Neuropathic Disease

Question 1

What are the CT layers of a spinal nerve?

Answer 1

• epineurium
• perineurium
• endoneurium

Question 2

The perineurium covers what part of a nerve?

Answer 2

-a fascicle

epi= whole nerve, endo= individual fiber

Question 3

Term for “disease of peripheral nerves”

Answer 3

-neuropathy

Question 4

1 inherited neuropathy

Answer 4

CMT

but note that all inherited neuropathies are rare

Question 5

What are the three types of CMT?

Answer 5

1) demyelinating
2) axonal
3) infantile

Question 6

CMT 1:
Type
Mutation

Answer 6

• Demyelinating

- AD, PMP22 gene (peripheral myelin protein)

Question 7

Appearance of nerves in CMT 1

Answer 7

Large, demyelinated –> remyelinated multiple times

Question 8

CMT clinical presentation

Answer 8

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

Question 9

CMT 2
type
inheritance pattern

Answer 9

axonal

AD

Question 10

CMT2 clinical presenation

Answer 10

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

Question 11

CMT3:

• type
• inheritance pattern
• aka

Answer 11

• infantile demyelinating
• AR
• Dejerine Sottas

Question 12

CMT3 clinical presenation

Answer 12

severe, delayed motor milestones

Question 13

CMT diagnosis

Answer 13

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

Question 14

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

Answer 14

• Amyloidosis

- Mitochondrial Disorders

Question 15

What are 5 autoimmune/ inflammatory neuropathies?

Answer 15

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

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Question 16

What are two infectious neuropathies?

Answer 16

1) HIV

2) Lyme (bilateral bells)

Question 17

What are vitamin related causes of neuropathy?

Answer 17

• B6 toxicity

- B12 deficiency

Question 18

What are four industrial exposures that may cause neuropathy?

Answer 18

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

Question 19

What are six drugs that are assc with neuropathies?

Answer 19

Colchicine Hydralazine Metronidazole Nitrous Oxide Paclitaxel Vincristine

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Question 20

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

Answer 20

-methylmalonic acid, if elevated may ~ B12 deficiency

Question 21

What are the 3 clinical symptoms of B12 deficiency

Answer 21

1) numbness
2) gait disturbance
3) neuropathic pain

+/- posterior column myelopathy

Question 22

In carpal tunnel, what muscles are weak ?

Answer 22

1) abductor pollicis brevis

2) opponens pollicis

Question 23

Where is the majority of sensory loss in diabetic neuropathy?

Answer 23

sock and glove distribution (hands and feet)

Question 24

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

Answer 24

1) burning pain

2) weakness in late stage

Question 25

What are the treatments for pain control in diabetic neuropathy?

Answer 25

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

Question 26

Is Guillain Barre an axonal or demyelinating neuropathy?

Answer 26

-primarily demyelinating, may see secondary axonal loss

Question 27

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

Answer 27

- EBV - CMV - Lyme Disease - Hepatitis - HIV

Question 28

Classic guillain barre presentation

Answer 28

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

Question 29

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

Answer 29

- 2-4 weeks | - 5-10%

Question 30

Dx for GBS

Answer 30

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

Question 31

Treatment for GBS

Answer 31

-PLEX -IVIG (one or the other not both) +/- ventilation

Question 32

Miller Fisher Variant of GBS: | presentation

Answer 32

- facial weakness - dysarthris - ophthalmoplegia - areflexia

Question 33

Ab assc with Miller Fisher GBS

Answer 33

Anti GQ1B

Question 34

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): pathogenesis

Answer 34

-macs + T cells destroy endoneurium - segmental demyelination

Question 35

CIDP is the peripheral equivalent of _____.

Answer 35

MS, just effects peripheral instead of central nerves

Question 36

Dx CIDP

Answer 36

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

Question 37

Treatment for CIDP

Answer 37

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

Question 38

Familial ALS: | Genetic Mutation

Answer 38

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

Question 39

ALS: | classic presentation

Answer 39

-upper AND lower motor neuron signs

Question 40

``` ALS: #1 COD + life expectancy ```

Answer 40

- ventilatory failure | - 3 years after onset of focal weakness

Question 41

FDA approved treatment for ALS

Answer 41

-riluzole --> prolongs life by three months