Anti-disialosyl-immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study.
anti-disialosyl antibodies
chronic inflammatory demyelinating polyneuropathy
sensory neuronopathy
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
12 2022
12 2022
Historique:
revised:
30
07
2022
received:
02
06
2022
accepted:
11
08
2022
pubmed:
16
8
2022
medline:
4
11
2022
entrez:
15
8
2022
Statut:
ppublish
Résumé
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). 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. 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.
Sections du résumé
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.
Substances chimiques
Immunoglobulin M
0
Immunoglobulins, Intravenous
0
Gangliosides
0
Types de publication
Multicenter Study
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3547-3555Informations de copyright
© 2022 European Academy of Neurology.
Références
Cutillo G, Saariaho A-H, Meri S. Physiology of gangliosides and the role of antiganglioside antibodies in human diseases. Cell Mol Immunol. 2020;17(4):313-322.
Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies. Brain. 2002;125(Pt 12):2591-2625.
Ilyas AA, Quarles RH, Dalakas MC, Fishman PH, Brady RO. Monoclonal IgM in a patient with paraproteinemic polyneuropathy binds to gangliosides containing disialosyl groups. Ann Neurol. 1985;18(6):655-659.
Willison HJ, O'Leary CP, Veitch J, et al. The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain. 2001 Oct;124(Pt 10):1968-1977.
Attarian S, Boucraut J, Hubert AM, et al. Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy. J Neurol Neurosurg Psychiatry. 2010;81(1):61-64.
Garcia-Santibanez R, Zaidman CM, Sommerville RB, et al. CANOMAD and other chronic ataxic neuropathies with disialosyl antibodies (CANDA). J Neurol. 2018;265(6):1402-1409.
Le Cann M, Bouhour F, Viala K, et al. CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell-targeted therapies. Blood. 2020;136(21):2428-2436.
Moshe-Lilie O, Ensrud E, Ragole T, Nizar C, Dimitrova D, Karam C. CIDP mimics: a case series. BMC Neurol. 2021;21(1):94.
Halpin S. A case of CANOMAD with review of the literature. Brain Disord Ther. 2015 Accessed August 1, 2021;4(3):1-4. Available from:. http://www.omicsgroup.org/journals/a-case-of-canomad-with-review-of-the-literature-2168-975X-1000166.php?aid=55923
Ahdab R, Lefaucheur JP, Malapert D, et al. Neuropathy with anti-disialosyl IgM antibodies and multifocal persistent motor conduction blocks. J Neurol Neurosurg Psychiatry. 2009;80(6):700-702.
Chabraoui F, Derrington EA, Mallie-Didier F, Confavreux C, Quincy C, Caudie C. Dot-blot immunodetection of antibodies against GM1 and other gangliosides on PVDF-P membranes. J Immunol Methods. 1993;165(2):225-230.
Van den Bergh PYK, Hadden RDM, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society-first revision. Eur J Neurol. 2010;17(3):356-363.
Camdessanché J-P, Jousserand G, Ferraud K, et al. The pattern and diagnostic criteria of sensory neuronopathy: a case-control study. Brain. 2009;132(7):1723-1733.
Fargeot G, Echaniz-Laguna A. Sensory neuronopathies: new genes, new antibodies and new concepts. J Neurol Neurosurg Psychiatry. 2021;92:398-406.
Kam C, Balaratnam MS, Purves A, et al. CANOMAD presenting without ophthalmoplegia and responding to intravenous immunoglobulin. Muscle Nerve. 2011;44(5):829-833.
Tagliapietra M, Zanusso G, Ferrari S, et al. Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b. J Peripher Nerv Syst. 2020;25:54-59.
Fargeot G, Viala K, Theaudin M, et al. Diagnostic usefulness of plexus magnetic resonance imaging in chronic inflammatory demyelinating polyradiculopathy without electrodiagnostic criteria of demyelination. Eur J Neurol. 2019;26(4):631-638.
Kusunoki S, Shimizu J, Chiba A, Ugawa Y, Hitoshi S, Kanazawa I. Experimental sensory neuropathy induced by sensitization with ganglioside GD1b. Ann Neurol. 1996;39(4):424-431.
Obi T, Murakami T, Takatsu M, et al. Clinicopathological study of an autopsy case with sensory-dominant polyradiculoneuropathy with antiganglioside antibodies. Muscle Nerve. 1999;22(10):1426-1431.
McKelvie PA, Gates PC, Day T. CANOMAD: report of a case with a 40-year history and autopsy. Is this a sensory ganglionopathy with neuromuscular junction blockade? Muscle Nerve. 2013;48(4):599-603.
Yuki N, Uncini A. Acute and chronic ataxic neuropathies with disialosyl antibodies: a continuous clinical spectrum and a common pathophysiological mechanism. Muscle Nerve. 2014;49(5):629-635.
Clark AJ, Kaller MS, Galino J, Willison HJ, Rinaldi S, Bennett DLH. Co-cultures with stem cell-derived human sensory neurons reveal regulators of peripheral myelination. Brain. 2017;140(4):898-913.
Conrad K, Schneider H, Ziemssen T, et al. A new line immunoassay for the multiparametric detection of antiganglioside autoantibodies in patients with autoimmune peripheral neuropathies. Ann N Y Acad Sci. 2007;1109:256-264.
Johannis W, Renno JH, Klatt AR, Wielckens K. Anti-glycolipid antibodies in patients with neuropathy: a diagnostic assessment. J Clin Neurosci. 2014;21:488-492.
Lardone RD, Alaniz ME, Irazoqui FJ, Nores GA. Unusual presence of antiGM1 IgG-antibodies in a healthy individual, and their possible involvement in the origin of disease-associated anti-GM1 antibodies. J Neuroimmunol. 2006;173:174-179.