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Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis
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Edité par CCSD ; American Academy of neurology -
International audience. Objective: To report paroxysmal episodes of cerebellar ataxia in a patient with anti–contactin- associated protein-like 2 (CASPR2) antibody-related autoimmune encephalitis and to search for similar paroxysmal ataxia in a cohort of patients with anti–CASPR2 antibody-associated autoim- mune encephalitis. Methods: We report a patient with paroxysmal episodes of cerebellar ataxia observed during auto- immune encephalitis with anti-CASPR2 antibodies. In addition, clinical analysis was performed in a retrospective cohort of 37 patients with anti-CASPR2 antibodies to search for transient epi- sodes of ataxia. Paroxysmal symptoms were further specified from the referral physicians, the pa- tients, or their relatives. Results: A 61-year-old man with limbic encephalitis and anti-CASPR2 antibodies developed ste- reotyped paroxysmal episodes of cerebellar ataxia, including gait imbalance, dysarthria, and dys- metria, 1 month after the onset of the encephalitis. The ataxic episodes were specifically triggered by orthostatism and emotions. Both limbic symptoms and transient ataxic episodes resolved after treatment with steroids and IV cyclophosphamide. Among 37 other patients with anti-CASPR2 antibodies, we identified 5 additional cases with similar paroxysmal ataxic episodes that included gait imbalance (5 cases), slurred speech (3 cases), limb dysmetria (3 cases), and nys- tagmus (1 case). All had concomitant limbic encephalitis. Paroxysmal ataxia was not observed in patients with neuromyotonia or Morvan syndrome. Triggering factors (orthostatism or anger) were reported in 4 patients. Episodes resolved with immunomodulatory treatments in 4 patients and spontaneously in 1 case.
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