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- Title
CANOMAD and other chronic ataxic neuropathies with disialosyl antibodies (CANDA).
- Authors
Garcia-Santibanez, Rocio; Zaidman, Craig M.; Sommerville, R. Brian; Lopate, Glenn; Weihl, Conrad C.; Pestronk, Alan; Bucelli, Robert C.
- Abstract
Introduction: CANOMAD/CANDA are syndromes characterized by ataxic neuropathy, ophthalmoplegia, monoclonal gammopathy, cold agglutinins and disialosyl antibodies.Methods: A retrospective review of our neuromuscular autoantibody panel database was performed. Anti-GD1b seropositive patients with ataxia were included.Results: Eleven patients were identified. Median age at onset was 56 years. Median disease duration was 6 years. All patients had gait disorders. Nine had ocular motility abnormalities. Most had a monoclonal protein and all had elevated serum IgM. Electrodiagnostic studies showed a mixed axonal/demyelinating pattern (6), an axonal pattern (4), or a pure demyelinating pattern (1). Ultrasounds showed nerve enlargement patterns consistent with acquired demyelination. A nerve biopsy showed near complete loss of myelinated axons with preservation of smaller axons. Rituximab was the most effective immunotherapy.Conclusion: CANOMAD/CANDA are rare and debilitating disorders with characteristic clinical and diagnostic findings. In our cohort, nerve ultrasound showed regional nerve enlargement and rituximab was the most effective immunomodulatory therapy.
- Subjects
PERIPHERAL neuropathy; EYE paralysis; MONOCLONAL gammopathies; AUTOANTIBODIES; DEMYELINATION; MONOCLONAL antibodies
- Publication
Journal of Neurology, 2018, Vol 265, Issue 6, p1402
- ISSN
0340-5354
- Publication type
Article
- DOI
10.1007/s00415-018-8853-4