Relapsing sensorimotor neuropathy with ophthalmoplegia, antidisialosyl antibodies, and extramembranous glomerulonephritis

Delval, Arnaud; Stojkovic, Tanya; Vermersch, Patrick

doi:10.1002/mus.20452

Cited by 12 publications

(9 citation statements)

References 22 publications

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“…However, these neuropathies are rare, and series such as this one nevertheless provide useful information about the range of responses to IVIg obtained in patients with these neuropathies. One important clinical aspect to which this study leads, in keeping with previous studies,3 16 17 20 31 is that anti-GD1b antibodies should be measured in all patients with otherwise idiopathic sensory ataxic neuropathies, even in the absence of monoclonal IgM paraprotein or demyelinating features detected at NCS or features of CANOMAD.…”

Section: Discussionsupporting

confidence: 55%

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

Attarian

Boucraut

Hubert

et al. 2009

Journal of Neurology, Neurosurgery & Psychiatry

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Methods: patients with chronic sensory ataxic neuropathy associated with anti-GD1b IgM antibodies followed in our department for at least 12 months between 2001 and 2008 were identified and studied retrospectively. Patients were tested at regular intervals using the INCAT disability score. Patients whose disability scores improved by at least one point were taken to have responded to the treatment. Intravenous immunoglobulin (IVIg; 2 g/kg) was administered for 3 to 5 days once every 6 weeks or corticosteroids at an initial daily dose of 1 mg/kg.

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Section: Discussionsupporting

confidence: 55%

“…Plasmapheresis improved in some of the patients treated,1 14 whereas only a few of the patients treated with corticosteroids improved 5 9 11 15 16 17 18 19 20 21. Intravenous immunoglobulin (IVIg) has been used in a few cases,9 16 17 19 21 and half of these patients improved. Anti-CD20 antibody has been attempted successfully on one patient 21.…”

mentioning

confidence: 99%

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

Attarian

Boucraut

Hubert

et al. 2009

Journal of Neurology, Neurosurgery & Psychiatry

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“…Three cases have been published previously as case reports. 21,25,26 Investigations Detection of monoclonal gammopathies, cold agglutinins, and antiganglioside antibodies were performed locally and were part of variables collected in the standardized questionnaire. Methods testing for the presence of antidisialosyl antibodies comprised enzyme-linked immunosorbent assay, 6,27 "home-made" immunodots, 28 or commercial tests (Generic Assays, Les Ulis, Courtaboeuf, France).…”

Section: Patientsmentioning

confidence: 99%

CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell–targeted therapies

Cann

Bouhour

Viala

et al. 2020

Blood

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CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies) is a rare syndrome characterized by chronic neuropathy with sensory ataxia, ocular and/or bulbar motor weakness, in the presence of a monoclonal IgM reacting against gangliosides containing disialosyl epitopes. Data regarding associated hematologic malignancies and effective therapies in CANOMAD are scarce. We conducted a French multicenter retrospective study that included 45 patients with serum IgM antibodies reacting against disialosyl epitopes in the context of evocating neurological symptoms. Main clinical features were sensitive symptoms (ataxia, paresthesia, hypoesthesia) (n=45, 100%), motor weakness (n=18, 40%), ophthalmoplegia (n=20, 45%) and bulbar symptoms (n=6, 13%). Forty five percent of the cohort had moderate to severe disability (modified Rankin score, 3-5). Cold agglutinins were identified in 15 (34%) patients. Electrophysiological studies showed a demyelinating or axonal pattern in respectively 60 and 27% of cases. All patients had serum monoclonal IgM gammopathy (median, 2.6 g/L; range, 0.1-40). Overt hematologic malignancies were diagnosed in 16 patients (36%), most frequently Waldenström macroglobulinemia (n=9, 20%). Forty-one patients (91%) required treatment for CANOMAD. Intravenous immunoglobulins (IVIg) and rituximab-based regimens were the most effective therapies with respectively 53 and 52% of partial or better clinical responses. Corticosteroids and immunosuppressive drugs were largely ineffective. Although more studies are warranted to better define the optimal therapeutic sequence, IVIg should be proposed as the standard of care for first-line and rituximab-based regimens for second-line treatment. These compiling data argue for CANOMAD to be included in neurological monoclonal gammopathy of clinical significance.

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“…The presence of cold agglutinins varied according to the case. The clinical picture is variable as the antibody responds against desialylated gangliosides [8] ( Figure 2).…”

Section: Discussionmentioning

confidence: 99%

CANOMAD: A Multi-Faceted Disease

Rickli¹,

Pomaleski²,

Gonçalves³

2020

Int J Neurol Neurother

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Relapsing sensorimotor neuropathy with ophthalmoplegia, antidisialosyl antibodies, and extramembranous glomerulonephritis

Cited by 12 publications

References 22 publications

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

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

CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell–targeted therapies

CANOMAD: A Multi-Faceted Disease

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