Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy

Attarian, Shahram; Boucraut, José; Hubert, A.; Uzenot, D.; Delmont, Émilien; Verschueren, Annie; Franques, Jérôme; Azulay, Jean‐Philippe; Pouget, Jean

doi:10.1136/jnnp.2009.185736

Cited by 50 publications

(34 citation statements)

References 32 publications

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“…The disease process in CANDA may be the result of antibody mediated attack of the nerve root, DRG, and nerves 111. In electrophysiologic studies, patients with CANDA have absent or reduced sensory responses and diminished motor responses, including demyelinating features 118119…”

Section: Differential Diagnosis Of Sensory Ataxiamentioning

confidence: 99%

Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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Sensory polyneuropathies, which are caused by dysfunction of peripheral sensory nerve fibers, are a heterogeneous group of disorders that range from the common diabetic neuropathy to the rare sensory neuronopathies. The presenting symptoms, acuity, time course, severity, and subsequent morbidity vary and depend on the type of fiber that is affected and the underlying cause. Damage to small thinly myelinated and unmyelinated nerve fibers results in neuropathic pain, whereas damage to large myelinated sensory afferents results in proprioceptive deficits and ataxia. The causes of these disorders are diverse and include metabolic, toxic, infectious, inflammatory, autoimmune, and genetic conditions. Idiopathic sensory polyneuropathies are common although they should be considered a diagnosis of exclusion. The diagnostic evaluation involves electrophysiologic testing including nerve conduction studies, histopathologic analysis of nerve tissue, serum studies, and sometimes autonomic testing and cerebrospinal fluid analysis. The treatment of these diseases depends on the underlying cause and may include immunotherapy, mitigation of risk factors, symptomatic treatment, and gene therapy, such as the recently developed RNA interference and antisense oligonucleotide therapies for transthyretin familial amyloid polyneuropathy. Many of these disorders have no directed treatment, in which case management remains symptomatic and supportive. More research is needed into the underlying pathophysiology of nerve damage in these polyneuropathies to guide advances in treatment.

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Section: Differential Diagnosis Of Sensory Ataxiamentioning

confidence: 99%

Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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“…The disorder has similarities to the Miller-Fisher variant of GuillainBarré syndrome, where there is an acute-phase IgG response against GQ1b and GT1a, but CANOMAD is chronic and progressive rather than being subacute and monophasic [69]. Case reports and series suggest that patients with CANOMAD may improve with rituximab [70], or intravenous immunoglobulin (IVIg) therapy [71].…”

Section: Canomadmentioning

confidence: 99%

An Update on Monoclonal Gammopathy and Neuropathy

Ramchandren

Lewis

2011

Curr Neurol Neurosci Rep

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Peripheral neuropathy associated with monoclonal gammopathy is a rare but important cause of neuropathy that can herald serious underlying disease. IgM monoclonal gammopathy of undetermined significance (MGUS) is the most commonly found monoclonal gammopathy associated with neuropathy, with characteristic clinical, electrophysiologic, and pathologic features. The IgG and IgA monoclonal gammopathies are rarely associated with specific neuropathies. Standard immunomodulatory agents including steroids, intravenous immunoglobulin, and plasmapheresis have shown limited efficacy in IgM MGUS. Neuropathies associated with specific lymphoproliferative disorders may not respond to treatments aimed at that disorder. Case series had shown promising results with rituximab, a monoclonal antibody that targets the B cell surface antigen CD20 and results in a rapid and sustained depletion of B cells; however, two recent randomized controlled trials with rituximab failed to provide evidence of efficacy in primary outcome measures, despite reduction in antibody levels. Long-term studies looking at the association between specific immunologic markers and disease recurrence are needed to ultimately develop targeted therapies.

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“…These patients have a sensory ataxic predominant presentation with extraocular and bulbar symptoms similar to Miller Fisher variant but with a chronic course. Although the exact prevalence of this disorder is not clear, partial response to immunomodulatory treatment has been reported [25], and therefore, laboratory testing in patients with appropriate clinical presentation and IgM paraproteins may provide a therapeutic option. A similar condition, referred to as GALOP syndrome (gait ataxia lateonset polyneuropathy), is also seen in the presence of serum IgM gammopathy.…”

Section: Anti-sulfatide Antibodymentioning

confidence: 99%

Advances in the Laboratory Evaluation of Peripheral Neuropathies

Huan

Bromberg

2011

Curr Neurol Neurosci Rep

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Peripheral neuropathy is a common clinical problem in neurology and laboratory testing is an integral part of diagnosis. In the past few years, practice parameters have been published to establish an evidence-based guide to neuropathy testing. There are many tests that are common and accepted in practice, but there is no clear mandate on which tests are necessary. This article reviews recent practice parameter publications as well as other updates in laboratory testing for peripheral neuropathy, including the roles of lipid profile, genetic testing, antibody titers, and B(12) testing in a specific dopamine-treated Parkinson's patients. These can serve as recommendations to help guide laboratory testing.

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Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy

Cited by 50 publications

References 32 publications

Diagnosis and management of sensory polyneuropathy

Diagnosis and management of sensory polyneuropathy

An Update on Monoclonal Gammopathy and Neuropathy

Advances in the Laboratory Evaluation of Peripheral Neuropathies

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