TY - JOUR
AU - Widman, Guido
AU - Golombeck, Kristin
AU - Hautzel, Hubertus
AU - Gross, Catharina C.
AU - Quesada, Carlos M.
AU - Witt, Juri-Alexander
AU - Rota Kops, Elena
AU - Ermert, Johannes
AU - Greschus, Susanne
AU - Surges, Rainer
AU - Helmstaedter, Christoph
AU - Wiendl, Heinz
AU - Melzer, Nico
AU - Elger, Christian E.
TI - Treating a GAD65 Antibody-Associated Limbic Encephalitis with Basiliximab: A Case Study
JO - Frontiers in neurology
VL - 6
SN - 1664-2295
CY - Lausanne
PB - Frontiers Research Foundation
M1 - FZJ-2016-07119
SP - 167
PY - 2015
AB - Background: Antibodies (ABs) against the 65-kDa isoform of the intracellular enzymeglutamate decarboxylase (GAD65) have been found in limbic encephalitis (LE) andother neurological conditions. The direct significance of anti-GAD65-ABs for epilepsyis unclear. However, in histological preparations from biopsies of resective epilepsysurgeries, predominantly cytotoxic T-lymphocytes were detected making close contactsto neurons. Activated T-lymphocytes can, in turn, be selectively controlled by therapeuticinterleukin-2 receptor Abs, such as basiliximab.Case presentation: We report of a 25-year-old male patient with epilepsy since theage of 18 and displaying clinical signs of LE and a high titer of GAD65 ABs in cerebro-spinal fluid (CSF) and serum. Monthly, repetitive, intravenous cortisone pulse therapiesthat were initially administered for 6 months failed to improve his condition. Subsequentflow-cytometry analysis of CSF showed especially an increased fraction of activatedHLA-DR+CD8+T-lymphocytes (fCD8+TL) when compared to controls. Thus, a second,intravenous cortisone pulse therapy with an additional basiliximab dose of 20 mg/monthwas started. After 3 months, the fCD8+TL in the CSF normalized; after 6 months, thepsychological impulse-control deficits normalized; and after 11 months the patientwas seizure free. However, 7 weeks later, seizures and, later on, psychological deficitsrecurred and fCD8+TL was once again present in the CSF. Flumazenil PET, magneticresonance imaging-volumetry, and neuropsychological changes during therapy aredescribed.Conclusion: The correlation of the fCD8+TL in the CSF with clinical and paraclinical measures of disease activity combined with the unambiguous response to basiliximabstrongly argues in favor of the putative pathogenic role fCD8+TL in anti-GAD65 LE. The clinical relapse at the end of the observation period might be due to the formation ofhuman anti-drug ABs, a well-known complication of therapy with chimeric ABs.
LB - PUB:(DE-HGF)16
UR - <Go to ISI:>//WOS:000363857200001
C6 - pmid:26284025
DO - DOI:10.3389/fneur.2015.00167
UR - https://juser.fz-juelich.de/record/824547
ER -