Universität zu Köln

von Rhein, B., Wagner, J., Widman, G., Malter, M. P., Elger, C. E. and Helmstaedter, C. (2017). Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy. Acta Neurol. Scand., 135 (1). S. 134 - 142. HOBOKEN: WILEY. ISSN 1600-0404

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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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code von Rhein, B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wagner, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Widman, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Malter, M. P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Elger, C. E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Helmstaedter, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-248226
DOI:	10.1111/ane.12575
Journal or Publication Title:	Acta Neurol. Scand.
Volume:	135
Number:	1
Page Range:	S. 134 - 142
Date:	2017
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1600-0404
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language TEMPORAL-LOBE EPILEPSY; AMYGDALA ENLARGEMENT; GABA(A) RECEPTOR; VGKC-COMPLEX; NMDAR; CLASSIFICATION; SEIZURES; ANTIGEN; ONSET Multiple languages Clinical Neurology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/24822