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Multicenter Study
. 2022 Dec;29(12):3547-3555.
doi: 10.1111/ene.15523. Epub 2022 Aug 29.

Anti-disialosyl-immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Claire Peillet  1   2 , David Adams  1   2   3 , Shahram Attarian  4 , Françoise Bouhour  5 , Cécile Cauquil  1   2   3 , Julien Cassereau  6 , Jean-Baptiste Chanson  7 , Pascal Cintas  8 , Alain Creange  9 , Emilien Delmont  4 , Guillaume Fargeot  10 , Steeve Genestet  11 , Antoine Gueguen  12 , Anne Laure Kaminsky  13 , Thierry Kuntzer  14 , Céline Labeyrie  1   2   3 , Maud Michaud  13 , Yann Pereon  15 , Angela Puma  16 , Karine Viala  10 , Pascale Chretien  17   18 , Clovis Adam  19 , Andoni Echaniz-Laguna  1   2   3
Affiliations
Multicenter Study

Anti-disialosyl-immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Claire Peillet et al. Eur J Neurol. 2022 Dec.

Abstract

Background and purpose: In this retrospective study involving 14 university hospitals from France and Switzerland, the aim was to define the clinicopathological features of chronic neuropathies with anti-disialosyl ganglioside immunoglobulin M (IgM) antibodies (CNDA).

Results: Fifty-five patients with a polyneuropathy evolving for more than 2 months and with at least one anti-disialosyl ganglioside IgM antibody, that is, anti-GD1b, -GT1b, -GQ1b, -GT1a, -GD2 and -GD3, were identified. Seventy-eight percent of patients were male, mean age at disease onset was 55 years (30-76) and disease onset was progressive (82%) or acute (18%). Patients presented with limb sensory symptoms (94% of cases), sensory ataxia (85%), oculomotor weakness (36%), limb motor symptoms (31%) and bulbar muscle weakness (18%). Sixty-five percent of patients had a demyelinating polyradiculoneuropathy electrodiagnostic profile and 24% a sensory neuronopathy profile. Anti-GD1b antibodies were found in 78% of cases, whilst other anti-disialosyl antibodies were each observed in less than 51% of patients. Other features included nerve biopsy demyelination (100% of cases), increased cerebrospinal fluid protein content (75%), IgM paraprotein (50%) and malignant hemopathy (8%). Eighty-six percent of CNDA patients were intravenous immunoglobulins-responsive, and rituximab was successfully used as second-line treatment in 50% of cases. Fifteen percent of patients had mild symptoms and were not treated. CNDA course was progressive (55%) or relapsing (45%), and 93% of patients still walked after a mean disease duration of 11 years.

Conclusion: Chronic neuropathies with anti-disialosyl ganglioside IgM antibodies have a recognizable phenotype, are mostly intravenous immunoglobulins-responsive and present with a good outcome in a majority of cases.

Keywords: anti-disialosyl antibodies; chronic inflammatory demyelinating polyneuropathy; sensory neuronopathy.

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References

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