Guillain-Barré-Strohl syndrome

Alternative eponyms

Related people

The commonest form of acquired neuropathy, most frequently affecting young adults. Commences with progressive muscular weakness of extremities that may lead to paralysis.

Description

The commonest form of acquired neuropathy affecting both sexes, most frequently affecting young adults and believed to be a form of autoimmune disease with a delayed hypersensitivity reaction.

Commences with progressive muscular weakness of extremities that may lead to paralysis. It spreads rapidly, ascending to involve the cranial nerves. Commences distally but spreads proximally and may involve the bulbar region and the diaphragm. Clinical features are pain in the back and the legs and weakness beginning in the feet and legs and progressing upwards. The respiratory muscles are affected in about half the cases, and this puts the patient in danger. The tendon reflexes are lost.

Recovery after a period varying from several weeks or months is the usual outcome. There is no specific therapy. Usually the spinal fluid contains an increased amount of protein but only rarely will the cells in the fluid be increased.

Before the invention of the respirator this disease was mortal in more than half of the cases.

The disturbance was clinically described by Auguste François Chomel (1788-1858) in 1828, James Wardrop (1782-1869) in 1834, and Robert James Graves (1797-1853) in 1848.

Guillain, Barré, and Strohl in 1916 described two soldiers acutely ill with muscular weakening, paresthesias, and muscular pain. The patients, 25 and 35 years of age, were thoroughly examined by Georges Guillain, head of the Centre Neurologique de VIe Armé. The great novelty in their examination, however, was done by Strohl, who investigated the spinal liquid and performed an electrophysiological test, finding major pathology in the Achilles reflex and the quadriceps muscle. The soldiers’ condition soon improved, however, and after a little over a month they were both released from the hospital, almost completely restituted.

Only weeks after Guillain, Barré, and Strohl, Marie and Chatelin reported three cases, Their patients were all restituted within three months.

It was H. Draganesco and J. Claudion who, in 1927, first used the term Guillain-Barré syndrome. Guillain and Barré obviously enjoyed this, forgetting both Strohl and Landry. Guillain contended that since lumbar puncture was not practiced at Landry’s time, the linking of Landry’s name with theirs spelled “une condition nosographique absolue.”

A variant characterized by ophthalmoplegia, ataxia, and areflexia is called the Miller Fisher syndrome. See under Miller Fisher, Canadian neurologist, born 1910.

Bibliography

  • Auguste François Chomel, in Journal Hebdomadaire de Médecine, 1828, 1: 333.
    An epidemic of acute polyneuritis formed the basis for Chomel's original description.
  • James Wardrop, in the Lancet, 1834. J. B. O. Landry:
    Note sur la paralysie ascendante aiguë.
    Gazette hebdomadaire de médecine et de chirurgie, 1859, 6: 472-474, 486-488.
    In his article Landry described ten cases of ascending paralysis, of which two died of asphyxiation.
  • A. Kussmaul:
    Zwei Fälle von Paraplegie mit tödlichem Ausgang ohne anatomisch nachweisbare oder toxische Ursache. Erlangen, 1859.
  • G. Guillain, J. A. Barré, A. Strohl:
    Le réflexe médico-plantaire: Étude de ses caracteres graphiques et de son temps perdu.
    Bulletin et mémoires de la Société des Médecins des Hôpitaux de Paris, 1916, 40: 1459-1462.
  • G. Guillain, J. A. Barré, A. Strohl:
    Sur un syndrome de radiculonévrite avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire. Remarques sur les caractères cliniques et graphiques des réflexes tendineux.
    Bulletins et mémoires de la Société des Médecins des Hôpitaux de Paris, 1916, 40: 1462-1470.
  • Pierre Marie, C. Chatelin:
    Note sur un syndrome de paralysie flasque plus ou moins généralisée avec abolition des reflexes, hyperalbuminose massive et xantochromie vers la guerison et de nature indéterminée.
    Revue neurologique, Paris, 1916, 30: 564-565.
  • H. Draganesco, J. Claudian:
    Sur un cas de radiculu-névrite curable (syndrome de Guillain-Barré) apparue au cours d’une ostéomyélite du bras.
    Revue neurologique, Paris, 1927, 2: 517-521.
  • G. Guillain, J. A. Barré:
    Quelques remarques sur notre “syndrome de radiculonévrite avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire.
    Revue neurologique, Paris, 1936, 65: 573-582.
  • G. Guellain:
    Considérations sur le syndrome de Guillain et Barré.
    Annales de médecine, 1953, 54: 81-92.
  • Joseph Heller, S. Voger:
    No Laughing Matter. London, Jonathan Cape, 1986.
    The author Joseph Heller (1923-), famous for his novel “Catch 22”, became affected with the syndrome in 1981.

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