This is the appearance of normal myelin by electron microscopy using the "freeze-fracture" technique. Note the beautifully delineated layers or lamellae.
GBS, also called Acute Post Infective Polyneuritis, is one of the feared complications of vaccinations such as Swine Flu and Pertussis.
More often it follows a viral syndrome by a week or two. It is an allergic type reaction wherein antibodies are created by lymphocytes which mistakenly attack the host's peripheral (not central - that is a distinct but analagous disease called Acute Disseminated Encephalomyelitis - the myelin is different in the CNS versus the PNS, made by oligendrocytes centrally, Schwann cells peripherally) myelin.
These are microscopic views of peripheral nerves showing lymphocytic infiltration on the LEFT and fragmentation of myelin on the RIGHT.
Guillain Barre may be difficult to diagnose at first. The victim is often young and healthy, complains of tingling in the extremities easily blamed on the high anxiety which is invariably present. The anxiety results from the victim's awareness of progressive paralysis.
Doctors are sometimes taught that it begins peripherally and spreads proximally, i.e. from the tips of the feet up the legs but this is simply untrue: GBS often begins in hip flexor or shoulder girdle muscles with subtle impairments of function such as going up or downstairs or raising the arms overhead. Difficult to confirm on individual muscle testing.
GBS is an EMERGENCY. It can and does progress over a few days to a week finally reaching a nadir or severity from moderate weakness to total quadriplegia in one to two weeks or less when promptly diagnosed and treated.
It can plateau and begin reversing at any point up but there may be a need for prolonged artificial ventilation.
Normal Myelinated Peripheral Nerve
GBS patients will recover eventually although re-myelination results in shortened intervals between the Nodes of Ranvier with resultant slowing of conduction.
It is CRITICAL to diagnose this condition ASAP because a highly effective treatment is available and the sooner the better. The treatment, PLASMAPHERESIS, consists of collecting some of the white cells and abnormal proteins from the patient's blood by centrifugation and throwing it away. Recovery time can be shortened 50% or more.
The disease at the outset is most commonly misdiagnosed as mental, in the mind, "supratentorial" is the code word. Or it can be mistaken for Botulism, some tick bites, some poisonous fish and molluscs, diphtheria, Rabies, myaesthenia....
The DISAPPEARANCE of deep tendon reflexes (e.g. the knee jerk) has been dependably confirmatory in my experience. The finding of an elevated CSF protein, 40 to 100 mg% without more than 10 white cells, (the famous "dissociation albumino-cytologique" of Guillain or maybe Barre) is equally strong evidence.
Although learned debate goes on about plasmapheresis, IV gamma globulin and steroid, I'll go for the first choice anytime. I've seen patients turn around during their first session - but get it early.
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