Guillain-Barré syndrome The first description of Guillain-Barré Syndrome (GBS) was made in 1859, by a young French Physician called Jean Baptiste Landry. In this paper, he described a group of patients who had a form of ascending motor paralysis associated with various forms of sensory disturbance .
In 1916, 3 French physicians, George Guillian, Jean Alexandre Barré and Albert Strohl published the paper: "Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquide cephalo-rachidien sans reaction cellulaire: remarques sur les caracteres cliniques et graphiques des reflexes tendineux." Here, they described soldiers in the First World War, who developed a motor weakness preventing them from wearing their backpacks, and who also had associated loss of their tendon reflexes. Lumbar puncture from these patients demonstrated an acellular CSF with a high protein.
There was some debate around the exact definition of the disease, following Guillain, Barre and Strohl's publication. In particular, many Neurologists did not accept that Landry's Ascending Paralysis was the same as GBS. However, a comprehensive study examining post-mortem data and relevant literature identified the similarities between these two conditions and other forms of areflexic paralysis that had been described subsequently, and concluded that Guillain-Barré Syndrome represents a heterogenous group of disorders consisting of both demyelinating (AIDP) and axonal (AMAN or AMSAN) forms .
The disease is associated with substantial morbidity and mortality. In approximately 20% of patients, the paralysis is so severe that prolonged mechanical ventilation is required, and recovery can be prolonged. This results in a mortality rate of 5-10% with 12% of survivors being left unable to walk after a year from studies in the United States.
The exact aetiology of GBS is uncertain, but it is thought to be an immune mediated condition to components of the nervous system. Antibodies to gangliosides are strongly implicated in forms of GBS, including AMAN and Miller-Fisher Syndrome and its variants. GBS commonly follows an infection or, rarely, vaccination.
Presents as an ascending paralysis, usually starting 2 weeks after an insult to the immune system. This can take the form of an antecedent infection (e.g. with Campylobacter), vaccination, respiratory tract infection, Herpes infection, or even surgery.
A flaccid paralysis develops with a loss of reflexes. The paralysis may then spread to cause upper limb weakness and bulbar involvement. Eye movements may also be impaired.
CT scan will be unremarkable. Lumbar puncture will often show an acellular CSF with high protein (although CSF protein can be normal during the first week of illness). EMG/Nerve Conduction studies are also helpful in diagnosis. Certain forms of GBS are associated with antibodies to gangliosides (95% of Miller-Fisher Syndrome patients will have antibodies to complex gangliosides)
Intravenous immunoglobulin therapy and plasmapharesis are equally effective. Main restrictions are cost and accessibility.
A recent Cochrane review did not show any evidence that steroids alone produce an improvement in symptoms, and indeed their use was associated with a slight (statistically insignificant) increase in steroid related complications including diabetes and hypertension. However, one study did suggest that the time to independent walking is improved if intravenous steroid is given in conjunction with immunoglobulin, and it can therefore be used as an adjunct treatment in cases where the patient’s recovery is prolonged.
- ↑ Landry, JB (1859) raité complet des paralysies. T. I. Paris, Masson, 1859.
- ↑ Guillain,G., Barre,J.A., and Strohl,A. (1916). Sur un syndrome de radiculo-neurite avec hyperalbuminose du liquide cephalo-rachidien sans reaction cellulaire: remarques sur les caracteres cliniques et graphiques des reflexes tendineux. Bull Soc Med Hop Paris; 40:1462-70
- ↑ Haymaker,W. and Kernohan J.W. The Landry-Guillain-Barre Syndrome: A clinicopathologic report of fifty fatal cases and a critique of the literature. 1949 Medicine (Baltimore) 28:59-141.
- ↑ Buzby JC, Allos BM, Roberts T. The economic burden of Campylobacter-associated Guillain-Barre syndrome. J Infect Dis. 1997 Dec;176 Suppl 2:S192-7.
- ↑ Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies. Brain. 2002 Dec;125(Pt 12):2591-625. Review.. (Direct link - may require subscription)
- ↑ Australian Technical Advisory Group on Immunisation of the Australian Government Department of Health and Ageing. Australian Technical Advisory Group on Immunisation (ATAGI) advice regarding influenza, influenza vaccines and Guillain-Barré Syndrome. Canberra: Australian Government, 2009 (??October); 1-10.
- ↑ Shahar,E.Current therapeutic options in severe Guillain-Barre syndrome. 2006 Jan-Feb;29(1):45-51.
- ↑ Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2006 Apr 19;(2):CD001446. Review.
- ↑ The Dutch Guillain-Barre Study Group. Treatment of Guillain-Barre syndrome with high-dose immune globulins combined with methylprednisolone: a pilot study. Ann Neurol. 1994 Jun;35(6):749-52. Erratum in: Ann Neurol 1994 Sep;36(3):457.