rss
J Neurol Neurosurg Psychiatry 72:300-303 doi:10.1136/jnnp.72.3.300
  • Original Article

Dystonia in multiple system atrophy

  1. S M Boesch,
  2. G K Wenning,
  3. G Ransmayr,
  4. W Poewe
  1. Department of Neurology, University Hospital, Innsbruck, Austria
  1. Correspondence to:
 Professor W Poewe, Department of Neurology, University Hospital, Anichstrasse 35, A-6020 Innsbruck, Austria;
 Werner.Poewe{at}uibk.ac.at
  • Received 28 November 2000
  • Accepted 26 June 2001
  • Revised 25 May 2001

Abstract

Objective: To delineate the frequency and nature of dystonia in multiple system atrophy (MSA).

Methods: a cohort of 24 patients with clinically probable MSA over the past 10 years were prospectively followed up. Motor features were either dominated by parkinsonism (MSA-P subtype, n=18) or cerebellar ataxia (MSA-C, n=6). Classification of dystonic features and their changes with time was based on clinical observation during 6–12 monthly follow up visits. Parkinsonian features and complications of drug therapy were assessed. Most patients (22/24) died during the observation period. Neuropathological examination was confirmatory in all of the five necropsied patients.

Results: At first neurological visit dystonia was present in 11 (46%) patients all of whom had been levodopa naive at this time point. Six patients (25%) exhibited cervical dystonia (antecollis) (MSA-P n=4, MSA-C n=2), five patients (21%) showed unilateral limb dystonia (MSA-P n=4; MSA-C n=1). A definite initial response to levodopa treatment was seen in 15/18 patients with MSA-P, but in none of the six patients with MSA-C. A subgroup of 12 patients with MSA-P developed levodopa induced dyskinesias 2.3 years (range 0.5–4) after initiation of levodopa therapy. Most patients had peak dose craniocervical dystonia; however, some patients experienced limb or generalised dystonia. Isolated peak dose limb chorea occurred in only one patient.

Conclusion: The prospective clinical study suggests that dystonia is common in untreated MSA-P. This finding may reflect younger age at disease onset and putaminal pathology in MSA-P. Levodopa induced dyskinesias were almost exclusively dystonic affecting predominantly craniocervical musculature. Future studies are required to elucidate the underlying pathophysiology of dystonia in MSA.

Footnotes

    Podcasts
    Visit the full archive of podcasts for JNNP here >>

    Free sample
    This recent issue is free to all users to allow everyone the opportunity to see the full scope and typical content of JNNP.
    View free sample issue >>

    Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.

    Navigate This Article