In 2016, a century had passed since the famous description of a syndrome of an acute,
ascendant and symmetrical paralysis involving the peripheral nerves with an albumin-cytologic
dissociation in the cerebrospinal fluid and a good recovery, by three French neurologists:
Guillain, Barré and Strohl. This syndrome, well known as “Guillain-Barré syndrome”,
is the most common cause of acute flaccid paralysis in the world. In June 2016, a
symposium in Glasgow celebrated a century of Guillain-Barré syndrome, patronized by
the Peripheral Nerve Society[1].
Guillain-Barré history
In 1916, Georges Charles Guillain, Jean Alexandre Barré and André Strohl ([Figure 1]) described in Bulletins et Mémoires de la Société Médicale des Hçpitaux de Paris
[2], a new syndrome in two young French soldiers characterized by motor disturbance,
abolition of the tendon reflex, paresthesia with mild disturbance of sensation, slight
changes in electric reaction of nerves and muscles and hyperalbuminosis of the cerebrospinal
fluid with absence of cytological reaction (albumin-cytologic dissociation). The cases
were presented at the Medical Society of the Hospitals in Paris on October 16th 1916, under the title “Sur un syndrome de radiculonévrite avec hyperalbuminose du liquide céphalorachidien
sans reaction cellulaire. Remarques sur le caractères cliniques et graphiques des
réflexes tendineux” ([Figure 2]). They thought that this syndrome was due to involvement of the spinal roots and
nerves, probably infectious or toxic in nature. The treatment consisted of rest, massage
and injections of strychnine. Both patients recovered after a few months.
Figure 1 Georges Charles Guillain (1876-1971), Jean Alexandre Barré (1880-1967) and André
Strohl (1887-1977).
Figure 2 Original article by Gullain-Barré-Strohl.
Guillain became professor of Neurology at the Salpêtrière Hospital in Paris. Barré
was professor of Neurology at Strasbourg. They became friends at the “Centre Neurologique”
of the 6th army near Amiens during the first world war, which is where they saw these cases[3]. Strohl was forgotten, probably because of his young age and his participation was
limited to electrophysiology.
Early description
In 1850, Jean Baptiste Octave Landry de Théizillat (1826-1865) ([Figure 3]) wrote “Note sur la paralysie ascendante aigue” where he reported on 10 patients,
five of whom he had personally examined[4]. He described three types of presentation: ascending paralysis without sensory signs
or symptoms; ascending paralysis with ascending anesthesia, and a progressive disorder
characterized by paralysis and slight sensory loss. His first patient, a 43-year-old
man, after a premonitory fever, complained of “weakness, tingling his fingers and
toes”. Touch sensation was lost in the fingers and feet but was little impaired in
the upper two thirds of the lower limbs. The paralysis ascended, with sensory loss.
By the third week, his limbs were paralyzed, he developed difficulty in breathing
and swallowing, with fever and coughing. Touch was lost in the feet and in the finger
tips, but without pain and temperature loss. He died in the third week. Necropsy included
histological examination only of the cord and soleus muscle. The main difference between
that report and the syndrome described by Guillain, Barré and Strohl was that Landry
did not perform a lumbar puncture, as this was introduced by Quincke only in 1891.
Figure 3 Jean Baptiste Octave Landry de Théizillat (1826-1865).
Landry was a gentle and modest man. At the age of 40, while attending the victims
of a cholera epidemic in Paris, Landry contracted the illness and died a few days
later[5]. Haymaker called this syndrome the “Landry-Guillain-Barré syndrome” and included
the name of Landry as one of the fathers of Neurology[6]. Landry’s description probably included all types of acute or subacute ascending
paralysis, such as some cases of poliomyelitis or ascending myelitis. In 1864, Louis
Stanislas Duménil, in Rouen, described one case of an acute and symmetrical ascending
paralysis. He thought it was due to nerve atrophy. Three years later he described
three other cases[1].
Sir William Osler (1849-1919) in “The Principles and Practice of Medicine” ([Figure 4]) wrote about an ascending paralysis he referred to as an acute infectious polyneuritis[3]. Guillain and Barré did not accept that description, as Osler’s patient had fever
in the beginning of the disease and had no lumbar puncture[7].
Figure 4 Willian Osler (1849-1919).
In Paris in 1828, Auguste François Chomel (1788-1858) described a patient with disturbed
sensations in their hands and feet, ascendant weakness with acute evolution, becoming
paralytic within a few days[1]. In 1881, Thomas Stewart (1837-1900), in Edinburgh, reported on three cases of that
same illness, calling attention to the loss of tendon reflexes[1].
Soon after 1916
Draganesco and Claudian were the first to use the term “Guillain-Barré syndrome” in
1927[1].
In 1936, Guillain published ten nonfatal cases and established the main conditions
of this syndrome: 1) albumin-cytologic dissociation in the cerebral spinal fluid;
2) onset with paralysis and paresthesia or pain, with or without premonitory symptoms;
3) flaccid paralysis of the lower limbs and later in the upper limbs; 4) occasional
fibrillatory twitching and slight atrophy of the distal muscles; 5) abolition of tendon
reflexes; 6) presence of subjective sensory disturbance; 7) objective minor or transitory
changes in sensibility; 8) transitory paralysis of the cranial nerves; 9) transitory
sphincter disturbance8.
In the same year, Alajouanine published a similar case in which death had occurred[9].
Guillain and Barré[7] refused to accept the cases described without albumin-cytologic dissociation in
the cerebral spinal fluid, and in the cases with fatal outcome, Landry and Alajouanine
were the objects of attack by Guillain[1],[3]. Firstly because they did not mention the albumin-cytologic dissociation in the
cerebral spinal fluid, and secondly, because the patients died from the disease. Two
years later, Guillain accepted that this syndrome could be fatal[3].
The first case in Brazil was described by Antonio Rodrigues de Mello ([Figure 5]) in a soldier who, without prodromal symptoms, presented with a subacute ascending
paralysis, slight sensory disturbances in the limbs with paralysis of the trigeminal
and facial nerves. The cerebral spinal fluid showed an albumin-cytologic dissociation.
He was treated with intravenous and intrathecal vitamin B and recovered in a few weeks.
Mello believed that the disease was due to lack of vitamins or due to a neurotrophic
virus[10].
Figure 5 Antonio Rodrigues de Mello (1911-1988).
Conclusion
The history of Guillain-Barré syndrome continues today. Many advances have been made
in the epidemiology, clinical variants, atypical cases, electrophysiology findings,
pathogenesis, preceding infections and vaccinations, nerve images, treatment and rehabilitation.
These are another story.
