Disorders of CNS and PNS

Question 1

cerebral ischemia results in infarction (necrosis) or brain tissue, death or disabled

Answer 1

Stroke

Question 2

rupture of saccular aneurysm

Answer 2

Subarachnoid hemorrhage

Question 3

autoimmune demyelinating disease, very common, relapsing-remitting, white matter plaques (sclerosis) in brain and cord (multiple), young adult females, accumulation of neurologic deficits (ataxia, diplopia, spasticity, bowel/bladder dysfunction)

Answer 3

Multiple sclerosis

Question 4

loss of spinal cord lower motor neurons, mixed upper and lower motor neuron damage, muscle atrophy, hyperreflexia, rapid progression, spares eye muscles (Stephen Hawking), usually death is rapid due to respiratory paralysis

Answer 4

Amyotrophic lateral sclerosis (Lou Gehrig disease)

Question 5

most common cause of dementia in elderly, idiopathic, cortical atrophy, β-amyloid plaque and Tau protein deposition, neurofibrillary tangles, profound disability (lethal)

Answer 5

Alzheimer disease

Question 6

damage to dopaminergic neurons of substantia nigra, Lewy body inclusions, “pill-rolling” tremor, rigidity, bradykinesia (slow movement), loss of facial expression, immobility within 10 years

Answer 6

Parkinson disease

Question 7

bilateral vestibular schwannomas (MRI), vision or hearing problems

Answer 7

Neurofibromatosis type II

Question 8

autosomal dominant, progressive and widespread cerebral degeneration (caudate nucleus atrophy, hydrocephalus ex vacuo), chorea (jerky movement), severe dementia, lethal within 15 years

Answer 8

Huntington disease

Question 9

cyst within the spinal cord (syrinx), pain, paralysis, weakness

Answer 9

Syringomyelia

Question 10

widespread white matter damage, commonly from motor vehicle accident, lethal

Answer 10

Diffuse axonal injury

Question 11

demyelination due to Schwann cell or myelin cells damage

Answer 11

Neuropathy

Question 12

diffuse neuropathy, stocking-and-glove paresthesia (diabetes)

Answer 12

Polyneuropathy

Question 13

most common cause of peripheral neuropathy, symmetrical, damage to vasa nervorum, undiscovered foot ulcerations, gangrenous necrosis, monofilament test

Answer 13

Diabetic neuropathy

Question 14

Merlin gene mutations, cutaneous neurofibromas, Lisch nodules of iris, café au lait spots AKA von Recklinghausen disease

Answer 14

Neurofibromatosis type I

Question 15

autoantibodies block postsynaptic acetylcholine receptors, ptosis, diplopia, extraocular muscle fatigue with activity, thymic hyperplasia

Answer 15

Myasthenia gravis

Question 16

autoantibodies inhibit presynaptic calcium channels, which reduces acetylcholine into synaptic cleft, muscle weakness, improves with activity (opposite of MG)

Answer 16

Lambert-Eaton syndrome

Question 17

chronic idiopathic demyelinization polyneuropathy, ascending paralysis (weakness), “rubbery legs,” from lower extremities, many die once progresses into respiratory muscles (respiratory failure)

Answer 17

Guillain-Barre syndrome

Question 18

reactive proliferation of Schwann cells to trauma, non-neoplastic, hands/feet

Answer 18

Traumatic neuroma

Question 19

dystrophin gene mutation, severe, pelvic girdle weakness, evident by at 5, wheelchair bound, lethal by early 20s, Gower sign, pseudohypertrophy of calf muscles

Answer 19

Duchenne muscular dystrophy

Question 20

dystrophin gene mutations, mild, evident in adolescence, variable prognosis

Answer 20

Becker muscular dystrophy