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Neuro > Neuropathic Disease > Flashcards

Neuropathic Disease Flashcards

1
Q

What are the CT layers of a spinal nerve?

A
  • epineurium
  • perineurium
  • endoneurium
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2
Q

The perineurium covers what part of a nerve?

A

-a fascicle

epi= whole nerve, endo= individual fiber

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3
Q

Term for “disease of peripheral nerves”

A

-neuropathy

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4
Q

1 inherited neuropathy

A

CMT

but note that all inherited neuropathies are rare

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5
Q

What are the three types of CMT?

A

1) demyelinating
2) axonal
3) infantile

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6
Q

CMT 1:
Type
Mutation

A
  • Demyelinating

- AD, PMP22 gene (peripheral myelin protein)

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7
Q

Appearance of nerves in CMT 1

A

Large, demyelinated –> remyelinated multiple times

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8
Q

CMT clinical presentation

A
  • high arches
  • hammer toes
  • distal muscle (leg) weakness + wasting
  • numbness, but patients don’t complain of it
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9
Q

CMT 2
type
inheritance pattern

A

axonal

AD

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10
Q

CMT2 clinical presenation

A

-CMT 1 but slower progression, less likely to need wheelchair

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11
Q

CMT3:

  • type
  • inheritance pattern
  • aka
A
  • infantile demyelinating
  • AR
  • Dejerine Sottas
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12
Q

CMT3 clinical presenation

A

severe, delayed motor milestones

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13
Q

CMT diagnosis

A
  • Nerve conduction studies to determine axonal vs demyelinating pattern
  • +/- genetic testing
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14
Q

In addition to CMT, what are two other hereditary neuropathies?

A
  • Amyloidosis

- Mitochondrial Disorders

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15
Q

What are 5 autoimmune/ inflammatory neuropathies?

A

1) MGUS
2) Sarcoid
3) Sjogrens
4) Rheumatoid arthritis
5) Vasculitis

Must Save Some Rose Vases

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16
Q

What are two infectious neuropathies?

A

1) HIV

2) Lyme (bilateral bells)

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17
Q

What are vitamin related causes of neuropathy?

A
  • B6 toxicity

- B12 deficiency

18
Q

What are four industrial exposures that may cause neuropathy?

A

1) arsenic
2) lead
3) mercury
4) organophosphates

19
Q

What are six drugs that are assc with neuropathies?

A

Colchicine Hydralazine Metronidazole Nitrous Oxide Paclitaxel Vincristine

Cheetas Headbutted Martinis. Nevertheless, Oprah Preached Vigilantly.

20
Q

In addition to checking serum B12, if B vitamin deficiency is expected, what other lab should be drawn?

A

-methylmalonic acid, if elevated may ~ B12 deficiency

21
Q

What are the 3 clinical symptoms of B12 deficiency

A

1) numbness
2) gait disturbance
3) neuropathic pain

+/- posterior column myelopathy

22
Q

In carpal tunnel, what muscles are weak ?

A

1) abductor pollicis brevis

2) opponens pollicis

23
Q

Where is the majority of sensory loss in diabetic neuropathy?

A

sock and glove distribution (hands and feet)

24
Q

In addition to sensory loss, what are the symptoms of diabetic neuropathy

A

1) burning pain

2) weakness in late stage

25
Q

What are the treatments for pain control in diabetic neuropathy?

A

1) gabapentin/ pregabalin
2) SNRIs
3) TCAs
4) AEDs
5) topical capscaisin

26
Q

Is Guillain Barre an axonal or demyelinating neuropathy?

A

-primarily demyelinating, may see secondary axonal loss

27
Q

In addition to Campylobacter, what infections are assc with Guillian Barre?

A
  • EBV
  • CMV
  • Lyme Disease
  • Hepatitis
  • HIV
28
Q

Classic guillain barre presentation

A

rapidly ascending weakness
loss of DTRs
midback pain
autonomic instability, respiratory failure

29
Q

How long does GBS typically last?

What is the mortality rate for GBS?

A
  • 2-4 weeks

- 5-10%

30
Q

Dx for GBS

A

1) cytoalbumic dissociation in CSF

2) slowed nerve conduction studies

31
Q

Treatment for GBS

A

-PLEX
-IVIG
(one or the other not both)

+/- ventilation

32
Q

Miller Fisher Variant of GBS:

presentation

A
  • facial weakness
  • dysarthris
  • ophthalmoplegia
  • areflexia
33
Q

Ab assc with Miller Fisher GBS

A

Anti GQ1B

34
Q

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): pathogenesis

A

-macs + T cells destroy endoneurium - segmental demyelination

35
Q

CIDP is the peripheral equivalent of _____.

A

MS, just effects peripheral instead of central nerves

36
Q

Dx CIDP

A
  • CSF: elevated protein
  • EMG/NCS
  • Biopsy (onion bulbing)
37
Q

Treatment for CIDP

A
  • steroids
  • PLEX
  • IVIG
  • immunosuppression (Azathioprine, mycophenolate, Cytoxan)
38
Q

Familial ALS:

Genetic Mutation

A
  • AD
  • SOF gene
  • only 5-10% of cases
39
Q

ALS:

classic presentation

A

-upper AND lower motor neuron signs

40
Q 
ALS: 
#1 COD + life expectancy
A
  • ventilatory failure

- 3 years after onset of focal weakness

41
Q

FDA approved treatment for ALS

A

-riluzole –> prolongs life by three months

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