Dear Doctor,

Three months ago, my husband was diagnosed with Guillain-Barre syndrome. He is 36 years old. The onset was gradual, beginning with numbness in his hands and feet, and severe pain in his back. After an MRI didn’t reveal any nerve problems in his spine, he was referred to a neurologist and began an IV of immunoglobulin. His condition improved dramatically, but two weeks ago, the symptoms returned, and he began a series of IVs again.

He feels small amounts of tingling or numbness in his toes and fingers. He gets very weak, and struggles to get up and go to work every day. Should he continue with this treatment, or try something else? Also, could his symptoms be an effect of Guillain – Barre syndrome, or could there be another problem? Lastly, is there any relation between Guillain – Barre syndrome and other autoimmune disorders?

Claire Ntege

Dear Claire

Gullain Barre syndrome is a malady causing sudden weakness of limbs due to affection of mostly peripheral nerves. There may be some pain, tingling and other abnormal sensations for a day or two, followed by sudden weakness of limbs. Mostly legs are affected but upper limbs can also be involved. Involvement of respiratory muscles can cause death due to respiratory failure. In case of damage to autonomic nervous system, there can be fluctuations in heart rate and blood pressure.

Cranial nerves can be involved resulting in difficulty in swallowing and or visual troubles. Neck muscles may be involved. Death can occur due to blood clot in lungs, superadded bacterial infection, pneumonia, respiratory paralysis or cardiac arrest. There are different variations of clinical features depending on which nerves are affected, but basic manifestation is paralysis in majority of cases.

It is said to occur due to immune mediated damage to the nerves caused due to some infection. The cover of nerves is damaged, leading to their dysfunction, this weakens the muscles supplied by them manifesting as paralysis. Mostly symptoms like flu or diarrhea and vomiting precede it. Campylobacter jejuni, cytomegalo virus influenza virus, some strains of herpes, among others, are the microbes implicated.

Gullain Barre syndrome is a very rare condition (one to two cases/100,000 individuals) and affects more men than women. It can occur at any age. Once paralysis starts, it plateaus in a week or so. After about four weeks or so, the symptoms may start to improve and there is complete recovery within four to six weeks to six months.

Death is rare (5 per cent cases). The diagnosis is established by excluding other causes for the sudden paralysis. Examination of cerebrospinal fluid, for example, fluid removed from backbone reveals high protein count as compared to cell count. Tests like electromyography and nerve conduction studies show the damage to nerve and or muscle.

There is no definitive treatment. Plasmapharesis is an intervention done in which blood is removed from the body, red and white blood cells are separated and put back in body minus plasma(liquid portion of the blood), which is formed quickly again. The exact mechanism on how this is helpful is not known but it does help. Probably the triggers causing nerve damage are removed with the plasma. This process is cumbersome and hazardous.

Another way of treatment is injection of IV immunglobulin. This probably helps by boosting body’s immune system and removing implicating microbes. This is safer and more easy to use.

The person may improve over weeks to months spontaneously as well. During the time when he is sick it is important to ensure proper feeding and nursing care to avoid malnutrition, dehydration and bed sores.

Physiotherapy is crucial. It helps the paralysed muscles to regain their normal tone and strength. Abnormal sensations take some time to recover, but ultimately improve. Extensive research is being carried out to see association of this malady with other autoimmune disorders.

Dr. Rachna is a specialist in internal medicine at Ruhengeri Hospital.