Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that results in progressive muscle weakness. It has an annual incidence of 0.6 to 2.4 cases per 100,000 people and is the most common cause of acute flaccid paralysis. The pathogenesis involves peripheral nerve demyelination mediated by humoral and cell-mediated immune responses. Clinical features include ascending limb weakness over hours to weeks, areflexia, and possible autonomic dysfunction. Treatment includes plasmapheresis or intravenous immunoglobulin, which hasten recovery. The prognosis is generally good, with 30% residual weakness possible after 3 years and a 3% mortality rate.
Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that results in progressive muscle weakness. It has an annual incidence of 0.6 to 2.4 cases per 100,000 people and is the most common cause of acute flaccid paralysis. The pathogenesis involves peripheral nerve demyelination mediated by humoral and cell-mediated immune responses. Clinical features include ascending limb weakness over hours to weeks, areflexia, and possible autonomic dysfunction. Treatment includes plasmapheresis or intravenous immunoglobulin, which hasten recovery. The prognosis is generally good, with 30% residual weakness possible after 3 years and a 3% mortality rate.
Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that results in progressive muscle weakness. It has an annual incidence of 0.6 to 2.4 cases per 100,000 people and is the most common cause of acute flaccid paralysis. The pathogenesis involves peripheral nerve demyelination mediated by humoral and cell-mediated immune responses. Clinical features include ascending limb weakness over hours to weeks, areflexia, and possible autonomic dysfunction. Treatment includes plasmapheresis or intravenous immunoglobulin, which hasten recovery. The prognosis is generally good, with 30% residual weakness possible after 3 years and a 3% mortality rate.
It has an annual incidence of 0.6 to 2.4 cases per 100,000 population and occurs at all ages and in both sexes With the marked decline in the incidence of polio, Guillain-Barr syndrome is now the most common cause of acute flaccid paralysis in healthy people
Guillain-Barr syndrome: is a rare immune mediated neuropathy characterized by progressive muscle weakness PATHOGENESIS Peripheral nerve demyelination in Guillain-Barr syndrome is believed to be immunologically mediated Humoral factors and cell-mediated immune phenomena have been implicated in the damage of myelin and/or the myelin-producing Schwann cells
Clinical feature Two-thirds of patients develop the neurologic symptoms 2- 4 weeks after what appears to be a benign respiratory or gastrointestinal infection The initial symptoms are fine paresthesias in the toes and fingertips, followed by lower extremity weakness that may ascend over hours to days to involve the arms, cranial nerves, and in severe cases the muscles of respiration Clinical feature Weakness in lower extremities progresses over hours to weeks and peaks in 14 days. Muscles of the distal extremities are severely affected. Common sx include: hypotonia (reduced muscle tone) and areflexia (lack of reflexes). Autonomic disturbances Physical Examination Symmetric limb weakness with diminished or absent reflexes Minimal loss of sensation despite paresthesias Signs of autonomic dysfunction are present in 50 percent of patients, including Cardiac dysrhythmias (asystole, bradycardia, sinus tachycardia, and atrial/ventricular tachyarrhythmias) Orthostatic hypotension Transient or persistent hypertension Paralytic ileus Bladder dysfunction Abnormal sweating Diagnose Based on pt hx and clinical manifestations. Lumbar puncture for CSF is normal or low protein. After 7 days protein is high. Electromyogram (EMG) test shows that nerve conduction is slower. Electrocardiogram (ECG) test shows heart irregularities. Nerve biopsy can be done examining the damaged nerve or axon. Prognosis The recovery ranges from 3 w to 3 y. After 3 y, 30% of the pts will have residual weakness. The mortality rate is 3% from GBS. Treatment The main modalities of therapy for Guillain-Barr syndrome include Plasmapheresis Administration of intravenous immune globulin AANs Class of evidence for therapy Class I. High quality randomized controlled trials (RCTs) Class II. Prospective matched group cohort studies or RCTs lacking adequate randomization concealment or blinding, or potentially liable to attrition or outcome ascertainment bias Class III. Other studies such as natural history studies Class IV. Uncontrolled studies, case series, or expert opinion AANs Recommendation Levels Level A Established as effective, ineffective or harmful, or as useful/predictive or not useful/predictive Level B Probably effective, ineffective or harmful, or as useful/predictive or not useful/predictive Level C Possibly effective, ineffective or harmful, or as useful/predictive or not useful/predictive Level U Data inadequate or conflicting; Treatment, test, or predictor unproven Conclusions (PE)
Plasma exchange hastens recovery in non-ambulant patients with GBS who present within four weeks from the onset of neuropathic symptoms (Class II evidence).
Plasma exchange also hastens recovery in ambulant patients who present within two weeks but the evidence is limited to one trial (Class II evidence).
The effects of plasma exchange and IVIg are equivalent in patients requiring aid to walk(Class I evidence).
Treatment with CSF filtration has not been adequately tested (Limited Class II evidence).
American Academy of Neurology Recommendations
PE is recommended in non-ambulant patients within four weeks from onset (Level A, Class II evidence).
PE is recommended for ambulant patients within two weeks from onset (Level B, limited Class II evidence).
American Academy of Neurology
Iv immune globuline Conclusions Intravenous immunoglobulin has not been adequately compared with placebo (limited Class II evidence). Such comparison is not now needed because, when started within two weeks from the onset, IVIg has equivalent efficacy to PE in hastening recovery from patients with GBS who require aid to walk (Class I evidence). Multiple complications were significantly less frequent with IVIg than with PE (Class I evidence). There is no evidence concerning the relative efficacy of PE and IVIg in patients with axonal forms of GBS. American Academy of Neurology
Recommendations
IVIg is recommended for patients with GBS who require aid to walk within two (Level A recommendation) or four weeks from the onset of neuropathic symptoms (Level B recommendation derived from Class II evidence concerning PE started within the first four weeks).
The effects of IVIg and plasma exchange are equivalent. (Level B recommendation Class I evidence concerning the comparisons between PE and IVIg started within the first two weeks).
American Academy of Neurology
Analysis of the evidence Combination treatments One Class I trial showed that PE followed by IVIg showed no significant benefit compared with PE alone in any measured outcome. Conclusions Sequential treatment with PE followed by IVIg does not have a superior effect to either treatment given alone (Class I evidence).
Sequential treatment with immunoabsorption followed by IVIg has not been adequately tested (Limited Class IV evidence).
American Academy of Neurology
Recommendations
Sequential treatment with PE followed by IVIg is not recommended (Level A recommendation, Class I evidence).
Immunoabsorption followed by IVIg is not recommended (Level U recommendation, Class IV evidence).
American Academy of Neurology
Analysis of the evidence Immunoabsorption An alternative technique to PE, which removes immunoglobulins. Has the advantage of not requiring the use of a human blood product as a replacement fluid. In a prospective trial there were no differences in outcome between 11 patients treated with PE and 13 treated with immunoabsorption There is only limited Class IV evidence from a single small non-randomized, unblinded study.
Conclusion The evidence is insufficient to recommend the use of immunoabsorption (Level U recommendation, Class IV evidence).
American Academy of Neurology
Recommendation A 17-year-old male developed flu-like symptoms, severe diarrhoea and abdominal pain 4 days after attending a dinner party at which he had eaten a chicken. Three other people who had attended the same party developed gastrointestinal symptoms. These symptoms settled within a few days. Stool cultures taken from all four individuals grew Campylobacter jejuni. About 10 days after the onset of diarrhoea, he developed diffuse aching around his shoulders and buttocks and pins and needles in his hands and feet. Over the next week the sensory changes worsened and spread to involve his arms and legs. His limbs became progressively weaker and 8 days after the onset of neurological symptoms he could not hold a cup or stand unaided. He was admitted to hospital and found to have severe symmetrical distal limb weakness and glove and stocking sensory loss to the elbows and knees. Nerve conduction studies showed evidence of a mixed motor and sensory neuropathy and examination of his cerebrospinal fluid (CSF) showed a very high total protein level at 4g/l but without any increase in the number of cells in the CSF. High titres of IgM and IgG antibodies to Campylobacter jejuni were found in his peripheral blood. Case