Status dystonicus and Hallervorden-Spatz disease: Treatment with intrathecal baclofen and pallidotomy

:  Severe dystonia or status dystonicus is a life threatening disorder that develops in patients with both primary and secondary dystonia. We present the case of a 9‐year‐old boy with Hallervorden‐Spatz disease (HVS) who developed status dystonicus, failing to respond to high dose oral therapy with...

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Bibliographic Details
Published in:Journal of paediatrics and child health 2004-05, Vol.40 (5-6), p.322-325
Main Authors: Kyriagis, M, Grattan-Smith, P, Scheinberg, A, Teo, C, Nakaji, N, Waugh, M
Format: Article
Language:English
Subjects:
Australia
Baclofen - therapeutic use
Boys
Child
Combined Modality Therapy
Dystonia
Dystonic Disorders - drug therapy
Dystonic Disorders - etiology
Dystonic Disorders - surgery
Globus Pallidus - surgery
Hallervorden-Spatz disease
Hallervorden-Spatz syndrome
Humans
Injections, Spinal
Intrathecal baclofen
intrathecal baclofen and pallidotomy
Magnetic Resonance Imaging
Male
Muscle Relaxants, Central - therapeutic use
Pallidotomy
Pantothenate Kinase-Associated Neurodegeneration - complications
Pantothenate Kinase-Associated Neurodegeneration - drug therapy
Pantothenate Kinase-Associated Neurodegeneration - surgery
Treatment
Treatment Outcome
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Summary::  Severe dystonia or status dystonicus is a life threatening disorder that develops in patients with both primary and secondary dystonia. We present the case of a 9‐year‐old boy with Hallervorden‐Spatz disease (HVS) who developed status dystonicus, failing to respond to high dose oral therapy with multiple antidystonic agents. High dose intravenous sedating agents combined with endotracheal intubation and mechanical ventilation were required to control the spasms. Alleviation of the spasms was achieved by a combination of temporary intrathecal baclofen infusions and bilateral pallidotomy. Although it could be argued this is a situation where only palliative measures should be used, we believe a relatively aggressive approach was justified. It relieved the intense pain associated with the spasms and allowed the child to be discharged home without the prolonged stay in intensive care, morbidity and mortality, which characterize status dystonicus.
ISSN:1034-4810
1440-1754
DOI:10.1111/j.1440-1754.2004.00374.x