Movement Disorders (revue)

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Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome.

Identifieur interne : 001A31 ( PubMed/Curation ); précédent : 001A30; suivant : 001A32

Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome.

Auteurs : Artemis D. Gika [Royaume-Uni] ; Elaine Hughes ; Sushma Goyal ; Matthew Sparkes ; Jean-Pierre Lin

Source :

RBID : pubmed:20063434

English descriptors

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.

DOI: 10.1002/mds.22926
PubMed: 20063434

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pubmed:20063434

Le document en format XML

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<nlm:affiliation>Department of Paediatric Neurology, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. adgika@doctors.org.uk</nlm:affiliation>
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<div type="abstract" xml:lang="en">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.</div>
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