Guillain–Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy, an autoimmune disorder affecting the peripheral nervous system, usually triggered by an acute infectious process. The syndrome was named after the French physicians Guillain, Barré and Strohl, who were the first to describe it in 1916. It is sometimes called Landry’s paralysis, after the French physician who first described a variant of it in 1859. It is included in the wider group of peripheral neuropathies. There are several types of the Barré syndrome, but unless otherwise stated, GBS refers to the most common form, AIDP. The Barré syndrome is rare and has an incidence of 1 or 2 people per 100,000. It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and autonomic nervous system problems are present. Guillain-Barré is one of the leading causes of non-trauma-induced paralysis in the world.

Cause

All forms of Barré syndrome are due to an immune response to foreign antigens (such as infectious agents) that are mistargeted at host nerve tissues instead. The targets of such immune attack are thought to be gangliosides, compounds naturally present in large quantities in human nerve tissues. The most common antecedent infection is the bacterium Campylobacter jejuni. However, 60% of cases do not have a known cause; one study suggests that some cases are triggered by the influenza virus, or by an immune reaction to the influenza virus.

The end result of such autoimmune attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances.

However, in mild cases, nerve axon (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage occurs, and recovery depends on the regeneration of this important tissue. Recent studies on the disorder have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disorder is indeed axon loss.

Barré, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig’s disease (ALS), is a peripheral nerve disorder and does not generally cause nerve damage to the brain or spinal cord.

Prognosis

Most of the time recovery starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5–10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disorder and despite all improvements in treatment and supportive care, the death rate among patients with this disorder is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 5–10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP).

Poor prognostic factors include: 1) age >40 years, 2) history of preceding diarrheal illness, 3) requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb muscle strength.

Case reports do exist of rapid patient recovery.

Epidemiology

The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population. The mother will generally improve with treatment but death of the fetus is a risk. The risk of Guillain–Barré syndrome increases after delivery, particularly during the first two weeks postpartum. There is evidence of Campylobacter jejuni as an antecedent infection in approximately 26% of disease cases, requiring special care in the preparation and handling of food. Congenital and neonatal Guillain–Barré syndrome have also been reported.

History

The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry’s ascending paralysis and Landry Guillain Barré syndrome.

barre syndrome

Barre Syndrome, Image:Chris Zahniser, B.S.N., R.N., M.P.H.

This photograph depicted a girl who’d been suspected of suffering from acute flaccid paralysis, which turned out to be Guillain-Barre Syndrome rather than polio. The photo, taken following her recovery, showed her standing outside in her home village with her father.

 

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