Guillain–Barré syndrome

Description

Guillain-Barré syndrome is the most common and severe acute inflammatory paralytic neuropathy. The classical description of GBS involves rapidly progressive bilateral weakness, usually starting in the distal lower extremities and ascending proximally.

Within GBS, the two main phenotypes are of either an acute inflammatory demyelinating polyneuropathy or acute motor axonal neuropathy. Miller Fisher syndrome – which affects the cranial nerves resulting in facial, oculomotor or bulbar weakness – is recognised as a variant within the spectrum of disease in GBS.

GBS is usually preceded by infection or other immune stimulation, resulting in an aberrant autoimmune response which targets peripheral nerves and spinal roots. Predominant infection found to be Campylobacter jejuni; but cytomegalovirus, Epstein-Barr virus, influenza A virus, Mycloplasma pneumoniae and Haemophilus influenzae have all also been associated with GBS.

GBS is a potentially fatal disease, with 20-30% of cases affected by respiratory failure.Treatment involves meticulous supportive care, as well as intravenous immunoglobulin or plasma exchange

History of Guillain–Barré syndrome

1828 – The likely first reference to what would eventually be referred to as GBS, was by Auguste François Chomel (1788-1858) when he described an epidemic of acute polyneuritis in Paris (epidemie de Paris)

  • Chomel A-F. De l’épidémie actuellement régnante à Paris. Journal Hebdomadaire de Médecine. 1828; 1(9): 331-338.

1830 – Robert James Graves (1796 – 1853) witnessed the epidemic in July and August of 1828 in Paris. He praises Chomel’s description and similarly describes these cases:

It began (frequently in persons of good constitution) with sensations of pricking and severe pain in the integuments of the hands and feet, accompanied by so acute a degree of sensibility, that the patients could not bear these parts to be touched by the bed-clothes. After some time, a few days, or even a few hours, a diminution or even abolition of sensation took place in the affected members, they became incapable of distinguishing the shape, texture, or temperature of bodies, the power of motion declined, and finally they were observed to become altogether paralytic.

Graves R. Clinical lectures p380-381

1834 – Scottish surgeon, James Wardrop (1782-1869) described a case of ascending paralysis in a healthy male. Wardrop treated this patient with laxatives (colocynth and calomel [mercury]) and noted a progressive restoration of motor function to the point that the patient eventually returned to normal function.

A robust-looking man, about thirty-five years of age, suddenly felt while walking in the street, a numbness in the calf of the left leg which gradually extended up the limb. In the evening the same day he had a similar sensation of numbness with loss of power in the left hand and arm. In a few days the same symptoms attacked the right leg and thing, and the right hand, and in ten days from the first symptoms making their appearance, he completely los the power of locomotion, and possessed no voluntary power but that of turning his head from side to side, and of moving his toes

Wardrop J. Clinical observations on various diseases Lancet, 1834

1859 – Despite these descriptions in the literature prior, Jean-Baptiste Octave Landry (1826 – 1865) has long been regarded as the first to provide case documentation of “acute ascending paralysis”. He was the first to correctly conclude that this was a disease affecting the peripheral nerves. Landry’s original treatment included volatile liniments (such as chloroform, turpentine, quinine), electrical stimulation and opium; as well as nourishment therapies including pork chops and warm Bordeaux wine.

1916 – Over a two week period in 1916, Guillain and Barré admitted two patients to the army hospital with motor difficulties, areflexia, preservation of cutaneous reflexes, parasthesias, muscle tenderness, alteration in nerve conduction and increased albumin in the absence of lymphocyte elevation (which differentiated it from the more common condition of poliomyelitis at that time). These clinical observations were confirmed with nerve conduction studies by the physician André Strohl (1887 – 1977).

Guillain and Barré termed the syndrome “notre syndrome” (our syndrome) and excluded Strohl, with there being no clear explanation for this. Some speculate it was related to his younger age, him not being a neurologist or him being from Alsace

1927 – The eponym, Guillain-Barré syndrome (GBS), was introduced by Drăgănescu and Claudian at the Congress of the Neurology Society of Paris (led by Barré) where Strohl’s name was not only omitted from the presentation title, but also removed from references to the original work.

Associated Persons
Alternative names
  • Landry’s ascending paralysis
  • Guillain – Barré syndrome [Drăgănescu 1927]
  • Guillain – Barré – Strohl syndrome
  • Landry-Guillain-Barré syndrome [Haymaker 1949]
  • Landry-Guillain-Barré-Strohl syndrome
Controversies

Despite being an original author on the seminal paper and contributing electrophysiological findings to demonstrate areflexia and altered nerve conduction, Strohl was omitted from further eponymous mentions of Guillain-Barré syndrome. No clear explanation of this omission has been identified, however a number of theories continue to circulate.

Possible explanations:

  1. Guillain and Barré published many articles throughout 1920 which did not include Strohl
  2. Strohl was a relatively newly graduated doctor at the time of the first publication, having only qualified 3 years before
  3. Strohl was not purely a neurologist by profession (including physiology and radiology), and it may have been felt that including his name reduced its standing amongst colleagues
  4. Strohl was the child of parents from Alsace
References

Original articles

Review articles

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Physician in training. German translator and lover of medical history.

BA MA (Oxon) MBChB (Edin) FACEM FFSEM. Associate Professor Curtin Medical School, Curtin University. Emergency physician, Sir Charles Gairdner Hospital.  Passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | Twitter |

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