1. Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome.
- Author
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Gika AD, Hughes E, Goyal S, Sparkes M, and Lin JP
- Subjects
- Adolescent, Child, Electrocardiography methods, Electroencephalography methods, Electromyography methods, Female, Humans, Video Recording methods, Young Adult, Antiparkinson Agents therapeutic use, Dystonic Disorders drug therapy, Dystonic Disorders etiology, Rett Syndrome complications, Trihexyphenidyl therapeutic use
- Abstract
In Rett syndrome (RS), acute life-threatening episodes (ALTEs) are usually attributed to epilepsy or autonomic dysfunction but they can represent a movement disorder (MD). We describe three girls with RS who experienced ALTEs from an early age. These were long considered epileptic until video-EEG in Patients 1 and 3 revealed their non-epileptic nature. A primary dystonic mechanism was suspected and Patients 1 and 2 were treated with Trihexyphenidyl with significantly reduced frequency of the ALTEs. Patient 3 died before Trihexyphenidyl was tried. Trihexyphenidyl in RS patients with similar presentations can modify the dystonia and prevent ALTEs., ((c) 2009 Movement Disorder Society.)
- Published
- 2010
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