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| {{Infobox_Disease |
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| Name = {{PAGENAME}} |
| | '''For patient information click, [[{{PAGENAME}} (patient information)|here]]''' |
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| DiseasesDB = 5465 |
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| ICD10 = {{ICD10|G|61|0|g|60}} |
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| ICD9 = {{ICD9|357.0}} |
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| MeshID = D020275 |
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| {{Guillain-Barré syndrome}} | | {{Guillain-Barré syndrome}} |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
| | {{CMG}}; {{AE}} {{Fs}} |
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| {{CMG}}; '''Associate Editors-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh@perfuse.org] | | {{SK}} Acute inflammatory demyelinating polyradiculoneuropathy |
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| ==[[Guillain-Barré syndrome overview|Overview]]== | | == [[Guillain-Barré syndrome overview|Overview]] == |
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| ==[[Guillain-Barré syndrome pathophysiology|Pathophysiology]]== | | == [[Guillain-Barré syndrome historical perspective|Historical Perspective]] == |
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| All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents or vaccines) but mistargeted to host nerve tissues instead (a form of [[antigenic mimicry]]). The targets of such immune attack are thought to be [[gangliosides]], which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the [[nodes of Ranvier]]. An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those preceded by ''[[Campylobacter jejuni]]'' infections. Another example is the GQ1b ganglioside, which is the target in the Miller Fisher syndrome variant (see below).
| | == [[Guillain-Barré syndrome classification|Classification]] == |
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| The end result of such [[autoimmune]] attack on the peripheral nerves is inflammation of [[myelin]] and conduction block, leading to a muscle [[paralysis]] that may be accompanied by sensory or [[autonomic]] disturbances.
| | == [[Guillain-Barré syndrome pathophysiology|Pathophysiology]] == |
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| However, in mild cases, axonal function remains intact and recovery can be rapid if remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see below), axonal degeneration occurs, and recovery depends on axonal regeneration. Recovery becomes much slower, and there is a greater degree of residual damage. Recent studies on the disease have demonstrated that approximately 80% of the patients have [[myelin]] loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed [[axon]] loss.
| | == [[Guillain-Barré syndrome causes|Causes]] == |
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| ==[[Guillain-Barré syndrome history and symptoms|History & Symptoms]]== | | == [[Guillain-Barré syndrome differential diagnosis|Differentiating Guillain-Barré Syndrome from other Diseases]] == |
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| ==[[Guillain-Barré syndrome physical examination|Physical Examination]]== | | == [[Guillain-Barré syndrome epidemiology and demographics|Epidemiology and Demographics]] == |
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| The disease is characterized by weakness which affects the lower limbs first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without [[dysthesias]] (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Frequently, the lower [[cranial nerves]] may be affected, leading to bulbar weakness, (oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance. Facial weakness is also commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant (see below.)
| | == [[Guillain-Barré syndrome risk factors|Risk Factors]] == |
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| Sensory loss, if present, usually takes the form of loss of [[proprioception]] (position sense) and areflexia (complete loss of deep tendon reflexes), an important feature of GBS. Loss of pain and temperature sensation is usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain usually in the weakened muscles, which patients compare to the pain from overexercising. These pains are self-limited and should be treated with standard analgesics. Bladder dysfunction may occur in severe cases but should be transient. If severe, spinal cord disease should be suspected.
| | == [[Guillain-Barré syndrome natural history, complications, and prognosis|Natural history, Complications and Prognosis]] == |
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| Fever should not be present, and if it is, another cause should be suspected.
| | == Diagnosis == |
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| In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, [[orthostatic hypotension]], and cardiac arrhythmias.
| | [[Guillain-Barré syndrome diagnostic criteria|Diagnostic Criteria]] | [[Guillain-Barré syndrome history and symptoms|History and Symptoms]] | [[Guillain-Barré syndrome physical examination|Physical Examination]] | [[Guillain-Barré syndrome laboratory tests|Laboratory Findings]] | [[Guillain-Barré syndrome electrocardiogram|Electrocardiogram]] | [[Guillain-Barré syndrome MRI|MRI]] | [[Guillain-Barré syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| ==[[Guillain-Barré syndrome classification|Classification]]== | | == Treatment == |
| Although ascending paralysis is the most common form of spread in GBS, other variants also exist.
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| * '''Miller-Fisher Syndrome (MFS)''' is a rare variant of GBS and manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the ocular muscles first and presents as [[ophthalmoplegia]], [[ataxia]], and [[areflexia]]. [[Anti-ganglioside antibodies#Anti-GQ1b|Anti-GQ1b]] antibodies are present in 90% of cases.
| | [[Guillain-Barré syndrome medical therapy|Medical Therapy]] | [[Guillain-Barré syndrome physical therapy|Physical Therapy]] | [[Guillain-Barré syndrome secondary prevention|Secondary Prevention]] |
| * '''[[Acute motor axonal neuropathy]] (AMAN)<ref>McKhann GM, Cornblath DR, Ho T, Li CY, Bai AY, Wu HS, Yei QF, Zhang WC, Zhaori Z, Jiang Z, et al. Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet 1991;338:593-7</ref>, aka. Chinese Paralytic Syndrome,''' attacks motor nodes of Ranvier and is prevalent in China and Mexico. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies<ref>Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, Asbury AK, Blaser MJ, McKhann GM. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 1995;118:597-605.</ref> are present. [[Anti-ganglioside antibodies#Anti-GQ1b|Anti-GD3]] antibodies are found more frequently in AMAN
| | == Case Study == |
| * '''Acute motor sensory axonal neuropathy (AMSAN)''' is similar to AMAN but also affects sensory nerves with severe axonal damage. Recovery is slow and often incomplete<ref>Griffin JW, Li CY, Ho TW, Xue P, Macko C, Gao CY, Yang C, Tian M, Mishu B, Cornblath DR, et al. Guillain-Barré syndrome in northern China: The spectrum of neuropathological changes in clinically defined cases. Brain 1995;118:577-95</ref>.
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| ==Diagnosis==
| | [[Guillain-Barré syndrome case study one|Case #1]] |
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| The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. CSF and ECD is used almost every time to verify symptoms, but because of the acute nature of the disease, they may not become abnormal until after the first week of onset of signs and symptoms.
| | ==Related Chapters== |
| * '''[[cerebrospinal fluid|CSF]]''' - typical CSF findings include an elevated protein level (100 - 1000 mg/dL) without an accompanying pleocytosis (increased cell count). A sustained pleocytosis may indicate an alternative diagnosis such as infection. | | *[[Acute motor axonal neuropathy]] |
| The diagnosis is confirmed by the presence of Albuminocytological dissociation in the CSF
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| * '''Electrodiagnostics''' - [[electromyography]] (EMG) and [[nerve conduction study]] (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.
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| ==[[Guillain-Barré syndrome differential diagnosis|Differentiating Guillain-Barré syndrome from other Diseases]]==
| | {{WH}} |
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| ==Treatment==
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| ==[[Guillain-Barré syndrome medical therapy|Medical Therapy]]==
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| ==[[Guillain-Barré syndrome natural history, complications, and prognosis|Natural history, Complications, and Prognosis]]==
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| ==[[Guillain-Barré syndrome historical perspective|Historical perspective]]==
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| ==References==
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| {{reflist|2}} | |
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| [[ca:Síndrome de Guillain-Barré]]
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| [[de:Guillain-Barré-Syndrom]]
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| [[es:Síndrome de Guillain-Barré]]
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| [[fr:Syndrome de Guillain-Barré]]
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| [[it:Sindrome di Guillain-Barré]]
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| [[he:תסמונת גיאן-בארה]]
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| [[lb:Guillain-Barré-Syndrom]]
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| [[ms:Sindrom Guillain-Barré]]
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| [[nl:Syndroom van Guillain-Barré]]
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| [[ja:ギラン・バレー症候群]]
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| [[pl:Zespół Guillaina-Barrégo]]
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| [[pt:Síndrome de Guillain-Barré]]
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| [[ru:Синдром Гийена-Барре]]
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| [[fi:Guillain-Barrén oireyhtymä]]
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| [[sv:Guillain-Barrés syndrom]]
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| [[tr:Guillain-Barré sendromu]]
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| [[zh:格林-巴利综合征]]
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| {{WH}}
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| {{WS}} | | {{WS}} |
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| [[Category:Neurology]] | | [[Category:Neurology]] |
| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Syndromes]]
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| [[Category:Overview complete]]
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| [[Category:Disease]] | | [[Category:Disease]] |
| [[Category:Emergency medicine]] | | [[Category:Emergency medicine]] |
| [[Category:Infectious disease]]
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