Syndrome of Guillain-Barred

The Syndrome of Guillain - Barred or syndrome of Guillain-Barred-Strohl is a auto-immune Maladie inflammatory peripheral Nervous system. It is an acquired disease. It is also called inflammatory acute polynevropathy démyélinisante , polyradiculonévrite acute idiopathic , polynévrite acute idiopathic and ascending paralysis of Landry .

General information

The disorder, commonly called Syndrome of Guillain-Barred is a rare disease which affects the peripheral nerves of the organization. It is characterized mainly by a weakness, even a paralysis and is often accompanied by abnormal feelings. The syndrome appears in a sporadic way. It is unforeseeable and can occur at any age, independently of the sex. Its gravity can even vary considerably average case being able not to be made available of a doctor, with that of a disease devastator related to an almost total paralysis placing the patient between the life and death. In spite of its scarcity, the doctors are trained to diagnose the disease (even if they very often forget the characteristics of this syndrome) the more so as it can appear very invalidating for the touched people.

Prevalence

1 to 2 per 100.000 inhabitants per annum.

Cause

The origin of the Syndrome of Guillain-Barred is not known. A certain number of demonstrations seem to start the disease. Many cases occur a few days, even a few weeks after a viral infection. These infections can go of a banal cooling, with evils of throat while passing by gastric and intestinal pains, accompanied by diarrheas. Certain cases were associated with specific infectious agents. Infections with Campylobacter jejuni often precede the syndrome by Guillain-Barred and are associated with a degeneration of the axons with slow recovery, and a severe residual handicap. However, it (S) mechanism (S) by (S) which (S) these micro-organisms can cause the Syndrome Guillain-Barred (A) was not given (S) yet. Certain cases appeared simultaneously with a rare disease of the red globules, the porphyrie. Certain cases similar to the Syndrome of Guillain-Barred occurred afterwards of the events apparently not reported such as the surgery, the insect bites and various injections. Certain cases appeared during the winter 1976-77 at people who had been vaccinated against the porcine influenza, vaccination common anti-flu itself being responsible for exceptional cases.

The Syndrome of Guillain-Barred can also occur during the pregnancy. Fortunately, according to an UK trial, repeated episodes are usually not represented at the time of future pregnancies. Some eruptions or grouped cases of Syndrome of Guillain-Barred were reported, including estival epidemics in children of the North of China, an eruption in Jordan in 1978 after an exposure to polluted water, and an eruption in Finland following a national campaign of oral vaccination against the virus of the poliomyelitis. In these various cases, it was difficult to identify a specific agent responsible for the appearance of the Syndrome of Guillain-Barred.

The Syndrome of Guillain-Barred and chronic idiopathic neuropathy can, on rare occasions, to develop among patients reached of other systemic diseases.

It is interesting to know that literally million people was exposed to events such as infections, the surgical operations and vaccines identified like agents responsible for the Syndrome for Guillain-Barred. And yet, only very a small number of people exposed to these episodes develop the Syndrome Guillain-Barred.

One does not know clearly why only certain people develop the Syndrome. Would they present a single genetic predisposition? This possibility does not seem probable since the Syndrome of Guillain-Barred does not appear within the same family.

Research carried out to date indicates that independently of the responsible agent, the nerves of the patient reached of the Syndrome of Guillain-Barred are attacked by its own immune system against certain antibodies of the disease and by white globules. Following this auto-immune attack, the sheath of the nerve (the myéline) and sometimes also the covered part of the nerve ensuring conduction (the axon) are damaged and the signals are slowed down or modified. Abnormal feelings and a weakness result from it.

Because the Syndrome of Guillain-Barred often appears after a viral disease, it is thought sometimes wrongly that it is contagious. However, there does not exist any element which would prove that it can be contagious, even if a person had contacts with the patient during the preceding viral infection. In fact, the virus often disappeared when it Syndrome develops.

Signs and symptoms

The gravity of the Syndrome of Guillain-Barred can vary considerably. In its light form, it can cause a swelling or a step of duck and possibly of swarmings and a weakness of the upper limbs being able to obstruct, briefly or lasting of the days or the weeks, the patient in his life of the every day. Certain doctors, general practitioners or family practitioners and internists, described certain patients complaining about fairly short swarmings and/or a weakness about the members accompanying or following a viral disease, like evils of throat or diarrheas. These joined together symptoms can constitute a very light form of the Syndrome of Guillain-Barred.

Contrary to these very light forms, a patient presenting the Syndrome Guillain-Barred can, to the other extreme, completely paralyzed being and to present many complications, among which incapacity to breathe, an abnormal pulse, an abnormal blood-pressure, a congestion of the lungs, infections, and blood clots endangering the life of the patient. A patient is only seldom struck total paralysis, at the point to find itself unable would be this only to stir up a finger, to raise the shoulders or to make a wink. Such patients can be “locked up indeed” or incompetents to communicate. They can still hear, which makes it possible to the patient to communicate with his family and the medical personnel.

Since the gravity of the Syndrome of Guillain-Barred is very variable, it is difficult to give a description of an average patient. Descriptions which one finds in the literature concerning of great groups of patients let suggest that a “standard” patient can be sick and treaty in an health care center acute and then in a center of rehabilitation, while being followed by a programme of rehabilitation except hospital lasting 3 to 12 months. Among these patients, 40% perhaps require a ventilation assisted because of the weakness of the respiratory muscles. More than 50% certainly, and probably more than 90% of the patients end up curing completely or almost and continue to live like front.

From 5 to 15% of the patients will remain invalid with more or less long run. approximately 35% will be perhaps struck light anomalies in the long run, like the drop on the level of the foot or the dizzy spells. Up to 5% can die, usually of pulmonary complications (respiratory) or cardiovascular.

Diagnosis

The criteria of which it is necessary to hold account to establish the diagnosis of the syndrome of Guillain-Barred can be summarized as follows:

fast Appearance, from a few days at three (or four) weeks of a weakness, leading sometimes to a frank paralysis, affecting the two sides of the body in a relatively identical, and typically ascending way to touch the legs, then the arms, and sometimes even respiratory muscles and the face.

Often, this is accompanied by sensory anomalies, such as reduced feelings, numbnesses, swarmings or pains.

Loss or marked enough reduction of the tendineux reflexes.

Protéinorachie, usually at the 10th day following the appearance of the symptoms, accompanied by a normal account of cells of the céphalo-rachidian liquid (it is the " dissociation albumino-cytologique"). Its observation by analysis of a taking away of the céphalo-rachidian liquid, constitutes the element of diagnosis more the discriminant.

Electromyography: velocity measurement of nervous conduction (VCN-EMG) highlighting a deceleration or an absence of nervous conduction.

Absence of very other causes justifying the presence of a peripheral Neuropathie, like: absence of anamnèse concerning the inhalation of a organic solvent, absorption of Lead or some Drug S, such as the Nitrofurantoïne or the Dapsone; miss evidence of the infectious causes of neuropathies, as observed in the Diphtérie, the Poliomyélite; miss acute proof of Porphyrie intermittent; analyzes of Urine S normals for the metabolites of Porphyrin S.

Treatment

Plasmaphérèses

The Plasmaphérèse S (plasmatic exchanges or subtractions) were used in several serious cases of the syndrome of Guillain-Barred. The results of a great multicentric study on the effects of the plasmaphérèses on the patients seriously reached of the syndrome of Guillain-Barred, published in 1985, indicate that on average, the forecast of the patients treated by plasmaphérèses was better than that of the patients not having profited from this treatment. They remained less longer under artificial respiration and remarchaient more quickly than the untreated patients. One can consequently conclude from it that the plasmaphérèses seem to be a promising treatment of the syndrome of Guillain-Barred.

A certain prudence is essential when one proceeds to the plasmaphérèses. This technique indeed requires special equipment which is available only in large hospitals and medical centres. This therapy should probably be under consideration for patients carrying the syndrome Guillain-Barred with the whole beginning of their disease if they are seriously reached or if their state is degraded. It would be preferable that the attending physician makes the decision to proceed to plasmaphérèses according to each patient, after having considered all the aspects of its situation. Many meetings (theoretical): on average 4, if need be 6 and only 2 if the form is not very severe.

Gammaglobulines

In 1988 and 1989, some researchers reported the beneficial effects of high amounts of gammaglobulines (or Immunoglobuline S) in the treatment of a restricted number of patients seriously reached by the syndrome of Guillain-Barred. As of the month of August 1990, the group of studies Dutch of the syndrome of Guillain-Barred brought back the preliminary results of a great study, indicating that the therapy by intravenous injection of gammaglobulines would seem at least as effective as the plasmaphérèse. The therapy by immunoglobulins has some inherent advantages compared to the plasmaphérèse, among which the absence of need for specialized equipment and qualified personnel and thus the possibility of managing this treatment more easily.

Other treatments

Cortisone with the long course often gives good performances and Immunosupresseurs (or Immunodépresseurs) as Azathioprine is proposed in the event of Corticorésistance or cortico-dependence like in the chronic cases.

The purpose of the majority of the other treatments are to prevent or treat the complications of the syndrome of Guillain-Barred. For example, the paralyzed patient, confined to bed, is predisposed with various problems which can often be avoided.

Forecast

6 to 10% of mortality (the vital prognosis is influenced by the respiratory disorders, the disorders of swallowing, the disorders tensionnels and the complications of the confinement). Risk long-term after-effects according to the cases.

History

In 1859, a French doctor, Jean Landry, described in details a nervous disorder paralyzing the respiratory legs, arms, neck and muscles. Several reports/ratios of a similar disorder arrived of other countries. The proof of Quinke in 1891, after a taking away of the céphalo-rachidian liquid by means of a needle placed in the bottom of the back, opened the way with three Parisian doctors, Georges Guillain, Jean Alexandre Barré and André Strohl which showed in 1916, the anomaly characteristic of an increase in proteins of the céphalo-rachidian Liquide with normal numeration of the cells among two soldiers with transitory generalized paralysis. Since then, several researchers gathered extra informations on this subject. In order to facilitate communication between doctors and patients, one uses simply the term “Syndrome of Guillain-Barred”.

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