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Neuropath 2 > NEUROPATH LAST QUIZ > Flashcards

NEUROPATH LAST QUIZ Flashcards

1
Q

What is the most commonly inherited neurological disorder?

A

CHARCOT-MARIE-TOOTH DISEASE aka peroneal muscular atrophy

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2
Q

What does it mean that CMT is genetically heterogeneous?

A

there are multiple different ways that it can result (genetically)

  • autosomal dominant
  • autosomal recessive
  • x chromosome linked pattern will see men affected
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3
Q

Differentiate CMT-1 vs. CMT-2

A

CMT-1: peripheral demyelination d/t abnormal myelin production
CMT-2: axonal death and degeneration WITHOUT a primary myelin defect

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4
Q

CMT is/is not progressive and does/does not have both sensory and motor symptoms

A

IS progressive (slowly)

DOES have both

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5
Q

Describe typical feet in someone with CMT:

A

pes cavus with hammer toes (curled)
foot drop
champagne bottle legs

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6
Q

Differentiate the signs and symptoms between CMT1 and CMT2

A

CMT1: proprioception lost in feet/ankles, diminished cutaneous sensation (pain and temperature)

CMT2: impaired proprioception. minimal sensory loss; tingling and burning in feet/legs.

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7
Q

How is CMT diagnosed?

A

nerve biopsy and nerve conduction studies

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8
Q

How is CMT treated?

A

symptomatic tx to try to minimize deformity and improve function
massage, ROM to prevent contractures
anti-inflammatories, analgesics, orthotic devices

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9
Q

B3 (niacin/nicotinic acid) deficiency can have what consequences?

A

delirium, dermatitis, diarrhea

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10
Q

Give an elevator pitch for Tay-Sachs disease.

A

a rare genetic disorder that must be inherited from both parents, involving absence of the enzyme HEXOSAMINIDASE, which helps break down fatty substances.

if these substances build up to toxic levels in the child’s brain, it will affect the function of nerve cells

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11
Q

Who is most likely to end up with Tay-Sachs disease?

A

Eastern and Central European Jews
French Canadian communities in Quebec
Old Order Amish in Pennsylvania
Cajun in Louisiana

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12
Q

When do infants with Tay-Sachs start showing symptoms?

A

~6 months

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13
Q

What are the most common S&S of Tay-Sachs?

A 
loss of motor skills
exaggerated reactions to loud noises
seizures
vision & hearing loss
cherry-red spots in the eyes
paresis, muscle weakness
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14
Q

How is Tay-Sach’s diagnosed?

A
  • eye exam for cherry red-spot
  • blood levels of hexosaminidase
  • heredity (family history)
  • pediatric neurologist exam
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15
Q

Symptomatic treatment for Tay-Sachs disease includes…

A

medication
respiratory care (chest physio to clear lung mucus)
feeding tubes
gastrostomy (assistive feeding tube)

physical and massage therapy
hopefully, in the future: gene therapy or enzyme replacement therapy

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16
Q

Niemann-Pick Disease is a rare, inherited disease that affects…

A

the body’s ability to metabolize fat (cholesterol, lipids) within cells

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17
Q

Niemann-Pick Disease is caused by a deficiency in the activity of what enzyme? What happens to the affected lipid?

A

sphingomyelinase

sphingomyelin accumulates in organs

18
Q

The 3 types of Niemann-Pick disease are differentiated based on…

A

when the symptoms appear

Type A - first few months of life
B - may not show for years
C - may not experience symptoms till adulthood

19
Q

Is there a cure for Niemann-Pick disease?

A

no

20
Q

Which type of Niemann-Pick disease is the least common/most rare?

A

Type C

21
Q

Which type of Niemann-Pick disease does not have brain or nervous system involvement??

A

Type B

22
Q

Tarsal tunnel syndrome has also been called…

A

posterior tibial neuralgia

23
Q

Freidreich ataxia is a rare, inherited disease… people with this disease will most commonly die from…

A

heart disease

24
Q

There is/is no cure or effective treatment for FA

A

there is NO

25
Q

Sacral plexus is formed from the ventral rami of…

A

L4-S4

26
Q

Both sciatica and piriformis are usually __lateral

A

unilateral

27
Q

Sciatica does/does not usually get better on its own

A

does

28
Q

Piriformis syndrome most commonly occurs during the…

A

4th & 5th decades of life, moreso in women than men

29
Q

The most common form of hereditary ataxia in the united states is…

A

Friedreich ataxia

30
Q

S&S of FA usually start between the ages of…

A

5-15

31
Q

50% of non-traumatic amputations are due to…

A

diabetes mellitus

32
Q

“Nutritional neuropathy” refers to…

A

alcoholic neuropathy, which is direct poisoning of nerves by alcohol & the effects of poor nutrition associated with alcoholism (esp. vB1 deficiency)

33
Q

Pernicious anemia refers to a deficiency of which B vitamin?

A

B12

34
Q

B12 deficiency seems to involve abnormal and then absent ____ of PNS and then CNS (in severe cases) nerves

A

myelination

35
Q

Wernicke-Korsakoff syndrome is…

A

a brain disorder d/t vitamin B1 (thiamine) deficiency

36
Q

What’s the difference between Wernicke encephalopathy and Korsakoff syndrome?

A

they often occur together

Wernicke encephalopathy causes brain damage in the thalamus and hypithalamus from lack of B1

Korsakoff psychosis is d/t permanent damage to the amygdala and hippocampus

37
Q

What is the most common cause of acute flaccid paralysis?

A

GBS

38
Q

What is the most common type of GBS?

A

Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

39
Q

What bacteria is one of the most common risk factors for GBS?

A

campylobacter jejuni

40
Q

Up to ___% of people who have GBS will have lasting/remaining neurologic defects

A

20%

41
Q

What is plasmapheresis?

A

removal of antibodies from the blood

42
Q

Toxic lead neuropathy is mainly… sensory or motor?

A

motor

Neuropath 2 (9 decks)

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