Inspired by the other path cards

Question 1

cortical degeneration –>

Answer 1

dementia

Question 2

basal ganglia degeneration –>

Answer 2

mvmt disorders

Question 3

spinocerebellar degeneration –>

Answer 3

ataxia

Question 4

motor neuron degeneration –>

Answer 4

upper and lower motor weakness

Question 5

Cortical degenerative diseases

Answer 5

Pick disease, AD

Question 6

AD characteristic lesions

Answer 6

neuronal plaques and neurofibrillary tangles (Presinillin gene)

Question 7

B amyloidopathy and tau-opathy

Answer 7

AD

Question 8

Pick disease

Answer 8

frontotemporal lobar degeneration, dementia with psychosocial sx
Pick bodies in cytoplasm of neuron

Question 9

Parkinson Disease

Answer 9

neurodegeneration in basal ganglia (SNpc)
Lewy bodies (filamentous alpha synuclein)
loss of neurons in substantia nigra

Question 10

Huntington Disease

Answer 10

caudate nucleus and putamen "batwing defomity"
Autosomal Dominant genetic disease
chromosome 4-CAG repeat expansion
GABA decreased
emotional sx

Question 11

Freidrich Ataxia

Answer 11

most common inherited ataxia
ataxia and dysarthria
degeneration/atrophy of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts

Question 12

Amyotropic Lateral Sclerosis

Answer 12

Upper and lower motor neuron disease

neuronal loss and gliosis

Question 13

Lewy Bodies

Answer 13

Parkinson disease, filamentous alpha synuclein

Question 14

• 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

Answer 14

Multiple Sclerosis

Question 15

• Demyelinating disorder with predilection for optic nerves and spinal cord
• Autoantibodies to aquaporin 4

Answer 15

Neuromyelitis optica

Question 16

• may follow immunization r infection with measles, varicella or rubella
• focal perivascular demyelination

Answer 16

Post vaccinal/infectious encephalomyelitis

Question 17

• autosomal recessive
• accumulation of cerebroside in white matter and peripheral nerves
• Arylsulfatase A deficiency

Answer 17

Metachromatic Leukodystrophy

Question 18

Autosomal recessive
Deficiency of galactocerebrosidase
Presence of Globoid cells
Severe Motor, Sensory and Cognitive Deficits

Answer 18

Krabbe Disease

Question 19

• X-Linked
• High levels of saturated very long chain fatty acids–>impaired degradation by adding CoA
• bilateral cerebral symmetrical demyelination
• Defect in peroxisomes

Answer 19

Adrenoleukodystrophy