THE GUILLAIN-BARRÉ SYNDROME:
ORIGINS
The FDA recently altered the labeling on the J&J Covid vaccine, warning of a small risk of Guillain-Barré syndrome in recipients. GBS, as it is sometimes called, has an interesting history.
Guillain, Barré, and Strohl (yes, three authors) first described the syndrome in two French soldiers during the First World War. The soldiers suffered from an ascending paralysis extending up to their arms, and dysesthesias, from which they recovered almost completely. Lumbar punctures were done on both, the fluid showing a high protein content and no polymorphonuclear (inflammatory) cells, a finding the authors said was distinctive. Lumbar puncture was relatively new at the time. Heinrich Quincke and Walter Wynter
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| Heinrich Quincke (Wikipedia) |
had almost simultaneously published the technique in 1891. Both physicians aimed to relieve elevated pressure and not to study the fluid.
The first of the 3 GBS authors, Georges Guillain, trained in neurology under Pierre Marie at the famous Salpêtrière Hospital in Paris. He was working his way up the academic ladder when the war broke out and promptly volunteered for service. He served in various field units, eventually becoming chief physician of the neurology section of the Sixth
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| Georges Guillain (Wikipedia) |
Army. Jean-Alexandre Barré trained under Joseph Babinski and Pierre Marie. In the war he also worked in various field stations, eventually heading a military neurology center in the Eastern Region.
Neurologic and brain injuries were especially common in the early years of WWI because of poorly designed French helmets that provided inadequate protection. Both La Salpêtrière and La Pitié hospitals militarized their neurology departments to handle the flood of patients. At the former, Pierre Marie noted that “War injuries have shown us a different set of facts: lesions of the cortex, with more or less exclusion of white
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| Jean-Alexandre Barré (Wikipedia) |
matter. Consequently, this new pathology is infinitely closer to the data of experimental physiology than was the old cerebral pathology.” At La Pitié Hospital, Joseph Babinski ran the military neurology unit. Guillain and Barré enjoyed a fruitful collaboration, publishing important papers on injuries to the brain and the spinal cord and delineating indications for surgery.
The third author of the original paper, André Strohl, had trained in physics and medicine. Strohl performed electromyograms on the calf muscles of the two
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| André Strohl (Wikipedia) |
patients, showing a weakening or absence of the reflex response and a delay in response. It was an early use of the technique. After the war, Strohl worked as professor of physiologic medicine in Algiers and later professor of physical medicine at the University of Paris. He was elected to the Académie de Médecine at the age of 35, an unusual honor. He wrote several books on nerve and muscle physiology, on nuclear isotopes, and a text on physical medicine.
Of course, other physicians had published cases of ascending paralysis before the war. Jean Baptiste Octave Landry, the best-known, had reported on acute ascending paralysis (10 cases) in 1859, before the age of lumbar puncture. Other reports followed, and a few earlier ones came to light. The term “Guillain-Barré syndrome” was first used by Barré at a neurology conference in 1927. Guillain and Barré maintained that their syndrome (they actually called it “notre syndrome”) was distinct, emphasizing the favorable recovery and the findings in the spinal fluid. Why the name Strohl was dropped from “our syndrome” by Guillain and Barré, was never quite clear. Various reasons have been suggested: his German name and his origin from Alsace and the fact that he was not a neurologist. Only one month after the original Guillain-Barré-Strohl report, Pierre Marie and Charles Chatelin published 3 more cases in soldiers, acknowledging the earlier report by G, B, and S. With slightly different timing, might this have been dubbed the “Marie-Chatelin” syndrome?
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| Salpêtrière Hospital, about 1660, after conversion from gunpowder factory (saltpeter was used to make gunpowder). Image by Jean-Pol GRANDMONT of engraving by Adam Pérelle (Wikipedia). |
Over the years, many more cases of ascending paralysis came to light, presenting with varying degrees of paralysis and recovery and not always with “typical” spinal fluid findings. Many were fatal. Guillain and Barré resisted the trend to broaden the spectrum of “their syndrome” but eventually they had to yield. The GBS syndrome now includes a wide range of presentations.
The range is wide enough, in fact, that a group of 5 authors recently wondered if Franklin D. Roosevelt might have suffered from GBS instead of polio. In early August 1921, Roosevelt, a 39-year-old practicing lawyer, was vacationing on Campobello Island, New Brunswick. After a day of vigorous activity, he developed aches and sensitivity to touch in his legs. His legs weakened and over a few days were completely paralyzed. Eventually the family consulted Robert W. Lovett, professor of orthopedics at Harvard, who had organized the first polio clinic at Boston Children’s Hospital. He diagnosed poliomyelitis and recommended a spinal tap, though it appears not to have been done. Roosevelt entered Presbyterian Hospital in New York under the care of Dr. George Draper, a classmate of FDR’s at Groton and Harvard, who had written a book on polio in 1917. As we all know, Roosevelt’s legs never recovered.
Was FDR’s disease really polio? Or could it have been Guillain-Barré syndrome? To make a case for GBS, the 5 authors pointed out differences between typical polio and GBS syndromes, using a statistical analysis to conclude that GBS was the more likely diagnosis. A subsequent paper challenged that conclusion. Lovett and Draper may not have seen the G, B, and S paper of 1916, written in French. Whatever the truth, it is likely that if Roosevelt’s diagnosis had not been polio the March of Dimes, the principal driver of the first polio vaccine, would not have come into being.
SOURCES:
Waclawik, A J, “The Legacy of the Seminal Publication by Guillain, Barré, and Strohl: The History Behind the Eponym.” 2018; Wisconsin Med J 117: 160-3.
Pietrzak, K, et al, “Georges Guillain.” 2016; J Neurol 263: 2148-9.
Green, D, “Infectious Polyneuritis and Professor André Strohl – A Historical Note.” 1962; New Engl J Med 267: 821-2.
Goldman, A S, et al, “Franklin Delano Roosevelt’s (FDR’s) (1882-1945) 1921 Neurologic Disease Revisited; the Most Likely Diagnosis Remains Guillain-Barré Syndrome.” 2015; J Med Biog 24 (4): 452-9.
Goldman, A S, et al, “What Was the Cause of Franklin Delano Roosevelt’s Paralytic Illness?” 2003; J Med Biog 11: 232-40.
Dittuno, J F, et al, “Franklin Delano Roosevelt: The Diagnosis of Poliomyelitis Revisited.” 2016; J Phys Med Rehab 8: 883-93.
Guillain, G, Barré, J-A, et Strohl, A, “Sur un Syndrome de Radiculo-Névrite avec Hyperalbuminose du Liquide Céphalo-Radicien sans Reaction Cellulaire.” 1916; Bull Memoires Société Hosp Parisser 3, v40,prt 2: 1462-70.
Walusinski, O, et al, “French Neurologists During World War One.” 2016; Frontiers Neurol Neuroscience 38: 107-118.
