinical Features & Transmission
- early parkinsonian features, psychosis, fluctuating consciousness
- genetic and sporadic transmission
Progressive supranuclear palsy: Clinical Features & Transmission
- bradykinesia, rigidity, falls
- abnormal vertical eye movements
- genetic and sporadic transmission
ALS: Clinical Features & Transmission
- weakness and atrophy
- fasciculations
- UMN & LMN signs
- genetic and sporadic transmission
CJD: Clinical Features & Transmission
- rapidly progressing dementia
- myoclonus (jerky muscle contraction) developing over months
- (spontaneous) onset usually 55-65yo
- genetic, spontaneous, and infectious transmission
Huntington’s disease: Clinical Features & Transmission
- dementia
- depression, sociopathy (aggression)
- chorea
- inherited: autosomal dominant
Kuru: Clinical Features & Transmission
- early tremor and ataxia
- caused by canabalism
Gertsmann Straussler Scheinker Syndrome (GSS): Clinical Features & Transmission
- clumsiness, incoordination, ataxia
- NO myoclonus
- varied presentations
- genetic only: autosomal dominant
Fatal Familial Insomnia disease: Clinical Features & Transmission
- progressive insomnia
- memory loss, confusion
- hallucinations
- genetic: autosomal dominant
Alzheimer’s disease: Neurochemistry & Neuropathology
- ACh deficit (cholinergic hypothesis)
- Amyloid plaques and neurofibrillary tangles
- diffuse atrophy globally
- cortex and hippocampus involved
Frontotemporal dementia: Neurochemistry & Neuropathology
- tau proteins vs. seratonin deficit
- frontal and temporal lobe most affected
- neurofibrillary tangles
- ubiquitin inclusions
- pick bodies
- no distinct histology
Parkinson’s disease: Neurochemistry & Neuropathology
- Chemistry
- Synuclein
- Dopamine deficit
- Pathology
- Lewy bodies
Lewy Body Dementia: Neurochemistry & Neuropathology
- ACh deficit
- Dopamine deficit
- Lewy bodies
Progressive supranuclear palsy: Neurochemistry & Neuropathology
- tau proteins
ALS: Neurochemistry & Neuropathology
- ubiquitin
CJD: Neurochemistry & Neuropathology
- prion protein
Huntington’s disease: Neurochemistry & Neuropathology
- polyglutamine
- characteristic gross changes w/out microscopic changes
- caudate nucleus atrophy
- ventricles ==> butterflies
Kuru disease: Neurochemistry
- prion protein
GSS: Neuropathology
- cerebellar problems
Fatal familial Insomnia: Neuropathology
- thalamus problems
Characteristics of prion diseases
- can be sporadic, heritable, or transmissible/infectious
- caused by proteinaceious infectious particle w/out nucleic acid
- natural protein ==> deforms to beta pleated sheet
- uniformly fatal diseases
