Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome
✍ Scribed by Artemis D. Gika; Elaine Hughes; Sushma Goyal; Matthew Sparkes; Jean-Pierre Lin
- Publisher
- John Wiley and Sons
- Year
- 2010
- Tongue
- English
- Weight
- 524 KB
- Volume
- 25
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
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. © 2009 Movement Disorder Society