Guillain–Barré syndrome
| Guillain–Barré syndrome | |
|---|---|
| Other names | Guillain–Barré–Strohl syndrome, Landry's paralysis, postinfectious polyneuritis, Acute Inflammatory Demyelinating Polyneuropathy[1] |
| Pronunciation | |
| Specialty | Neurology |
| Symptoms | Muscle weakness beginning in the feet and hands, usually ascending[4] |
| Complications | Breathing difficulties, heart and blood pressure problems[1][4] |
| Usual onset | Rapid (hours to weeks)[4] |
| Causes | Typically triggered by an infection; occasionally by surgery |
| Diagnostic method | Based on symptoms, nerve conduction studies, lumbar puncture[4] |
| Treatment | Supportive care, intravenous immunoglobulin, plasmapheresis |
| Prognosis | Weeks to years for recovery[4] |
| Frequency | 2 per 100,000 people per year[4][5] |
| Deaths | 7.5% of those affected[1] |
| Named after |
|
Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system.[4] Typically, both sides of the body are involved, and the initial symptoms are changes in sensation or pain often in the back along with muscle weakness, beginning in the feet and hands, often spreading to the arms and upper body.[4] The symptoms may develop over hours to a few weeks.[4] During the acute phase, the disorder can be life-threatening, with about 15% of people developing respiratory muscle weakness requiring mechanical ventilation.[1] Some are affected by changes in the function of the autonomic nervous system, which can lead to dangerous abnormalities in heart rate and blood pressure.[4]
Although the cause is unknown, the underlying mechanism involves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation.[4] Sometimes this immune dysfunction is triggered by an infection or, less commonly, by surgery, and by vaccination.[1][4] The diagnosis is usually based on the signs and symptoms through the exclusion of alternative causes and supported by tests such as nerve conduction studies and examination of the cerebrospinal fluid.[4] There are several subtypes based on the areas of weakness, results of nerve conduction studies, and the presence of certain antibodies.[6] It is classified as an acute polyneuropathy.[1]
In those with severe weakness, prompt treatment with intravenous immunoglobulins or plasmapheresis, together with supportive care, will lead to good recovery in the majority of cases.[4] Recovery may take weeks to years, with about a third having some permanent weakness.[4] Globally, death occurs in approximately 7.5% of those affected.[1] Guillain–Barré syndrome is rare, at 1 or 2 cases per 100,000 people every year.[4][5] The illness that afflicted US president Franklin D. Roosevelt, and left him paralysed from the waist down, which was believed at the time to be polio, may have been Guillain–Barré syndrome, according to more recent research.[7]
The syndrome is named after the French neurologists Georges Guillain and Jean Alexandre Barré, who, together with French physician André Strohl, described the condition in 1916.[8][9]
- ^ a b c d e f g Ferri FF (2016). Ferri's Clinical Advisor 2017: 5 Books in 1. Elsevier Health Sciences. p. 529. ISBN 9780323448383. Archived from the original on 2016-08-21.
- ^ "Guillain–Barré, n.". Oxford English Dictionary (Online ed.). Oxford University Press. doi:10.1093/OED/2577463821. (Subscription or participating institution membership required.)
- ^ a b "Guillain-Barré syndrome". Cambridge Dictionaries (Online). Cambridge University Press. n.d.
- ^ a b c d e f g h i j k l m n o p "Guillain–Barré Syndrome Fact Sheet". NIAMS. June 1, 2016. Archived from the original on 5 August 2016. Retrieved 29 August 2022.
- ^ a b Sejvar JJ, Baughman AL, Wise M, Morgan OW (2011). "Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis". Neuroepidemiology. 36 (2): 123–33. doi:10.1159/000324710. PMC 5703046. PMID 21422765.
- ^ van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA (August 2014). "Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis". Nature Reviews. Neurology. 10 (8): 469–82. doi:10.1038/nrneurol.2014.121. PMID 25023340. S2CID 25154778.
- ^ "Did FDR Have Guillain-Barré?".
- ^ van Doorn PA, Ruts L, Jacobs BC (October 2008). "Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome". The Lancet. Neurology. 7 (10): 939–50. doi:10.1016/S1474-4422(08)70215-1. PMID 18848313. S2CID 9307245.
- ^ Eldar AH, Chapman J (April 2014). "Guillain Barré syndrome and other immune mediated neuropathies: diagnosis and classification". Autoimmunity Reviews. 13 (4–5): 525–30. doi:10.1016/j.autrev.2014.01.033. PMID 24434363.