With respect to Guillain Barre Syndrome and Myasthenia Gravis, compare and contrast the respective:
a) Clinical features. (30% marks)
b) Investigations needed to make the diagnosis. (30% marks)
c) Specific treatment strategies with the relevant evidence/recommendations for the strategies listed (40% marks)
Condition |
Clinical Features |
Investigations |
Treatment Strategies |
Guillain-Barre Syndrome |
Diarrhoeal prodrome Autonomic instability Symmetrical, progressive ascending weakness with associated areflexia. Ventilatory failure May be cranial nerve involvement. Sensory loss usually mild or absent. |
Nerve conduction studies show demyelination (reduction in conduction velocities) CSF analysis: high protein and normal WCC |
IVIG Plasmapheresis The use of plasma exchange or IVIG depends on the institution or whether the patient has a contraindication to IG such as IgA deficiency. Both have been shown to be equally efficacious. One Cochrane review suggested that IVIG given within 2 weeks of the onset was as efficacious as plasma exchange, and more likely to be completed than plasma exchange. Their combination is not supported. |
Myasthenia Gravis |
Fluctuating, often fatiguable, weakness and possible progression to ventilator failure but reflexes intact. Ptosis with associated diplopia. |
Auto-antibodies against nicotinic Ach receptors and MuSK Antibodies EMG and NCS studies |
Anticholinesterases e.g. Pyridostigmine – symptomatic treatment more effective in antiAChR than MuSK Rapid Immunotherapy (IVIG and PLEX) – recommended for myasthenic crises Chronic Immunotherpay (Steroids and immunosuppressants) – majority require this with antiAChase Thymectomy – not recommended routinely for age>60 unless thymoma present |
a)
Features | Guillain-Barre | Myasthenia Gravis |
Clinical setting |
|
|
Motor weakness |
|
|
Sensory loss |
|
|
Reflexes |
|
|
Cranial nerve involvement |
|
|
Dysautonomia |
|
|
b)
Guillain Barre Syndrome |
|
Myasthenia Gravis |
|
c)
Guillain Barre Syndrome |
|
Myasthenia Gravis |
|
Chapter 57 (pp. 617) Neuromuscular diseases in intensive care by George Skowronski and Manoj K Saxena
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