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Guillain-Barré & Associated

Inflammatory Neuropathies

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History of GBS

Descriptions of progressive numbness and weakness over a short period followed by spontaneous recovery exist in medical papers of the early 19th century. However the peripheral nervous system was little understood so no informed explanation for the symptoms was possible. It was not until 1848 (Graves) that it was suggested that such paralysis came from within the nervous system.

 

Landry's Ascending Paralysis
 
Certainly the best description of 'ascending paralysis' in this period was made by a Frenchman named Jean Baptiste Octave Landry de Thézillat in 1859.

Landry's description was based on ten cases, five of his own and five from the medical literature. In one case, Landry gave a long description of a patient who eventually died of asphyxia. Landry's superior, a Dr Glauber who had admitted the patient, had diagnosed hysteria but Landry predicted the patient's demise at an early stage.

Landry offered no explanation as no abnormality was found during a post mortem. Glauber added a note however, speculating a close connection between Landry's cases and the paralysis that follows dyptheria.

While Guillain-Barré syndrome was subsequently found to be unconnected with dyptheria, Glauber's observation was vindicated as both types of paralysis are due to a demyelinating neuropathy.

A translation of Landry's paper reads:

'The sensory and motor systems may be equally affected. However the main problem is usually a motor disorder characterised by a gradual diminution of muscular strength with flaccid limbs and without contractures, convulsions or reflex movements of any kind. In almost all cases micturition and defaecation remain normal. One does not observe any symptoms referable to the central nervous system, spinal pain or tenderness, headache or delirium. The intellectual faculties are preserved until the end. The onset of the paralysis can be preceded by a general feeling of weakness, pins and needles and even slight cramps. Alternatively the illness may begin suddenly and end unexpectedly. In both cases the weakness spreads rapidly from the lower to the upper parts of the body with a universal tendency to become generalised.

'The first symptoms always affect the extremities of the limbs and the lower limbs particularly. When the whole body becomes affected the order of progression is more or less constant: (1) toe and foot muscles, then the hamstrings and glutei, and finally the anterior and adductor muscles of the thigh; (2) finger and hand, arm and then shoulder muscles; (3) trunk muscles; (4) respiratory muscles, tongue, pharynx, oesophagus, etc. The paralysis then becomes generalised but more severe in the distal parts of the extremities. The progression can be more or less rapid. It was eight days in one and fifteen days in another case which I believe can be classified as acute. More often it is scarcely two or three days and sometimes only a few hours.

'When the paralysis reaches its maximum intensity the danger of asphyxia is always imminent. However in eight out of ten cases death was avoided either by skillful professional intervention or a spontaneous remission of this phase of the illness. In two cases death occurred at this stage . . . When the paralysis recedes it demonstrates the reverse of the phenomenon which signaled its development. The upper parts of the body, the last to be affected, are the first to recover their mobility which then returns from above downwards.'

The term 'Landry's ascending paralysis' was first used in 1876 (Westpahl). The usual treatment was with strychnine which probably did the unfortunate patients more harm than good. Landry contributed no more to neurology for he died of cholera just six years after publishing his paper.

 

Acute Febrile Polyneuritis

Acute febrile polyneuritis was one of six classes of polyneuropathy proposed by Ostler in 1892. Ostler considered that some of Landry's patients had fallen into this category while others had suffered from myelitis (inflammation of the myelon [spinal cord]).

Ostler's description was of an illness similar to what we now call GBS but with the fundamental difference of showing a fever. In 1918, Bradford et al described 'acute infective polyneuritis'. They stated this to be the same as acute febrile polyneuritis, any fever having recovered before the onset of the neurological symptoms or being due to subsequent infection.

In the meantime, developments had been published in France...

 

                         

 

 

 

Georges Guillain

                   Jean-Alexandre Barré

           André Strohl

 

Guillain and Barré were medical students together at the Saltpêtrière in Paris at the turn of the century and specialised in neurology. During the First World War, they were both serving as doctors in the French Army. They noted the cases of two soldiers who had become partially paralysed. One, in particular, had fallen over when he had put his pack on and had been unable to get up. Both the soldiers quickly recovered, possibly assisted by treatment with pork chops and claret.

Together with Strohl, they published their classic paper in 1916. It was noted that reflexes were reduced and that the protein level in the cerebrospinal fluid was raised though this was not accompanied with a high white blood cell count. This was a crucial discovery as two common infections of the time, syphilis and tuberculosis, would have shown such an increase.
The cause of the condition was left unanswered, assumed to be some unknown kind of infection or poisoning. Guillain personally was not convinced that the condition that he and his partners had described was the same as Landry's. Landry had noted how the condition could cause respiratory failure but Guillain saw no such evidence and believed the illness he had described was not particularly serious.
 
Guillain-Barré Syndrome
 
After World War One, doctors were faced with three similar conditions with slightly different definitions: Landry's ascending paralysis, acute febrile (or perhaps infectious) neuropathy and the radiculoneuritis described by Guillain et al.
It was in 1927 when the term Guillain-Barré syndrome was first used at a presentation by Dragonescu and Claudian. Their presentation was introduced by Barré himself but Strohl's name was omitted not only from the title of the presentation but also from the list of authors in the reference to the 1916 paper.
Later, and inevitably, it was suggested that the three conditions were one but Guillain would not countenance it. He emphasised that fever was not in his description, that the essential element of his definition was the raised cerebrospinal protein level which Ostler and others had not mentioned (because lumbar punctures had not been introduced), and that Landry's cases were a miscellany of conditions including poliomyelitis and encephalomyelitis.
In 1949 Haymaker and Kernohan suggested a wider definition of the illness, suggesting that Landry's ascending paralysis and Guillain-Barré syndrome were indistinguishable and called the condition Landry-Guillain-Barré syndrome. Guillain, who two years previously had retired from his position of Professor of Neurology at the Saltpêtrière, was outraged and continued to stress his own narrower definition.
Guillain's last paper was in 1953. He believed the syndrome to be generally benign though the death of a patient, who after post-mortem was found to have extensive peripheral nerve damage, had necessitated a shift in his position. Guillain still suspected an unknown infection as the cause and dismissed suggestions, made ten years earlier (Bannwarth), that the cause was due to allergy.
 
Later Developments
 
In 1956, Charles Miller Fisher, a Canadian neurologist, described three patients with acute external ophthalmoplegia (eye paralysis), sluggish pupil reflexes, ataxia (lack of balance) and areflexia (absent tendon reflexes). Two patients had no weakness; the other had a facial palsy and possible weakness. All three recovered spontaneously.

Because some patients with GBS had ophthalmoplegia and there were other similarities, Dr Fisher concluded that these patients had suffered a disorder akin to GBS.

In 1958, a paper was published by Dr JH Austin who described a chronic form of GBS (recurrent polyradiculoneuropathy). Austin's paper was based on a review of 30 cases, the earliest of which went back to 1894, and on two of his own. This chronic form has gone through a variety of names (chronic inflammatory polyradiculoneuropathy - CIP - Dyck et al 1975) and attempts to define it though it now generally known as CIDP (Dyck et al 1982).

Guillain died in 1961 and Barré in 1967. Having first published the relationship between ascending paralysis and an increased protein count in cerebrospinal fluid in 1916, they had seen a huge increase in knowledge as well as witnessed the use of early intensive care techniques.

In the 1980s, western doctors were astonished to learn of a yearly GBS-type epidemic amongst children in a province of China. Investigations in China and in other rural communities around the world confirmed the existence of a variant or possibly variants of GBS that attacked the nerve axons rather than the myelin sheaths. It was also discovered that the bacterium Campylobacter jejuni was of major importance. Since then, an enormous amount of research has been conducted into isolating the particular antibodies responsible for the different 'syndromes').

So until the day when everything is known and redefined, Guillain and Barré will continue to receive the recognition. But spare a thought for poor neglected Strohl who history has ignored and consider too the work of Landry, the victim of an early death from an illness he contracted from his own patients