Inspired by the other path cards Flashcards
cortical degeneration –>
dementia
basal ganglia degeneration –>
mvmt disorders
spinocerebellar degeneration –>
ataxia
motor neuron degeneration –>
upper and lower motor weakness
Cortical degenerative diseases
Pick disease, AD
AD characteristic lesions
neuronal plaques and neurofibrillary tangles (Presinillin gene)
B amyloidopathy and tau-opathy
AD
Pick disease
frontotemporal lobar degeneration, dementia with psychosocial sx
Pick bodies in cytoplasm of neuron
Parkinson Disease
neurodegeneration in basal ganglia (SNpc)
Lewy bodies (filamentous alpha synuclein)
loss of neurons in substantia nigra
Huntington Disease
caudate nucleus and putamen "batwing defomity" Autosomal Dominant genetic disease chromosome 4-CAG repeat expansion GABA decreased emotional sx
Freidrich Ataxia
most common inherited ataxia
ataxia and dysarthria
degeneration/atrophy of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts
Amyotropic Lateral Sclerosis
Upper and lower motor neuron disease
neuronal loss and gliosis
Lewy Bodies
Parkinson disease, filamentous alpha synuclein
- Scattered plaques on brain and spinal cord-areas of demyelination
- blurred vision/vision loss in one eye
- may be sporadic numbness/weakness
- relapses and remits
Multiple Sclerosis
- Demyelinating disorder with predilection for optic nerves and spinal cord
- Autoantibodies to aquaporin 4
Neuromyelitis optica
- may follow immunization r infection with measles, varicella or rubella
- focal perivascular demyelination
Post vaccinal/infectious encephalomyelitis
- autosomal recessive
- accumulation of cerebroside in white matter and peripheral nerves
- Arylsulfatase A deficiency
Metachromatic Leukodystrophy
Autosomal recessive
Deficiency of galactocerebrosidase
Presence of Globoid cells
Severe Motor, Sensory and Cognitive Deficits
Krabbe Disease
- X-Linked
- High levels of saturated very long chain fatty acids–>impaired degradation by adding CoA
- bilateral cerebral symmetrical demyelination
- Defect in peroxisomes
Adrenoleukodystrophy