Intranasal midazolam as a treatment of autonomic crises in patients with familial dysautonomia

To evaluate the efficacy and safety of intranasal midazolam in the treatmen t of autonomic crises in children with familial dysautonomia, intranasal mi dazolam was administered at the hospital to six patients during nine episod es of autonomic crisis. Treatment was successful in seven of nine episodes of autonomic crisis in five of six patients, with a mean interval to respon se of 9.25 +/- 1.25 minutes. The parents of five patients in whom the treat ment was successful were instructed to use midazolam at home, At home, 16 a dditional episodes were treated by the parents, with successful control ach ieved in 14 (87%), The mean interval to response was 12.8 +/- 2 minutes. No significant side effects were observed at the hospital or at home after in tranasal administration of midazolam, Midazolam, given intranasally, is eff ective and safe in the management of autonomic crises in patients with fami lial dysautonomia, either in the hospital or at home by the parents after a ppropriate instruction. (C) 2000 by Elsevier Science Inc. All rights reserv ed.