Guillain-Barre Syndrome - Diagnosis

9/28/01 (Franco)

Question: What are the diagnostic criteria for Guillain-Barre syndrome?

Link Directly to Fulltext article in Ovid

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[Link Directly to Fulltext Article in OVID]

Unique Identifier: 21298931 / PubMed Identifier: 11405806

Authors: Gordon PH. Wilbourn AJ.

Institution: Department of Neurology, University of New Mexico School of Medicine, 915 Camino de Salud NE, Albuquerque, NM 87131, USA. pgordon@salud.unm.edu

Abstract: CONTEXT: Guillain-Barre syndrome (GBS) is the foremost cause of acute, generalized, peripheral neuropathic weakness. Although nerve conduction studies are a diagnostic aid, the characteristic electrical changes may not evolve for several weeks. Early diagnosis of GBS is important, however, because early treatment has been shown to improve outcome. OBJECTIVES: To describe the electrodiagnostic abnormalities detectable in the first week of GBS, to determine if there are early patterns suggestive of GBS, and to identify the percentage of patients whose condition can be diagnosed with reasonable certainty in the first week. DESIGN AND SETTING: We retrospectively reviewed the medical records of all patients admitted to the Cleveland Clinic Foundation, Cleveland, Ohio, having the discharge diagnosis GBS during the past 16 years. Patients who underwent nerve conduction studies within 7 days of muscle weakness were selected for this study. RESULTS: The H reflex was absent in 30 (97%) of 31 patients. Nineteen patients (61%) had low-amplitude or absent sensory nerve action potential (SNAP) in the upper extremity. Fifteen patients (48%) overall, including 21 (67%) of the 31 patients, including 14 (67%) of the 21 patients younger than 60 years, had an abnormal upper extremity SNAP combined with a normal sural SNAP. Other findings included an abnormal F wave (25 patients [84%]), reduced compound muscle action potential amplitude (22 patients [71%]), prolonged distal latency (20 patients [65%]), temporal dispersion (18 patients [58%]), slowed motor conduction velocity (16 patients [52%]), and motor conduction block (4 patients [13%]). Definite diagnosis was possible in 17 patients (55%), but not commonly until the fifth day. CONCLUSIONS: The H reflex is the most sensitive test for early GBS. Upper extremity SNAPs are also frequently abnormal in early GBS. Absent H response, abnormal F wave, and abnormal upper extremity SNAP combined with a normal sural SNAP are characteristic of early GBS. If multiple nerves are tested, definite diagnosis is possible in half the patients, but not until the fifth day after the onset of symptoms.

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Unique Identifier: 21203590 / PubMed Identifier: 11306855

Authors: Van der Meche FG. Van Doorn PA. Meulstee J. Jennekens FG. GBS-consensus group of the Dutch Neuromuscular Research Support Centre.

Institution: University Hospital, Rotterdam, The Netherlands. vandermeche@neur.azr.nl

Abstract: BACKGROUND: Diagnostic criteria for the Guillain-Barre syndrome (GBS) have been available since 1978. Since then, several variants have been described. More recently, a distinction has been made between pure motor forms, severe sensory forms, primary axonal and primary demyelinating varieties. Associations of clinical characteristics, and specific infections and the presence of antiganglioside antibodies have been found. For further studies on GBS, it is therefore necessary to reconsider the available diagnostic criteria and add additional criteria for subclassification. METHODS: A panel of (20) experts was formed. The literature representing the recent developments in GBS subclassification was reviewed. Following a consensus protocol, diagnostic and classification criteria were formulated. RESULTS: The diagnosis of GBS can usually be made on clinical characteristics. A schedule for subclassification has been made to cover also the clinical variants in a systematic manner. [References: 32]

Link Directly to Fulltext Article at Science Direct

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[Link Directly to Fulltext Article in OVID]

Unique Identifier: 98417122 / PubMed Identifier: 9746040

Authors: Hahn AF.

Institution: Clinical Neurological Sciences, University of Western Ontario, London Health Sciences Centre, Canada. angelika.hahn@lhsc.on.ca

Abstract: Guillain-Barre syndrome (GBS) is viewed as a reactive, self-limited, autoimmune disease triggered by a preceding bacterial or viral infection. Campylobacter jejuni, a major cause of bacterial gastroenteritis worldwide, is the most frequent antecedent pathogen. It is likely that immune responses directed towards the infecting organisms are involved in the pathogenesis of GBS by cross-reaction with neural tissues. The infecting organism induces humoral and cellular immune responses that, because of the sharing of homologous epitopes (molecular mimicry), cross-react with ganglioside surface components of peripheral nerves. Immune reactions against target epitopes in Schwann-cell surface membrane or myelin result in acute inflammatory demyelinating neuropathy (85% of cases); reactions against epitopes contained in the axonal membrane cause the acute axonal forms of GBS (15% of cases). Care for such patients may be challenging, yet the prognosis overall is favourable. Optimal supportive care and anticipation and prevention of complications are the mainstay of therapy. Admission to the intensive-care unit is necessary in 33% of patients who require intubation and assisted ventilation. Immunomodulation with infusions of IgG or plasma exchange treatments foreshorten the disease course. [References: 74]

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Unique Identifier: 96285298 / PubMed Identifier: 8677835

Authors: Dematteis JA.

Institution: Hamot Medical Center, Erie, Pennsylvania, USA.

Abstract: Guillain-Barre syndrome is the most frequently acquired demyelinating peripheral polyneuropathy. In approximately two-thirds of cases, Guillain-Barre syndrome is preceded by a viral respiratory or gastrointestinal infection. The mechanism of injury is unclear but is believed to be immunologic. The cardinal clinical feature is symmetric and rapidly progressive weakness. Aspiration and respiratory failure are the major concerns. Sensory symptoms, such as paresthesias, are common. The most severe stage of the disease is reached two to four weeks after onset. Dysautonomia has replaced respiratory failure as the most common cause of death. Recovery is variable: 50 percent of patients recover completely, about 35 percent experience permanent neurologic sequelae, and 15 percent are significantly and permanently damaged. About 10 percent relapse before complete recovery, and 2 to 5 percent experience recurrence after full recovery. Laboratory confirmation of Guillain-Barre syndrome includes the typical cerebrospinal fluid cytoalbumin dissociation (elevated protein without white blood cells). Treatment is primarily symptomatic and preventive. Convalescent patients require intensive inpatient physical and occupational therapy to improve strength and prevent disabling contractures. [References: 19]

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Unique Identifier: 90302957 / PubMed Identifier: 2194422

Authors: Asbury AK. Cornblath DR.

Institution: Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia.

Abstract: Criteria for the diagnosis of Guillain-Barre syndrome are reaffirmed. Electrodiagnostic criteria are expanded and specific detail added. [References: 13]