A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside

Pestronk, Alan; Cornblath, David R.; Ilyas, Amjad A.; Baba, Hiroko; Quarles, Richard H.; Griffin, John W.; Alderson, Kathy; Adams, Robert N.

doi:10.1002/ana.410240113

Cited by 585 publications

(331 citation statements)

References 28 publications

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“…This is important as different neuropathies may require specific therapeutic strategies (Nobile‐Orazio, 2014; Pestronk et al., 1988; van Schaik, van den Berg, de Haan, & Vermeulen, 2005). Comparative studies of brachial plexus MRI have not been performed.…”

Section: Introductionmentioning

confidence: 99%

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

et al. 2017

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ObjectiveThe main objective of this study was to evaluate the correlation between the distribution of brachial plexus magnetic resonance imaging (MRI) abnormalities and clinical weakness, and to evaluate the value of brachial plexus MRI in predicting disease course and response to treatment in multifocal motor neuropathy (MMN), Lewis‐Sumner syndrome (LSS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).MethodsSixty‐seven patients with an inflammatory neuropathy diagnosed at our tertiary referral center for neuromuscular diseases had undergone bilateral T2‐weighted short tau inversion recovery (STIR) MRI of the brachial plexus. We obtained clinical follow‐up data and scored all MRIs for abnormalities and the symmetry of their distribution.ResultsBrachial plexus MRI abnormalities were detected in 45% of the patients. An abnormal MRI did not predict disease course in terms of patterns of weakness, sensory disturbances or response to treatment. Within the spectrum of radiological abnormalities, asymmetrical clinical syndromes, MMN and LSS were significantly associated with asymmetrical radiological abnormalities, whereas symmetrical abnormalities predominated in CIDP (p < .001, phi 0.791).ConclusionT2 STIR brachial plexus MRI abnormalities correspond with the distribution of neurological deficits in inflammatory neuropathies, but do not correlate with specific clinical characteristics, response to treatment or disease course.

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Section: Introductionmentioning

confidence: 99%

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

et al. 2017

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“…Subsequently, Parry and Clarke 29 (and in a separate report, Pestronk et al 30 ), reported similar patients. Since then, there have been a number of studies describing patients who have multifocal motor neuropathy (MMN) with persistent conduction block.…”

Section: Multifocal Motor Neuropathymentioning

confidence: 78%

Inflammatory Demyelinating Neuropathies and Neuropathies Associated with Monoclonal Gammopathies: Treatment Update

2008

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Summary:This review focuses on recent data regarding inflammatory demyelinating neuropathies and neuropathies associated with monoclonal gammopathies. We describe both acute and chronic inflammatory neuropathies, and we discuss conditions ranging from mostly cell-mediated to antibody-mediated disorders. These diseases are characterized by proximal and distal sensory motor involvement. Treatments are based on immune-modulation and/or immune-suppression. Work-up sequence and therapeutical modes are discussed in the light of recently published data, with a special interest on new treatment modalities.

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“…However, several relevant autoantibodies in nonparaneoplastic inflammatory neuropathies are of the IgM isotype. This includes anti‐MAG antibodies associated to monoclonal gammopathy of unknown significance and plasma cell dyscrasias,30 anti‐GM1 antibodies in multifocal motor neuropathy31, and anti‐disialosyl antibodies in CANOMAD syndrome 32. Four out of 10 patients with sensory‐motor neuropathy stained Schwann cells.…”

Section: Discussionmentioning

confidence: 99%

Antibodies against cell adhesion molecules and neural structures in paraneoplastic neuropathies

Siles

Martínez‐Hernández

Araque

et al. 2018

Ann Clin Transl Neurol

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ObjectiveParaneoplastic neurological syndromes (PNS) are rare neurological disorders in which ectopic expression of neural antigens by a tumor results in an autoimmune attack against the nervous system. Onconeural antibodies not only guide PNS diagnosis but may also help detecting underlying malignancies. Our project aims to uncover new potential antibodies in paraneoplastic neuropathies (PN).MethodsThirty‐four patients fulfilling diagnostic criteria of possible (n = 9; 26.5%) and definite (n = 25; 73.5%) PN without onconeural antibodies and 28 healthy controls were included in our study. Sera were tested for known antibodies against neural cell adhesion molecules and screened for novel IgG and IgM reactivities against nerve components: dorsal root ganglia (DRG) neurons, motor neurons, and Schwann cells. Patients showing autoantibodies against any of these cell types were used for immunoprecipitation (IP) studies.ResultsOverall, 9 (26.5%) patients showed significant reactivity against DRG neurons, motor neurons, or Schwann cells, whereas 5 (17.9%) healthy controls only showed moderate reactivity. Compared with control sera, serum samples from patients with paraneoplastic sensory‐motor neuropathies had a higher frequency of IgM antibodies against Schwann cells (0% vs. 40%; P = 0.0028). No novel antigens were identified from our IP experiments. Antibodies against the neural adhesion molecules CNTN1, NF155, NF140, NF186, NCAM1, L1CAM, and the CNTN1/CASPR1 complex were not detected in patients with PN. One (2.9%) patient with CIDP and thymoma had CASPR2 antibodies.InterpretationAlmost 30% of patients with PN harbor antibodies targeting neural structures, suggesting that novel neoplasm‐associated antigens remain to be discovered.

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A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside

Cited by 585 publications

References 28 publications

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

Inflammatory Demyelinating Neuropathies and Neuropathies Associated with Monoclonal Gammopathies: Treatment Update

Antibodies against cell adhesion molecules and neural structures in paraneoplastic neuropathies

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