6: Demyelinating, Degenerative, Toxic, and Metabolic Disorders Flashcards
demyelinating vs degenerative disease major difference
- demyelinating: affects white matter
2. degenerative: affects grey matter
critical initiation event in developing Alzheimer’s**
AB generation
what is Tau?
abnormally hyper-phosphorylated axonal microtubule-associated proteins
from what protein are AB peptides derived?
APP (amyloid precursor protein)
what correlates with the degree of dementia in Alzhemiers?
of neurofibrillary tangles
MPTP
synthetic heroine that caused acute parkinsonism
nigrostriatal pathway in basal ganglia major role
regulates synaptic pathways that modulate feedback from thalamus to motor cortex
two major COD in Huntingtons
PNA or suicide
anticipation in a genetic disease
repeat expansions during spermatogenesis -> next generation will get earlier onset of the disease
spinocerebellar degenerations group of diseases
loss/dysfunction in the cerebellum, SC, and peripheral nerves
progressive muscular atrophy vs primary lateral sclerosis
Progressive muscular atrophy: ALS cases where LMN involvement predominates
Primary lateral sclerosis: ALS cases where UMN involvement dominates
three types of genetic metabolic diseases
neuronal storage diseases, leukodystrophies, mitochondrial encephalopathies
why is metachromatic dystrophy named that way?
crystalloid structures composed of sulfatides appear in peripheral nerves -> stain with Toluidine blue -> metachromatic
Heteroplasmy
presence of both wild type and mutated mitos within a single cell -> wide variation in disease expression
what does MELAS stand for?
mitochondrial encephalopathy, lactic acidosis, stroke-like episodes
