A VERY YOUNG GİRL LİMBİC ENCEPHALİTİS WİTH LGI1 ANTİBODİES
Gülcan Akyüz, Gülten Öztürk, Olcay Ünver, Dilşad Türkdoğan
Anti LGI1 encephalitis is an autoimmune disease characterized by antibodies aganist leucine-rich glioma inactivated 1 (LGI1) proteins and most commonly seen in male patient at an age range of 30-80 years.Memory disturbances, confusion and seizures are accompanied by distonic faciobrahcial seizures presenting with myoclonus or dystonia often not responsive to anti epileptics. Patients might also have sleep disturbance or hyponatremia. Case presentation We present an 8 years 9 months old girl who presented with tonic seizures, memory disturbances, involuntary distonic movements, ataxia, sleep disorders. The electroencephalogram (EEG) showed bilateral posterior delta slow wave activity and right fronto-central spike and wave activities and focal status. Her paraneoplastic and limbic encephalitis panels were normal. Her blood autoimmune encephalitis panel had LGI1 antibodies. She was admitted to hospital after a seizure and memory problems and was given levatiracetam .Pulse steroid, IVIG therapy was given her . Her memory disturbance and ataxia were recovered after the applied immunotherapies. The EEG returned to normal shortly. The patient received azathioprine as a steroid sparing immunomodulatory threapy. She is symptom free since then. Discussion Anti LGI1 is the second most common cause of autoimmune encephalitis after anti NMDA encephalitis. To the best of our knowledge this is the youngest patient reported in the literature described so far. LGI1 encephalitis is well responsive to immünosupresive therapy and should be considered in strongly suspected autoimmune encephalitis in case of especially distonic fasiobarcial seizures, memory problems and free other autoimmun antibodies.
Keywords: limbic encephalitis, anti LGI1, memory dysfunction,ımmunotherapy
Gülcan Akyüz
University of Heath Sciences, Sancaktepe Training and Research Hospital
Turkey
Gülten Öztürk
University of Marmara, Pendik Training and Research Hospital
Turkey
Olcay Ünver
University of Marmara, Pendik Training and Research Hospital
Turkey
Dilşad Türkdoğan
University of Marmara, Pendik Training and Research Hospital
Turkey
Anti LGI1 encephalitis is an autoimmune disease characterized by antibodies aganist leucine-rich glioma inactivated 1 (LGI1) proteins and most commonly seen in male patient at an age range of 30-80 years.Memory disturbances, confusion and seizures are accompanied by distonic faciobrahcial seizures presenting with myoclonus or dystonia often not responsive to anti epileptics. Patients might also have sleep disturbance or hyponatremia. Case presentation We present an 8 years 9 months old girl who presented with tonic seizures, memory disturbances, involuntary distonic movements, ataxia, sleep disorders. The electroencephalogram (EEG) showed bilateral posterior delta slow wave activity and right fronto-central spike and wave activities and focal status. Her paraneoplastic and limbic encephalitis panels were normal. Her blood autoimmune encephalitis panel had LGI1 antibodies. She was admitted to hospital after a seizure and memory problems and was given levatiracetam .Pulse steroid, IVIG therapy was given her . Her memory disturbance and ataxia were recovered after the applied immunotherapies. The EEG returned to normal shortly. The patient received azathioprine as a steroid sparing immunomodulatory threapy. She is symptom free since then. Discussion Anti LGI1 is the second most common cause of autoimmune encephalitis after anti NMDA encephalitis. To the best of our knowledge this is the youngest patient reported in the literature described so far. LGI1 encephalitis is well responsive to immünosupresive therapy and should be considered in strongly suspected autoimmune encephalitis in case of especially distonic fasiobarcial seizures, memory problems and free other autoimmun antibodies.
Keywords: limbic encephalitis, anti LGI1, memory dysfunction,ımmunotherapy
Gülcan Akyüz
University of Heath Sciences, Sancaktepe Training and Research Hospital
Turkey
Gülten Öztürk
University of Marmara, Pendik Training and Research Hospital
Turkey
Olcay Ünver
University of Marmara, Pendik Training and Research Hospital
Turkey
Dilşad Türkdoğan
University of Marmara, Pendik Training and Research Hospital
Turkey
Gülcan Akyüz
University of Heath Sciences, Sancaktepe Training and Research Hospital Turkey
University of Heath Sciences, Sancaktepe Training and Research Hospital Turkey