History of Guillain-Barré syndrome

1916: birth in Paris of a modest syndrome presented to a weekly meeting of the Société Médicale by Georges Guillain, Jean-Alexandre Barré and André Strohl. 1981: glorification of the syndrome as a world event in Santa Inez Valley at the International Conference held under the auspices of the Kroc Foundation. This is indeed a long way from a few clinicians and internists to the representatives of all branches of the Neurological Sciences for "la radiculo-névrite avec dissociation albumino-cytologique à évolution spontanément régressive "studied in two soldiers of the Vth French Army. Every neurologist from clinician to researcher currently knows this model of inflammatory and demyelinating diseases of the peripheral nervous system and the pros and cons of cellular vs humeral immunity which are presumed to be its pathophysiological process. What is less known is "la petite histoire" i.e. that of men and events which surrounded its birth and growth to being an entity. Why did André Strohl disappear? Who were L. Duménil and O. Landry? Should we say Guillain-Barré?, Landry-Guillain-Barré?, Duménil-Landry-Guillain-Barré? Unexpected or poorly known facts are not lacking in this story the last of which being that most references to that most French syndrome are to be found in English and American books.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is currently divided into the two major subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). This review highlights relevant recent publications, particularly on the pathophysiology of AMAN. Molecular mimicry of the bacterial lipo-oligosaccharide by the human gangliosides is now considered an important cause of AMAN. Gangliosides GM1, GM1b, GD1a, and GalNAc-GD1a expressed on the motor axolemma are likely to be the epitopes for antibodies in AMAN. At the nodes or paranodes, deposition of antiganglioside antibodies initially cause reversible conduction block followed by axonal degeneration. Electrodiagnostic findings support this process. Disruption of glycolipids, which are important to maintain ion channel clustering at the nodes and paranode, may impair nerve conduction. Genetic polymorphisms of Campylobacter jejuni determine the expression of the gangliosides on the bacterial wall. In contrast, target molecules in AIDP have not yet been identified. Meta-analyses show efficacy of plasmapheresis and immunoglobulin therapy, but not corticosteroids, in hastening recovery.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute-onset, monophasic, immune-mediated polyneuropathy that often follows an antecedent infection. The diagnosis relies heavily on the clinical impression obtained from the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually provide evidence supportive of the diagnosis. The clinician must also be familiar with mimics and variants to promptly and efficiently reach an accurate diagnosis. Intravenous immunoglobulin and plasma exchange are efficacious treatments. Supportive care during and following hospitalization is also crucial.     

Long-term outcome of Guillain-Barré syndrome

Objectives  

To investigate long-term neurological residua after Guillain-Barré syndrome (GBS) and to evaluate the predictive value of respiratory insufficiency during the acute stage of the disease.     

Epidemiology of the Guillain-Barré syndrome

This review focuses on recent epidemiological findings on Guillain-Barré syndrome regarding incidence, antecedent events related to the disease, prognosis and prognostic indicators, and treatment. Moreover, this review summarizes recent observations on clinical variants of Guillain-Barré syndrome and their relationship with the prevailing clinical presentation of the disease. The epidemiological observations which have advanced the understanding of the pathogenesis of Guillain-Barré syndrome are also discussed.     

New concepts of Guillain-Barré syndrome

Guillain-Barré syndrome is an acute autoimmune polyradiculoneuropathy with a clinical presentation of flaccid paralysis with areflexia, variable sensory disturbance, and elevated cerebrospinal fluid protein without pleocytosis. Although Guillain-Barré syndrome previously had been viewed as a unitary disorder with variations, it currently is viewed as a group of syndromes with several distinctive subtypes. These include the principal subtype prevalent in the Western world (acute inflammatory demyelinating polyradiculoneuropathy, and others, each with distinctive electrodiagnostic and pathologic features, including acute motor axonal neuropathy), acute motor-sensory axonal neuropathy, Miller Fisher syndrome, and perhaps others. The clinical and pathologic features of these Guillain-Barré syndrome subtypes are reviewed, and the role of antecedent infections, particularly Campylobacter jejuni gastroenteritis, and the role of antiganglioside antibody responses are reviewed with respect to pathogenesis. Treatment of Guillain-Barré syndrome includes both important supportive measures and immunotherapies, specifically high-dose intravenous immunoglobulin and plasma exchange.     

HIV-associated Guillain-Barré syndrome

Human immunodeficiency virus-associated Guillain-Barré syndrome (HIV-GBS) has been reported since 1985. Based on previous reports, this neuropathy typically occurs early in HIV infection, even at seroconversion, prior to developing acquired immunodeficiency syndrome (AIDS). Patients with GBS and CD4 counts of <50 have been proposed to have cytomegalovirus (CMV) infection and empiric gancyclovir is recommended. We reviewed medical records of 10 patients with HIV-GBS at five hospitals from 1986 to 1999. The mean CD4 count was 367/mm(3) (range 55-800). GBS was the first symptom of HIV infection in three patients. Four patients had AIDS with CD4 counts ranging from 55 to 190. CSF white blood cell (WBC) was 0 wbc/mm(3) in four patients, 2-10 wbc/mm(3) in three and 11-17 wbc/mm(3) in two. Three had recurrent weakness from 9 weeks to 4 years after the onset of symptoms, which persisted. HIV-GBS occurs in early and late stages of HIV infection, and may follow the onset of AIDS. No patients were seen with severe immunosuppression (CD4<50). A mild cerebrospinal fluid (CSF) pleocytosis in GBS suggests HIV infection, but is frequently absent. Compared to HIV-negative people, HIV-GBS may be associated with more frequent recurrent episodes or the development of CIDP.     

Sensory Guillain-Barré syndrome

OBJECTIVE: To report eight cases of sensory Guillain-Barré syndrome (GBS). BACKGROUND: The concept of sensory equivalent to ascending paralysis of GBS was raised in 1958, and the diagnostic criteria for a sensory loss and areflexia variant of GBS were proposed in 1981. However, clinical cases meeting these criteria have been relatively scarce. METHODS: During a 13-year period between 1986 and 1999, the authors collected eight cases of an acute sensory demyelinating neuropathy that met most of the proposed diagnostic criteria of a sensory variant of GBS. RESULTS: In all patients, sensory neuropathy was sudden at onset and peaked to maximal deficit within 4 weeks. In five (63%) cases, there was an antecedent viral illness. All patients had objective sensory loss and diminished or absent reflexes. None showed any muscle weakness. In all four patients in whom the spinal fluid was examined during the first 4 weeks, there was albuminocytologic dissociation. All of the patients had electrophysiologic evidence of demyelination in at least two nerves. Demyelination was demonstrated in motor nerve conduction in seven patients and in sensory nerve conduction in one, indicating that motor nerve conduction studies were the key for the diagnosis of demyelinating neuropathy. All patients had sensory nerve conduction abnormalities in at least one nerve. Three patients responded to immunotherapies. All had a favorable outcome, with a monophasic course of disease and no sign of relapse. CONCLUSION: The current study confirms the existence of sensory GBS.     

Fatal Guillain-Barré syndrome

Fourteen of 320 patients (4%) admitted with Guillain-Barré syndrome (GBS) died as a direct result of the illness. Deaths most commonly resulted from ventilator-associated pneumonia. In comparison with 101 other patients with severe GBS admitted to the intensive care unit, the patients who died were older (p = 0.006) and more likely to have underlying pulmonary disease (p = 0.004). In a specialized center, the primary event leading to death in GBS was ventilator-associated pulmonary infection, predominantly in elderly patients with significant comorbidity.     

Guillain-Barré syndrome.

The concepts of Guillain-Barré syndrome have changed substantially over the last 10 years, and the last 2 years have been no exception. Guillain-Barré syndrome is now recognized as a heterogeneous disorder with many clinical manifestations. Most current investigations are centered on the hypothesis of molecular mimicry. The major challenge now is to identify the precise mechanisms of nerve fiber injury and to determine how to prevent immune injury.     

Treatment of Guillain-Barré syndrome and CIDP

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating poly-(radiculo)neuropathy (CIDP) are immune-mediated disorders with a variable duration of progression and a range in severity of weakness. Infections can trigger GBS and exacerbate CIDP. Anti-ganglioside antibodies are important, but there is debate on the role of genetic factors in the pathogenesis of these disorders. Randomized controlled trials (RCT) have shown that intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in both GBS and CIDP. Most CIDP patients also improve after steroid therapy. Despite current treatment options, many patients have residual deficits or need to be treated for a long period of time. Therefore, new treatment trials are highly indicated. This review focuses on the current and possible new treatment options that could be guided by recent results from laboratory experiments.     

Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome

BACKGROUND: Pharyngeal-cervical-brachial weakness (PCB) is considered a variant of Guillain-Barré syndrome (GBS). Because of its rarity, there have been no studies of large numbers of patients with PCB. OBJECTIVE: To clarify the nosological classification of PCB. DESIGN: Retrospective study. SETTING: Academic research. Patients Medical records were reviewed of patients who manifested progressive weakness of the pharynx, neck, and upper limbs within 4 weeks of initial onset. MAIN OUTCOME MEASURES: Clinical features were analyzed, and antecedent infections and antiganglioside antibodies were investigated. RESULTS: Diagnoses for 100 patients were "pure PCB" (n = 13), PCB with preserved muscle stretch reflexes (n = 8), GBS overlap (n = 48), Fisher syndrome overlap (n = 26), and Bickerstaff brainstem encephalitis overlap (n = 5). Serological test results showed that 31.0% of antecedent infections in PCB were caused by Campylobacter jejuni. Of the antiganglioside antibodies tested, anti-GT1a IgG antibodies were positive in 51.0% of the patients. Anti-GQ1b IgG antibodies (a serological marker of Fisher syndrome and Bickerstaff brainstem encephalitis) were positive in 39.0%. The IgG antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a (serological markers of an axonal GBS subtype) were positive in 27.0%. CONCLUSION: This large study identified the clinical profiles of PCB. Clinical overlapping, frequent C jejuni infection, and common antiganglioside antibodies present in PCB, GBS, Fisher syndrome, and Bickerstaff brainstem encephalitis provide conclusive evidence that PCB and these conditions form a continuous spectrum.     

Guillain-Barré syndrome as the first manifestation of POEMS syndrome

POEMS syndrome is a rare multisystem disorder, characterized by the presence of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. The variety of clinical pictures and asynchronous manifestation of dominant features make diagnosis difficult. We report a case of a 42-year-old man with polyneuropathy who was initially negative for monoclonal protein and so Guillain-Barré syndrome was diagnosed. Other signs and symptoms, including monoclonal gammopathy, developed later in the course of the disease and finally POEMS syndrome was diagnosed.     

Characteristics of Guillain-Barré syndrome in super-elderly individuals

Journal of Neurology - Japan has the world’s largest super-aging population, and the number of elderly patients with various diseases is increasing. Herein, we reported the characteristics of...