Non-anti-MAG DADS neuropathy as a variant of CIDP: clinical, electrophysiological, laboratory features and response to treatment in 10 cases

Larue, Sandrine; Bombelli, Francesco; Viala, Karine; Neil, Jean; Maisonobe, Thierry; Bouché, P; Musset, Lucile; Fournier, Emmanuel; Léger, Jean‐Marc

doi:10.1111/j.1468-1331.2010.03312.x

Cited by 80 publications

(64 citation statements)

References 36 publications

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“…DADS associated with positivity for anti-MAG antibodies, termed anti-MAG neuropathy, is separated from CIDP according to the 2010 EFNS/PNS guidelines [3], [22]. In contrast, DADS without anti-MAG antibodies is often considered to be a variant of CIDP, and some reports have described differences in the response to immune treatment between DADS patients with and without anti-MAG antibodies [23], [24]. In the present study, we assessed the effects of sera obtained from three patients with DADS without anti-MAG antibodies, and found that the level of BNB damage after exposure to the sera from these patients was milder than that observed following exposure to the sera of the t-CIDP patients.…”

Section: Discussionmentioning

confidence: 99%

Severity and Patterns of Blood-Nerve Barrier Breakdown in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Correlations with Clinical Subtypes

et al. 2014

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ObjectiveChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is currently classified into clinical subtypes, including typical and atypical forms (multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) and distal acquired demyelinating symmetric neuropathy (DADS)). The aim of this study was to elucidate the patterns and severity of breakdown of the blood-nerve barrier (BNB) in each CIDP subtype.MethodsWe evaluated the effects of sera obtained from patients with typical CIDP, MADSAM and DADS and control subjects on the expression levels of tight junction proteins and transendothelial electrical resistance (TEER) value in human peripheral nerve microvascular endothelial cells (PnMECs).ResultsThe sera obtained from the patients with the three clinical phenotypes of CIDP decreased the amount of claudin-5 protein levels and TEER values in the PnMECs. In addition, the sera obtained from typical CIDP patients more prominently reduced claudin-5 protein levels and TEER values in the PnMECs than did that obtained from the MADSAM and DADS patients. Furthermore, the severity of BNB disruption after exposure to the sera was associated with higher Hughes grade, lower MRC score, more pronounced slowing of motor nerve conduction in the median nerve and higher frequency of abnormal temporal dispersion.ConclusionsSera derived from typical CIDP patients destroy the BNB more severely than those from MADSAM or DADS patients. The extent of BNB disruption in the setting of CIDP is associated with clinical disability and demyelination in the nerve trunk. These observations may explain the phenotypical differences between CIDP subtypes.

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Section: Discussionmentioning

confidence: 99%

Severity and Patterns of Blood-Nerve Barrier Breakdown in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Correlations with Clinical Subtypes

et al. 2014

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“…There is increasing evidence that DADS (without M) is a variant of CIDP and is truly distinct from DADS-M particularly in response to treatment [52]. One issue that remains unclear is whether DADS-M with anti-MAG antibodies is distinct from DADS-M without anti-MAG.…”

Section: Monoclonal Gammopathy Of Undetermined Significance (Mgus)mentioning

confidence: 92%

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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“…Patients who have an identical clinical and electrophysiologic phenotype but lack MAG antibodies can be classified as having DADS-CIDP36127; such patients may carry a better prognosis and respond more favorably to intravenous immunoglobulins, corticosteroids, and plasma exchange 127. The clinical hallmark of DADS neuropathy is the gradual onset of sensory ataxia resulting from impaired proprioception 128.…”

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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Non-anti-MAG DADS neuropathy as a variant of CIDP: clinical, electrophysiological, laboratory features and response to treatment in 10 cases

Abstract: DADS neuropathy without anti-MAG antibodies is more likely to be considered a variant of CIDP. In addition, such patients should be systematically investigated for an associated haematological or immunological condition.

Cited by 80 publications

References 36 publications

Severity and Patterns of Blood-Nerve Barrier Breakdown in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Correlations with Clinical Subtypes

Severity and Patterns of Blood-Nerve Barrier Breakdown in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Correlations with Clinical Subtypes

An Update on Monoclonal Gammopathy and Neuropathy

Diagnosis and management of sensory polyneuropathy

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