Non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis in a pre-pubertal girl.
Limbic encephalitis (LE) is increasingly recognized as a precipitating factor of adult onset temporal lobe epilepsy frequently associated with bilateral hippocampal damage. So far, clinical data in children are rare and only comprise paraneoplastic forms of LE. We describe a 13-year-old pre-pubertal girl in whom non-paraneoplastic LE was diagnosed according to diagnostic criteria proposed by Bien and Elger (2007). The girl presented with a subacute syndrome comprising memory impairment, affective disturbances, and refractory temporal lobe seizures. Serial MRI scans demonstrated an initial temporo-medial swelling with T2/FLAIR signal increase progressing to bilateral hippocampal atrophy within seven months. Two years after onset of symptoms, antibodies to potassium channels were found to be slightly elevated. Immunosuppressive therapy with steroid-pulses was followed by a transient reduction of seizure frequency, even though this was started more than two years after onset of first symptoms. However, extended immunotherapy was refused by the patient's parents, so no full assessment of the treatment response was possible. In conclusion, this case shows that non-paraneoplastic LE leading to mesial temporal lobe epilepsy is not restricted to adult patients. The proposed diagnostic criteria therefore should be adapted for paediatric patients. Patients may profit from immunosuppressive therapy even when it is started at a late stage with already overt hippocampal sclerosis.
Kröll-Seger
Judith
J
Swiss Epilepsy Center, Bleulerstrasse 60, 8008 Zurich, Switzerland. judith.kroell@swissepi.ch
Bien
Christian G
CG
Huppertz
Hans-Jürgen
HJ
D000293:Adolescent; D000342:Affective Symptoms / Q000276:immunology; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood*; D004569:Electroencephalography; D004833:Epilepsy, Temporal Lobe / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005260:Female; D006624:Hippocampus / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020363:Limbic Encephalitis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008569:Memory Disorders / Q000276:immunology; D015221:Potassium Channels / Q000276:immunology*; D012598:Sclerosis / Q000276:immunology; D013702:Temporal Lobe / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D015221:Potassium Channels