Encephalopathy Associated With Neurochondrin Autoantibodies
Authors: Winfried St√∂cker
Bernd Axel Neubauer
Andreas Hahn
Borros Arneth
Harald Renz
Anita Windhorst
Frank Risto Rommel
Myriam Schumacher
Journal: Journal of child neurology (2019)
Discipline: NEUROSCIENCE & BEHAVIOR
Keywords: autoantibodies; autoimmune; encephalitis; neurochondrin; pediatric.
Affiliations: 1 Department of Child Neurology, Justus-Liebig University Gie√üen, Gie√üen, Germany., 2 Institute of Laboratory Medicine and Pathobiochemistry, Molecular Diagnostics, Justus-Liebig University Gie√üen, Gie√üen, Germany., 3 Institute of Experimental Immunology, Euroimmun AG, L√ºbeck, Germany., 4 Institute for Medical Informatics, Justus-Liebig-University, Giessen, Germany.

Abstract:  We determined the prevalence of autoantibodies against an extended number of established and novel neural antigens in children and adolescents with suspected autoimmune encephalitis, epilepsy, single seizures, or marked epileptiform activity in electroencephalography (EEG). Prospectively, 103 patients were recruited aged between 0 and 18 years and 104 controls. A panel of 35 autoantibodies against neural cell-surface and intracellular antigens was screened. Sixteen of 103 patients (15.5%) showed a positive result for 1 or more autoantibodies, compared to 6 of 104 controls (5.8%, P = .02). Neurochondrin was identified as a possible new target of autoantibodies in 3 patients within this cohort, but none in controls. The patients showed severe behavioral disturbances, memory and cognitive impairment, episodes of reduced responsiveness, but no seizures, and normal MRI. Clinical findings, course, and treatment response of these 3 patients are presented. 

Retracted: No

PubPeer comments:
 Brought to my attention by a reader. In this paper, three patients are presented with  autoimmune encephalitis , and the patients serum was tested for the presence of autoantibodies against neurochondrin using indirect fluorescence with EuroImmun reagents (Figure 3). One author on this paper is was an EuroImmun employee. However, Figure 3 of this paper shows unexpected similarities to Figure 4 of a 2017 paper describing a different set of patients, but also EuroImmun assays. This paper is: Neurochondrin is a neuronal target antigen in autoimmune cerebellar degeneration, Ramona Miske et al., Neurol Neuroimmunol Neuroinflamm 2017;4:e307; doi: 10.1212 NXI.0000000000000307, PubPeer: https: pubpeer.com publications 1BFC4C2E24E53051CECD169EB91BEB The 2017 paper (Miske et al.) describes three patients recruited at the Department of Neurology, University of Muu0308nster, Germany, with pronounced cerebellar and brainstem syndrome of unknown origin. * Patient 1, male, 21 months history beginning at age 51y * Patient 2, male, 54 months history beginning at age 23y * Patient 3, female, 120 months history beginning at age 18y This 2019 paper (Schumacher et al.) describes three patients recruited at the Department for Child Neurology at University hospital Gieu00dfen, with autoimmune encephalitis. * Patient 1, male, age 6y * Patient 2, female, age 7y * Patient 3, male, age 14y Figure 4 of the 2017 Miske et al. paper looks unexpectedly similar to Figure 3 of the 2019 Schumacher et al. paper (this paper). Shown with red boxes. Could the authors please check? It is unexpected that different patients, recruited at different hospitals and with different clinical symptoms have such similar assay results.  , 
 Correction, April 2020, found here: https: journals.sagepub.com doi 10.1177 0883073820922333  In the above-referenced article, Figure 3 should be corrected as shown in the revised figure below. The conclusion and text of the paper is not affected by this error. ,