Limbiczne zapalenie mózgu z obecnością przeciwciał anty-LGI1 u 3-letniego chłopca. Przedstawienie przypadku Limbic encephalitis with anti-LGI1 antibodies in 3-year old boy. A case report
2014
Introduction. Nowadays, autoimmune encephalitis is more frequently recognized as a cause of epilepsy, especially drugresistant. In its pathogenesis autoantibodies against antigens such as ion channels, receptors and other synaptic proteins which are vital for neuronal transmission (e.g. NMDA, AMPA, GABA, potassium channel complex, LG1, GAD) play an important role. Sometimes cytotoxic processes occur as well. Aim of this study is to present a case of a boy, now 5-year old, with autoimmune epilepsy in the course of limbic encephalitis, with normal mental development prior to the onset of the disease. Case report. The onset of refractory epilepsy with polymorphic seizures and psychomotor regression following respiratory tract infection occurred at the age of 3 years. EEG showed „hypsarrhytmia-like” trace, in FLAIR MRI hyperintensive changes in temporal lobe were seen and antibodies against LGI 1 in serum were found. AED were ineffective and immunomodulatory treatment was only partially effective. Full remission of seizures and normalization of EEG as well as progress in mental development was achieved after introducing the immunosuppressive drug (Tacrolimus). Conclusion. In some cases of refractory epilepsies autoimmune etiology should be considered. Investigation for a presence of anti-neuronal antibodies in serum or other markers of such a process should be performed. Recognition of underlying cause of epilepsy allows early introduction of appropriate immunomodulatory treatment, which may lead to clinical improvement or even to full remission of disease.
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