Guillain–Barré syndrome

Landry-Guillain-Barré-Strohl syndrome (GBS) is the most common and severe acute inflammatory paralytic neuropathy worldwide. The syndrome is classically characterised by rapidly progressive, symmetrical weakness with hyporeflexia or areflexia, frequently ascending from the lower extremities.

GBS encompasses several clinical variants, primarily acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The Miller Fisher syndrome, marked by ophthalmoplegia, ataxia, and areflexia, is considered a variant of GBS. The syndrome is typically post-infectious, often associated with Campylobacter jejuni, influenza, CMV, EBV, and other pathogens.

The eponym reflects contributions from Jean-Baptiste Octave Landry (1859), who first described “ascending paralysis”, and Georges Guillain, Jean-Alexandre Barré, and André Strohl, who refined its clinical, electrophysiological, and cerebrospinal fluid (CSF) characteristics in 1916. Notably, Strohl’s contributions were historically underrecognised in the naming of the syndrome.

Over time, GBS has become a model disease for understanding autoimmune neuropathies. Advances in immunotherapy, such as IVIG and plasma exchange, have significantly improved outcomes, though mortality remains 3–6%, and 10–15% of survivors may experience permanent deficits.

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) He described a “sensation of pricking and pain in the extremities, progressing to paralysis.”

1828 – Robert James Graves (1796-1853) witnessed the epidemic in July and August of 1828 in Paris. Graves describes cases of sensorimotor polyneuropathy, expanding awareness of peripheral nerve contributions to paralysis. He praises Chomel’s description and 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 prior descriptions, 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.

Landry published Note sur la paralysie ascendante aiguë describing 10 cases of acute ascending paralysis, distinguishing between sensory and motor subtypes. “Ascending paralysis with or without sensory involvement… no spinal cord pathology.”

1859 – Adolf Kussmaul (1822–1902) experiences and reports on a similar syndrome in Zwei Fälle von Paraplegie mit tödlichem Ausgang ohne anatomisch nachweisbare oder toxische Ursache; later associated with the eponym Landry-Kussmaul paralysis.

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).

The syndrome is characterised by motor disorders, abolition of the tendon reflexes with preservation of the cutaneous reflexes, paraesthesias with slight disturbance of objective sensation, pain on pressure of the muscle masses, marked modifications in the electrical reactions of the nerves and muscles, and remarkable hyperalbuminosis of the cerebrospinal fluid with absence of cytological reaction (albuminocytological dissociation).

This syndrome seemed to us to depend on a concomitant injury of the spinal roots, the nerves, and the muscles, probably of infectious or toxic nature

Guillan, Barré, Strohl. 1916

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 – Drăgănescu and Claudian introduce the term Guillain-Barré syndrome (GBS), omitting Strohl and earlier contributors at the Congress of the Neurology Society of Paris (led by Barré). Strohl’s name was not only omitted from the presentation title, but also removed from references to the original work. Sur un cas de radiculo-névrite curable (syndrome de Guillain et Barré) apparue au cours d’une osteomyelite du bras

1936 – Alajouanine et al. describe segmental mononuclear infiltration in peripheral nerves, confirming the inflammatory nature of GBS.

1949 – Haymaker and Kernohan propose the eponym Landry-Guillain-Barré syndrome, based on pathological correlation.

1956 – Charles Miller Fisher (1913-2012) describes the Miller Fisher variant of GBS: ophthalmoplegia, ataxia, and areflexia.

1978 – NINCDS diagnostic criteria for GBS are established after the U.S. influenza vaccine-related GBS epidemic.

1978–1985 – Plasmapheresis demonstrated as effective in GBS (Hammersmith Hospital; GBS Study Group USA).

1988 – IVIG shown to be effective as first-line therapy for GBS (van der Meché et al.).

Associated Persons

• Auguste François Chomel (1788-1858) – Early epidemic description.
• Robert James Graves (1796-1853) – Peripheral neuropathy recognition.
• James Wardrop (1782-1869) – Case report of ascending paralysis.
• Jean-Baptiste Octave Landry (1826-1865) – Ascending paralysis.
• Adolph Kussmaul (1822-1902) – Personal experience and early reports.
• Georges Charles Guillain (1876-1961) – 1916 seminal description.
• Jean-Alexandre Barré (1880-1967) – 1916 seminal description.
• André Strohl (1887-1977) – 1916 seminal description.
• Charles Miller Fisher (1913-2012) – Miller Fisher variant of GBS

Alternative names

• Landry’s ascending paralysis [Introduced by Karl Friedrich Otto Westphal (1833–1890)]
• Guillain-Barré syndrome [Drăgănescu 1927]
• Guillain-Barré-Strohl syndrome
• Landry-Guillain-Barré syndrome [Haymaker 1949]
• Landry-Guillain-Barré-Strohl syndrome

References

Original articles

• Chomel A-F. De l’épidémie actuellement regnante à Paris. Journal Hebdomadaire de Médecine. 1828; 1: 333.
• Graves RJ. Pathology of Nervous Diseases. In: Clinical lectures on the practice of medicine (2e). 1848; II: 504-505.
• Wardrop J. Loss of voluntary motion successfully treated by Purgatives. In: Clinical observations on various diseases. Lancet 1834; 23(588): 380-381
• Landry JBO. Note sur la paralysie ascendante aiguë. Gazette hebdomadaire de médecine et de chirurgie, 1859; 6(30): 472-474 and 1859;6(31):486-488
• Kussmaul A. Zwei Fälle von Paraplegie mit tödlichem Ausgang ohne anatomisch nachweisbare oder toxische Ursache. [Two cases of paraplegia with fatal outcome without anatomical demonstrable or toxic cause] Erlangen, 1859.
• Guillain G, Barré JA, Strohl A. Sur un syndrome de radiculo-névrite 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 médicale des hôpitaux de Paris, 1916; 40: 1462-1470

Review articles

• Bailey P, Ewing J. A contribution to the study of acute ascending (Landry’s) paralysis. New York. 1896.
• Cowan J, Ballantyne JA, MacDonald D. A case of Landry’s paralysis. 1909.
• Draganescu S, Claudian J. Sur un cas de radiculo-névrite curable (syndrome de Guillain et Barré) apparue au cours d’une osteomyelite du bras. Revue Neurologique 1927; 2: 517-519
• Viets HR. History of peripheral neuritis as a clinical entity. Arch NeurPsych. 1934; 32(2): 377-394
• Haymaker W, Kemohan JW. The Landry-Guillain-Barre syndrome: a clinicopathologic report of fifty fatal cases and a critique of the literature. Medicine 1949; 28: 59-141.
• Pearce JMS. Octave Landry’s ascending paralysis and the Landry-Guillain-Barre-Strohl syndrome. J Neurol Neurosurg Psychiatry. 1997; 62: 495, 500.
• Afifi K. The Landry-Guillain-Barré Strohl Syndrome 1859 to 1992 A Historical Perspective. J Family Community Med. 1994; 1(1): 30–34
• Young P. Landry, Kussmaul, and Guillain-Barré-Strohl syndrome. Medical Journal of Chile, 2014; 142(7): 930-931
• Nickson C. Guillain-Barré syndrome (GBS). CCC