Adrenocorticotropic hormone therapy in acquired childhood epileptic aphasia
Although Landau-Kleffner syndrome, a childhood-acquired epileptic aphasia, is frequently studied either the underlying pathophysiology or the optimal therapy remained unknown. In our study we aimed to investigate the efficacy of ACTH therapy in Landau-Kleffner syndrome. We have analysed retrospectively the documentation of five children treated by ACTH, who suffered from Landau-Kleffner syndrome. We studied the longitudinal changes of the four most characteristic symptoms and signs of the syndrome: epileptiform EEG, speech and behaviour disorders, seizures together with the ACTH regimes. Besides, we analysed the relation between the starting date of the therapy and its efficacy. Before giving ACTH, epileptiform EEG and speech disorders were observed in all the five children, seizures in four of them, behaviour disorders in three of them. In two patients the speech disorder had been persisting for years before. Due to the starting ACTH stoss-therapy (20 E/day for one-two weeks) all the four examined signs disappeared or showed quick softening in all the five children in maximum two weeks. We adjusted long-term low dose maintenance therapy to avoid relapses in the long-term follow-up. Epileptiform EEGs have normalised in one case and have decreased in four cases. Speech disorders have disappeared in two and have softened in three children. Behaviour disorders have cured in 3/4 cases, softened in one case. Seizures have disappeared in all cases. One child is totally asymptomatic, four of them lives with softened symptoms. Analysing our data we found that the earlier the therapy starts, the more effective it is. On the basis of our data ACTH is an effective treatment for Landau-Kleffner syndrome. After giving it for only a short period, relapses often occur, to avoid relapses adjustment of long term low dose maintenance therapy is advisable.