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Neurology. 2015 Jun 16;84(24):2403-12. doi: 10.1212/WNL.0000000000001682. Epub 2015 May 15.

Encephalitis and AMPA receptor antibodies: Novel findings in a case series of 22 patients.

Author information

1
From the Service of Neurology (R.H., L.S., A.S., F.G.), Hospital Clínic, Universitat de Barcelona and Institut d´Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Spain; the Institute of Neurology (R.H.), Medical University of Vienna, Austria; the Department of Neurology (A.v.S., M.J.T.), Erasmus Medical Center, Rotterdam; the Department of Neurology (A.v.S.), Haga Hospital, the Hague, the Netherlands; the Department of Neurology and Institute of Clinical Chemistry (F.L.), University Medical Center Schleswig-Holstein Campus Lübeck, Germany; the Department of Neurology (D.H.), Ochsner Health System, New Orleans, LA; the Memory and Aging Center (M.G., J.G.) and the Department of Neurology (M.P.), University of California, San Francisco; Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), IDIBAPS, Hospital Clínic, Barcelona, Spain; and the Department of Neurology (J.D.), University of Pennsylvania, Philadelphia.
2
From the Service of Neurology (R.H., L.S., A.S., F.G.), Hospital Clínic, Universitat de Barcelona and Institut d´Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Spain; the Institute of Neurology (R.H.), Medical University of Vienna, Austria; the Department of Neurology (A.v.S., M.J.T.), Erasmus Medical Center, Rotterdam; the Department of Neurology (A.v.S.), Haga Hospital, the Hague, the Netherlands; the Department of Neurology and Institute of Clinical Chemistry (F.L.), University Medical Center Schleswig-Holstein Campus Lübeck, Germany; the Department of Neurology (D.H.), Ochsner Health System, New Orleans, LA; the Memory and Aging Center (M.G., J.G.) and the Department of Neurology (M.P.), University of California, San Francisco; Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), IDIBAPS, Hospital Clínic, Barcelona, Spain; and the Department of Neurology (J.D.), University of Pennsylvania, Philadelphia. jdalmau@clinic.ub.es.

Abstract

OBJECTIVE:

We report the clinical features, comorbidities, and outcome of 22 newly identified patients with antibodies to the α-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.

PMID:
25979696
PMCID:
PMC4478035
DOI:
10.1212/WNL.0000000000001682
[Indexed for MEDLINE]
Free PMC Article

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