期刊
NEUROLOGY
卷 84, 期 24, 页码 2403-2412出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/wnl.0000000000001682
关键词
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资金
- Fondo de Investigaciones Sanitarias, Madrid, Spain [PI12/00611]
- NIH [RO1NS077851, RO1MH094741]
- Instituto Carlos III (FIS, Spain) [14/00203]
- Fundacio la Marato de TV3
- Euroimmun
- ErasmusMC fellowship
- Netherlands Organisation for Scientific Research (NWO, Veni incentive)
- Dutch Epilepsy Foundation [14-19]
- ICREA Funding Source: Custom
Objective: We report the clinical features, comorbidities, and outcome of 22 newly identified patients with antibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR). Methods: This was a retrospective review of patients diagnosed between May 2009 and March 2014. Immunologic techniques have been reported previously. Results: Patients' median age was 62 years (range 23-81; 14 female). Four syndromes were identified: 12 (55%) patients presented with distinctive limbic encephalitis (LE), 8 (36%) with limbic dysfunction along with multifocal/diffuse encephalopathy, one with LE preceded by motor deficits, and one with psychosis with bipolar features. Fourteen patients (64%) had a tumor demonstrated pathologically (5 lung, 4 thymoma, 2 breast, 2 ovarian teratoma) or radiologically (1 lung). Additional antibodies occurred in 7 patients (3 onconeuronal, 1 tumor-related, 2 cell surface, and 1 tumor-related and cell surface), all with neurologic symptoms or tumor reflecting the concurrent autoimmunity. Treatment and outcome were available from 21 patients (median follow-up 72 weeks, range 5-266): 5 had good response to immunotherapy and tumor therapy, 10 partial response, and 6 did not improve. Eventually 5 patients died; all had a tumor or additional paraneoplastic symptoms related to onconeuronal antibodies. Coexistence of onconeuronal antibodies predicted a poor outcome (p = 0.009). Conclusion: Anti-AMPAR encephalitis usually manifests as LE, can present with other symptoms or psychosis, and is paraneoplastic in 64% of cases. Complete and impressive neurologic improvement can occur, but most patients have partial recovery. Screening for a tumor and onconeuronal antibodies is important because their detection influences outcome.
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