title: Neuronal autoantibodies in patients with Rasmussen's encephalitis.
abstract: Rasmussen's encephalitis (RE) is a rare disease with unknown pathophysiology. To disclose whether anti-neuronal autoimmunity participates in the aetiology of RE, various neuronal autoantibodies (NAAbs) were investigated in sera of patients with RE and controls. The study included five patients who fulfilled the RE diagnostic criteria (clinical, EEG, and MRI findings) as the patient group, and 50 multiple sclerosis patients and 50 healthy subjects as the control groups. Sera were evaluated for various NAAbs by radioimmunoassay or cell-based assays. All sera were also screened for uncharacterized antibodies to neuronal cell surface or synapse antigens by indirect immunofluorescence using hippocampal cell cultures. The mean age at onset of seizures was 8.3±3.4 years (range: 4-13.5) and mean follow-up time was 11.2±5.4 years (range: 5-19). All patients had unihemispheric atrophy of the cerebral cortex and epilepsia partialis continua. Two of the patients had moderate cognitive impairment, while the others were severely affected, as shown by neuropsychological testing. NAAb positivity was not detected in any of the patients. Immune aetiology is thought to have a role in RE, but the responsible players have not yet been elucidated. Our extensive antibody screening in a small number of patients does not support the presence of antigen-specific anti-neuronal autoimmunity in RE pathophysiology.
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University.
authors.forename: Bedia
authors.initials: B
authors.lastname: Samanci
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University.
authors.forename: Pınar
authors.initials: P
authors.lastname: Tektürk
authors.affiliation: Department of Neuroscience, Institute of Experimental Medicine, Istanbul University.
authors.forename: Erdem
authors.initials: E
authors.lastname: Tüzün
authors.affiliation: Department of Neuroscience, Institute of Experimental Medicine, Istanbul University.
authors.forename: Ece
authors.initials: E
authors.lastname: Erdağ
authors.affiliation: Department of Neurology, Okmeydani Training and Research Hospital, Istanbul, Turkey.
authors.forename: Demet
authors.initials: D
authors.lastname: Kınay
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University.
authors.forename: Zuhal
authors.initials: Z
authors.lastname: Yapıcı
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University.
authors.forename: Betül
authors.initials: B
authors.lastname: Baykan
mesh_terms: D000293:Adolescent; D001284:Atrophy / Q000000981:diagnostic imaging / Q000276:immunology; D001323:Autoantibodies; D002540:Cerebral Cortex / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D017036:Epilepsia Partialis Continua / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009474:Neurons / Q000276:immunology* / Q000473:pathology; D009483:Neuropsychological Tests; D055815:Young Adult
chemical_list: D001323:Autoantibodies
keywords: Rasmussen syndrome; Rasmussen's encephalitis; anti-neuronal antibodies; autoimmunity; neuronal autoantibodies