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Flashcards in Neuropathic Disease Deck (41):
1

What are the CT layers of a spinal nerve?

-epineurium
-perineurium
-endoneurium

2

The perineurium covers what part of a nerve?

-a fascicle
(epi= whole nerve, endo= individual fiber)

3

Term for "disease of peripheral nerves"

-neuropathy

4

#1 inherited neuropathy

CMT
(but note that all inherited neuropathies are rare)

5

What are the three types of CMT?

1) demyelinating
2) axonal
3) infantile

6

CMT 1:
Type
Mutation

-Demyelinating
-AD, PMP22 gene (peripheral myelin protein)

7

Appearance of nerves in CMT 1

Large, demyelinated --> remyelinated multiple times

8

CMT clinical presentation

-high arches
-hammer toes
-distal muscle (leg) weakness + wasting
-numbness, but patients don't complain of it

9

CMT 2
type
inheritance pattern

axonal
AD

10

CMT2 clinical presenation

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

11

CMT3:
-type
-inheritance pattern
-aka

-infantile demyelinating
-AR
-Dejerine Sottas

12

CMT3 clinical presenation

severe, delayed motor milestones

13

CMT diagnosis

-Nerve conduction studies to determine axonal vs demyelinating pattern
-+/- genetic testing

14

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

-Amyloidosis
-Mitochondrial Disorders

15

What are 5 autoimmune/ inflammatory neuropathies?

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

Must Save Some Rose Vases

16

What are two infectious neuropathies?

1) HIV
2) Lyme (bilateral bells)

17

What are vitamin related causes of neuropathy?

-B6 toxicity
-B12 deficiency

18

What are four industrial exposures that may cause neuropathy?

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

19

What are six drugs that are assc with neuropathies?

Colchicine Hydralazine Metronidazole Nitrous Oxide Paclitaxel Vincristine

Cheetas Headbutted Martinis. Nevertheless, Oprah Preached Vigilantly.

20

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

-methylmalonic acid, if elevated may ~ B12 deficiency

21

What are the 3 clinical symptoms of B12 deficiency

1) numbness
2) gait disturbance
3) neuropathic pain

+/- posterior column myelopathy

22

In carpal tunnel, what muscles are weak ?

1) abductor pollicis brevis
2) opponens pollicis

23

Where is the majority of sensory loss in diabetic neuropathy?

sock and glove distribution (hands and feet)

24

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

1) burning pain
2) weakness in late stage

25

What are the treatments for pain control in diabetic neuropathy?

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

26

Is Guillain Barre an axonal or demyelinating neuropathy?

-primarily demyelinating, may see secondary axonal loss

27

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

-EBV
-CMV
-Lyme Disease
-Hepatitis
-HIV

28

Classic guillain barre presentation

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

29

How long does GBS typically last?
What is the mortality rate for GBS?

-2-4 weeks
-5-10%

30

Dx for GBS

1) cytoalbumic dissociation in CSF
2) slowed nerve conduction studies

31

Treatment for GBS

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

+/- ventilation

32

Miller Fisher Variant of GBS:
presentation

-facial weakness
-dysarthris
-ophthalmoplegia
-areflexia

33

Ab assc with Miller Fisher GBS

Anti GQ1B

34

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): pathogenesis

-macs + T cells destroy endoneurium - segmental demyelination

35

CIDP is the peripheral equivalent of _____.

MS, just effects peripheral instead of central nerves

36

Dx CIDP

-CSF: elevated protein
-EMG/NCS
-Biopsy (onion bulbing)

37

Treatment for CIDP

-steroids
-PLEX
-IVIG
-immunosuppression (Azathioprine, mycophenolate, Cytoxan)

38

Familial ALS:
Genetic Mutation

-AD
-SOF gene
-only 5-10% of cases

39

ALS:
classic presentation

-upper AND lower motor neuron signs

40

ALS:
#1 COD + life expectancy

-ventilatory failure
-3 years after onset of focal weakness

41

FDA approved treatment for ALS

-riluzole --> prolongs life by three months