GBS

Question 1

name the 4 neurological deficits in GBS

Answer 1

1. muscle paralysis
2. sensory deficits
3. autonomic dysfunction
4. areflexia

Question 2

what is AIDP, which pathogens are responsible for it and which part does it affect?

Answer 2

acute inflammatory demyelinating polyneuropathy, demyelination, cytomegalovirus and Epstein-Barr virus, sensorimotor

Question 3

what is AMAN, which pathogen is responsible for it and which part does it affect?

Answer 3

acute motor axonal neuropathy, axonal degeneration, C. jejuni, pure motor

Question 4

which antibodies are present in GBS?

Answer 4

antibodies against gangliosides (IgM, IgG1, IgG3 –> sialic acid containing glycolipids) which are mainly present in neuronal cell membranes and myelin

Question 5

why is GBS not a typical classic auto-immune disease?

Answer 5

1. no predominance in females
2. no association with other auto-immune diseases
3. no relapsing-remitting or chronic disease course
4. no association with specific HLA haplotypes
5. no improvement after corticosteroids

Question 6

what is molecular mimicry?

Answer 6

structural similarities between self-antigen and pathogen molecules leads to the formation of cross-reactive auto-antibodies

Question 7

Name the 3 components of the peripheral nerve immunity

Answer 7

1. blood-nerve barrier; passage of activated T-cells, macrophages and immunoglobulins
2. antigen presentation; endoneurial macrophages
3. peripheral nerves; require intact cell surface for normal electrophysiology

Question 8

name 1 acute and 3 chronic immune-mediated peripheral neuropathies

Answer 8

acute; Guillain-barré syndrome
chronic; chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), paraprotein-related neuropathy (MAG)

Question 9

what causes nerve damage?

Answer 9

complement deposition and macrophage recruitment

Question 10

what is the physiology of the blood-nerve barrier?

Answer 10

leaky at the nerve roots, ganglia and the neuromuscular junction