Neurodegenerative Diseases Flashcards
Alzheimer’s disease
Present with early memory ad visuospatial problems
Frontotemporal dementia (FTD)
Present with early behavioral, executive and language problems
Lewy Body dementia
Present with early Parkinsonian features, PSYCHOSIS, fluctuating consciousness
Parkinson’s disease
Present with tremor, rigidity and bradykinesia
Progressive supranuclear palsy (PSP)
Present with bradykinesia, rigidity, falls, abnormal vertical eye movements
Fall early in disease than other ND dx
Amyotrophic lateral sclerosis
Present with weakness and atrophy, fasciculations, and the combination of both upper and lower motor neuron signs
Prion diseases (Creutzfeldt-Jakob)
Present with rapidly progressive dementia with myoclonus (a prion disease)
What is a Prion?
A proteinaceous infectious particle, lacks nucleic acid
The prion protein PrP appears to be the sole constituent of these agents. Normal PrP is expressed in most human tissues, with the highest levels found in the CNS. A prion diseae is a CONFORMSTIONAL CHANGE in the protein
Presentation of Prion Diseases
Present with ataxia, abnormal movement, and neuropsychiatric features (depression, executive decision making, memory, etc)
Tell me some extra-scary things about Prion Diseases
It is sporadic, heritable AND transmissible
ALWAYS fatal
Can happen REALLY fast (ie CJD can go from onset to death in only 3 months)
Gerstmann-Straussler-Scheinker Syndrome
*Autosomal dominant
*1 to 10 cases per 100 million population per year
*Cerebellar findings (Clumsiness, incoordination, and gait ataxia
Myoclonus is usually absent in GSS)
*The presence of dementia, and its severity, varies among affected families and individuals within the same family
The neurochemistry in various degenerative diseases
Tau :FTD, PSP
Synuclein: Parkinson’s
Ubiquitin : ALS
Polyglutamine: Huntington’s
Prion protein: CJD
