inflammatory polyneuropathies

Question 1

What might you expect to find on CSF analysis of patient with AIDP (GBS)?

Answer 1

High protein in the absence of white cells - called albuminocytic dissociation. normal protein suggests an infectious mimic, poliomyositis in the old days

Question 2

What blood tests might be useful if you suspect Guillian Barre syndrome?

Question 3

What might you find on EMG in Guillian Barre syndrome?

Answer 3

Initially can be normal. After 2 weeks you might see conduction block, temporal dispersion of responses and delayed late responses (f waves).

Question 4

What might you see on lumbar MRI in Guillian Barre syndrome?

Answer 4

You can see some enhancement of lumbar nerve roots.

Question 5

What nerve tends to be spared in GBS?

Answer 5

often you see sural sparing

Question 6

what is the natural history of GBS?

Answer 6

acute monophasic illness reaching nadir within 4 weeks

Question 7

What is the supportive management of GBS?

Answer 7

check respiratory function, check neck flexion strength, make a plan to keep monitoring resp function with FVC. Consider early intubation as crash intubation dangerous in GBS. Pain relief for back pain.

Question 8

What is the medical treatment of GBS?

Answer 8

plasma exchange and IVIG. one not better than other. No evidence for giving both, but most people would if patient worsening. Advantages to IVIG first is that less invasive. Advantages to plasmapheresis first is that won’t skim off all your IVIG if given first. Neither induces recovery but both hasten it.

Question 9

What is Miller Fisher GBS?

Answer 9

Variant of GBS in which there is ophthalmoplegia, ataxia and areflexia. Tend to be GQ1B antibody positive.

Question 10

What are the differences in the treatment of CIDP and AIDP (GBS)?

Answer 10

Main difference is that CIDP responds to steroids in a way that GBS does not. With both conditions, mainstay of treatment is IVIG infusions but in CIDP other immunosuppressive medication can be considered due to its long term nature.

Question 11

AMAN is an axonal variant of CIDP. What does it stand for?

Answer 11

Acute motor axonal neuropathy

Question 12

AMSAN is a mixed axonal variant of CIDP. What does is stand for?

Answer 12

Acute motor and sensory axonal neuropathy

Question 13

What conditions are most strongly associated with GQ1B antibody positivity?

Answer 13

Miller Fisher syndrome, Bickerstaff brainstem encephalitis

Question 14

How does Bickerstaff brainstem encephalitis present?

Answer 14

Presents like miller fisher plus encephalitis (opthalmoplegia, areflexia, ataxia and encephalitis). Some people think it doesn’t really exist.

Question 15

What is the name of the CIDP variant causing motor and sensory symptoms in the distribution of multiple, discreet nerves. Demyelinating process.
Similar EMG findings to usual CIDP but less diffuse. CSF protein elevated often.

Answer 15

MADSAM or Lewis-sumner syndrome.

Question 16

What do you know about MMN?

Answer 16

This is a variant of CIDP affecting motor nerves only (multifocal motor neuropathy). As it effects motor nerves only it is a common differential for ALS. No sensory findings. Lower motor neuron signs only.

Question 17

What antibody is strongly associated with multifocal motor neuropathy?

Answer 17

GM-1 ganglioside antibody

Question 18

What do you know about the CIDP variant DADS?

Answer 18

Distal symmetric sensory neuropathy. Slow progression, tends to affect older patients, difficult to treat condition. Associated with anti MAG antibodies.