neurodegenerative Flashcards

1
Q

syringomyelia

A

C8-T1. cystic cavity called a syrinx, crossing anterior spinal commissures are damaged first. cape-like distribution loss of pain and temperature in the upper extremities. fine touch is preserved. associated with chiari malformations and trauma.

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2
Q

what is the supraoptic for

A

ADH

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3
Q

what is the paraventricular nucleus for

A

oxytocin

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4
Q

what is the lateral hypothalamus control?

A

hunger. leptin inhibits this

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5
Q

what is the venteromedial hypothalamus for

A

satiety, leptin stimulates this

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6
Q

what is the anterior hypothalamus for

A

cooling and parasympathetics

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7
Q

what is the posteior

A

heating and sympathetics

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8
Q

what is the suprachiasmatic nucleus for

A

this is for circadian rhythms and sleep wake.

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9
Q

huntingtons

A

20-50 AD trinucleotide repeat chromosome 4. choreiform movements, aggression, dementia. there is increased dopamine, decreased GABA and decreased ACh. there is neuronal death via glutamate overstimulation. atrophy of the caudate. ex vacuo dilation of the frontal horns. CAG repeat.

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10
Q

poliomyelitis or werdig-hoffman (spinal muscular atrophy

A

lower motor neuron signs only. there is destruction of anterior horns with flaccid paralysis.

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11
Q

amyotrophic lateral sclerosis

A

combined upper motor and lower motor neuron signs. with NO sensory or ocularmotor deficits. UMN.LMN caused by super oxide dismutase. presents with fasciculations and eventually atrophy and weakness of the hands.

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12
Q

tabes dorsalis

A

demyelination of the dorsal columns and roots, impaired proprioception. progressive sensory ataxia. absence of deep tendon reflexes and rhomberg sign

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13
Q

vitamin B 12 deficiency

A

subacute combined degeneration. there is demyelination of the dorsal columns, lateral corticospinal tracts and spinocerebrellar tracts. ataxia, paresthesia, impaired position sense and vibration sense.

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14
Q

charcot-marie-toothh

A

hereditary motor and sensory neuropathy. defective proteins of the structure and function of myelin. presents with scoliosis and foot deformities.

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15
Q

krabbe

A

lysosomal storage disease due to decreased galactocerebrosidase destroys the myelin sheath. there are globoid cells, developmental delay, optic neuropathy

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16
Q

metachromatic leukodystrophy

A

lysosomal storage disease -build up of sulfatides. there is impaired production of myelin. central and peripheral demyelination with ataxia and dementia

17
Q

progressive multifocal leukencephalopathy

A

JC virus mediated rapidly progressive, fatal loss of oligodendrocytes.