Autoimmune encephalitis associated with voltage-gated potassium channels-complex and leucine-rich glioma-inactivated 1 antibodies - a national cohort study

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  • Marko Celicanin, Bispebjerg Movement Disorder Biobank, Bispebjerg-Frederiksberg Hospital, University Hospital of Copenhagen
  • ,
  • Mia Blaabjerg, Department of Neurology, Roskilde University Hospital, Roskilde, Denmark.
  • ,
  • Camilla Charlotte Maersk-Moller, Department of Neurology, Aalborg Hospital, Aarhus University Hospital, Aalborg, Denmark.
  • ,
  • Sandor Beniczky, Department of Clinical Neurophysiology, Danish Epilepsy Centre, Dianalund, Denmark; Institute of Regional Health Research, University of Southern Denmark, Odense, Denmark.
  • ,
  • L Marner, d Department of Clinical Physiology, Nuclear Medicine and PET, Glostrup section , Rigshospitalet , Copenhagen , Denmark ;
  • ,
  • Christine Thomsen, Department of Diagnostic Radiology, Rigshospitalet, Copenhagen, Denmark.
  • ,
  • Flemming Winther Bach Bach
  • Daniel Kondziella, Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Denmark
  • ,
  • Henning Andersen
  • Finn Somnier, Department of Autoimmunology, Statens Serum Institut, Copenhagen, Denmark.
  • ,
  • Zsolt Illes, Institute of Regional Health Research, University of Southern Denmark, Odense, Denmark; MS-Clinic of Southern Jutland (Sønderborg, Esbjerg, Kolding), Department of Neurology, Sønderborg, Denmark.
  • ,
  • Lars Hageman Pinborg, Neurobiology Research Unit, Department of Neurology, Rigshospitalet, Copenhagen, Denmark.

BACKGROUND AND PURPOSE: The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2 antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016.

METHODS: We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment. All magnetic resonance imaging, electroencephalography and 18 F-fluorodeoxyglucose positron emission tomography scans were re-evaluated by experts in the field.

RESULTS: A total of 28/192 patients tested positive for VGKC-complex antibodies by radioimmunoassay and indirect immunofluorescence; 17 had antibodies to LGI1 and 6/7 of the available cerebrospinal fluids from these patients were seropositive. These 17 patients all had a clinical phenotype appropriate to LGI1 antibodies. The remaining 11 were LGI1 negative (n = 4) or not tested (n = 7). Of these, two had a phenotype consistent with limbic encephalitis. The remaining phenotypes were Guillain-Barré syndrome, Creutzfeldt-Jakob disease, neuromyotonia and anti-N-methyl-D-aspartate receptor encephalitis. Magnetic resonance imaging abnormalities were demonstrated in 69% of the LGI1-positive patients. Two patients with normal magnetic resonance imaging demonstrated temporal lobe hypermetabolism using 18 F-fluorodeoxyglucose positron emission tomography. Abnormal electroencephalography recordings were found in 86% of the patients. Upon follow-up (median 3.2 years), the median modified Rankin Scale score of anti-LGI1-positive patients was 2 and only two patients reported seizures in the past year.

CONCLUSIONS: Patients diagnosed with anti-LGI1 autoimmune encephalitis increased significantly from 2009 to 2014, probably due to increased awareness. In contrast to seropositive anti-VGKC-complex patients, all anti-LGI1-positive patients presented with a classical limbic encephalitis. The majority of patients recovered well.

TidsskriftEuropean Journal of Neurology
Sider (fra-til)999-1005
StatusUdgivet - aug. 2017

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