Autoimmune limbic encephalitis in a girl with chronic immune thrombocytopenia

Abstract

Autoimmune encephalitis occurs in children and adolescents, often in association with other autoimmune diseases, and may be the cause of temporal lobe epilepsy. Immunomodulatory therapy may lead to a favorable outcome. So far, clinical data in children are rare and refer exclusively to paraneoplastic forms. The semiology of seizures involves temporal lobe and patients have behavioral and psychiatric problems. It is not rare for patients to be initially misdiagnosed. In a 16-year-old girl, non-paraneoplastic limbic encephalitis was diagnosed according to Bien and Elger. She had complex partial seizures and developed behavioral disturbances including anxiety, fear, feeling of unreality, auditory hallucinations and insomnia. Immune thrombocytopenia manifesting in chronic form was diagnosed at the age of six. EEG showed frontal fast beta activity. MRI scans demonstrated temporal swelling with T2/FLAIR signal increase. Cerebrospinal fluid showed pleocytosis and positive oligoclonal bands. Serologic tests for neurotropic viruses and PCR analysis for herpes simplex virus were negative. Immunomodulation therapy led to improvement of her clinical status.