Saturday, July 5, 2008

Rare Disease Column: Guillain-Barre

Guillain-Barre may sound like a pub with a bit of a French flare, but this nasty little syndrome strikes seemingly at random and causes progressive muscle weakness, loss of sensation, paralysis, and in about 5% of cases, even death.

Guillain-Barre belongs to a class of poorly understood syndromes and disorders known as "autoimmune disease" - which basically means that your body's immune system has got its wire's crossed and is attacking you! A bit of friendly fire later and just about anything can go wrong with a body. In the case of Guillain-Barre, the immune system has set its sights on a very important part of the peripheral nervous system - myelin.

Myelin (see photo) is a layer of cells that cover the long branching fibers of nerve tissue, acting as both insulation and more importantly, speed enhancer for nerve signals. Nerves with myelin coverings transmit signals many times faster than nerves without myelin. That's why Guillain-Barre causes such devastating weakness and even paralysis - with the myelin damaged and out of commission, the signals from the brain to the body and back get crossed and move slower than your average arthritic turtle.

A neuron with its branching nerve fibers and myelin sheath.

All this leads to a rapid progression of weakness, generally starting in the feet and progressing up the body. The weakness can progress all the way to full paralysis. Weakness generally peaks by 2-3 weeks after onset of symptoms and can take a very long time to resolve completely. 30% of patients still experience weakness three years after their initial onset, and a small percentage of patients may have recurrence or relapse of symptoms many years after recovery.

So what causes Guillain-Barre? We'd like to know, too. The truth is, while we know it isn't contagious and it is caused by an auto-immune process, how it occurs is poorly understood. In most cases, symptoms begin within a few weeks of a respiratory or GI infection (cold, flu, stomach bug, vomitting, diarrhea). It makes sense, because after the immune system fights off an infection, it is vulnerable to other problems. Symptoms can also be triggered by surgery or immunizations - two more things that mess with your immune function.

The effects of this syndrome are temporary for most sufferers, but during the worst part of the weakness or paralysis, the person is totally dependant on others for daily needs. Even chewing food or breathing may be too demanding for sufferers of Guillain-Barre and it is not uncommon for artificial feedings, IV nutrition, and even respirators/ventillators to be employed. Guillain-Barre can be mild enough to require a great deal of assistance for a home recovery, but in most cases an admission to a hospital and often an intensive-care unit (ICU) is required.

Side effects of the syndrome - poor nutrition, loss of muscle tone, risk of falls, risk of pressure sores (bed sores), and the risk of failing respiratory effort - can lead to further complications. Recovery requires a great deal of physical therapy, and during the high point of the symptoms and thereafter, sufferers have a significant risk for being unable to emotionally cope with their sudden loss of function and dependence on others for basic needs. Emotional support and counseling are highly recommended.

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