Guillain Barré SyndromeGBS/CIDP des

Guillain Barré Syndrome
GBS/CIDP described
GBS is a syndrome; i.e. it is a collection of symptoms and signs. It is not a single disease, but rather consists of serveral related diseases. It is a rare disorder affecting 1–2 people per 100,000 fairly consistently around the world. This implies that about 40 to 80 New Zealanders are affected each year although recent research has suggested this may be as high as 100 annually. All of these diseases have in common an immune attack on the components of the peripheral motor sensory nervous system resulting in rapidly progressive weakness, sometimes progressing to severe or even complete paralysis. Progression of the disease always stops within 4 weeks and there is a prolonged recovery phase.
Treatment

The is no prevention or cure as such for GBS and it usually gets better on its own over time with the demyelinated nerves repairing themselves. No drugs have been proven to make any difference to the speed of recovery, but good nursing and, for the more severe cases, early treatment using one of the two available blood related treatments, may shorten the duration of the illness and improve the ultimate outcome for the patient.

The two treatments are plasma exchange (plasmaphereses) where over a period of days the patient's blood is passed through a machine where the antibodies in the plasma that are attacking the nerves are removed. The alternative is gamma globulin (also called immunoglobulin – IvIg), which is a human blood product and is given intravenously over a period of days. This treatment is expensive but more simple than plasma exchange.

CIDP patients can also benefit from the use of some drugs such as steroids and azathioprine, which aim to suppress the damaging autoimmune reaction, though this has to be balanced against the risk of suppressing the normal immune response to infections.

Physiotherapy is vital to minimise muscle wasting and to regain muscle strength and activity, but exercise must be balanced with the need for rest.

Recovery can take anything from a few weeks to several months – the average is 3 to 6 months. Two thirds of those who suffer from GBS recover completely. The remainder can be left with varying degrees of weakness and discomfort. In most cases this does not affect people's lives seriously. A few however are unable to go back to their former occupation because of residual disabilities, for example, hand weakness, foot drop or the need for walking aids. In these cases long term rehabilitation may be required.