학술논문

Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy.
Document Type
Article
Source
Acta Neurologica Scandinavica. Jan2017, Vol. 135 Issue 1, p134-141. 8p.
Subject
*TREATMENT of encephalitis
*IMMUNOGLOBULINS
*AUTOIMMUNE diseases
*IMMUNOTHERAPY
*PEOPLE with epilepsy
*TEMPORAL lobe epilepsy
*THERAPEUTICS
Language
ISSN
0001-6314
Abstract
Objectives Whether and when to immunologically treat epilepsy patients with suggested autoantibody ( AB)-negative limbic encephalitis ( LE) is clinically challenging. Therefore, we evaluated the clinical outcome and eventual outcome predictors of immunotherapy in a group of AB-negative patients with recent-onset temporal lobe epilepsy ( TLE), magnetic resonance imaging ( MRI) indicators of LE, subjective cognitive decline, and/or psychiatric symptoms. Methods This retrospective, observational, uncontrolled study monitored 28 TLE patients with suggested AB-negative LE along with methylprednisolone immunotherapy. Results All patients had seizures, amygdala and/or -hippocampal enlargement, subjective cognitive decline and/or behavioral problems. Eighty-six percent (24/28) were impaired in executive or memory functions, 39% (10/25) depressed, 81% were on antiepileptic drugs when pulse therapy started. After a median follow-up of 18 months, 46% (13/28) of the patients were seizure free (>2 months), 48% (13/27) showed MRI improvements (amygdala and/or hippocampal volume reduction), cognition improved in 57% (16/28), worsened in 32% (9/28), mood improved in 14% (4/25), and deteriorated in 11% (3/25). Immunotherapy was discontinued in 75% (21/28). Clinical changes did not correlate to each other. Outcomes could not be predicted. Conclusion Immunological treatment of suggested AB-negative LE showed reasonable seizure control, MRI and cognitive improvements. Treatment success was not predictable from clinical features, nor definitely attributable to immunological treatment. Lacking biomarkers for the reliable diagnosis of AB-negative LE, we suggest that in presence of mild manifestations, and after initiating antiepileptic drug therapy, negative dynamics in MRI, seizures, cognition, and behavior should be documented before immunosuppressive treatment is initiated. [ABSTRACT FROM AUTHOR]