LANDAU-KLEFFNER SYNDROME - CONSISTENT RESPONSE TO REPEATED INTRAVENOUS GAMMA-GLOBULIN DOSES - A CASE-REPORT

Purpose: Although several treatments have been tried for Landau-Kleffn er syndrome (LKS) too many patients are refractory to known therapies. We report an 8-year-old girl who failed other therapies but who had a consistent response after treatment with intravenous (i.v.) gamma-glo bulin. Methods: We monitored the girl from the age of 6 years, when sh e presented with a 6-month history of loss of language with normal hea ring, normal brain magnetic resonance imaging (MRI), increased cerebro spinal fluid (CSF) IgG index, and an EEG showing almost continuous, pr e dominantly left-sided spike- and slow-wave complexes. She had no cli nical seizures and did not respond to consecutive trials of valproate (VPA), clonazepam (CZP), prednisone, and carbamazepine (CBZ). She rece ived three courses of intravenous (i.v.) gamma-globulin; after each co urse, clinical and electrographic improvement lasted a few months. Aft er each of the initial two courses, clinical improvement lasted 3-4 mo nths but was followed by recurrence of the spikes on the EEG and by sp eech deterioration. Results: However, her last remission has been cont inuous for the past 16 months. Her CSF IgG index became normal after t he first i.v, gamma-globulin infusion. Conclusions: Based on our exper ience with this patient and on other investigators' experience, we bel ieve that further research into immunologic mechanisms and therapies o f this syndrome are warranted.