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Neuropediatrics 1984; 15(4): 208-210
DOI: 10.1055/s-2008-1052368
© Georg Thieme Verlag KG Stuttgart · New York

Treatment of Non-Fluctuating Progressive Dystonia: A Neuropharmacological Approach

J.  Willemse , O.  van Nieuwenhuizen , R. H. Gooskens , H. G. M. Westenberg1
  • 1Departments of Childneurology and Psychiatry, University Hospital Utrecht, 3500 CG Utrecht, The Netherlands
  • University Hospital Utrecht, 3500 CG Utrecht, The Netherlands
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

HVA and 5-HIAA in CSF were determined in five patients with non-fluctuating progressive dystonia before and after treatment with L-Dopa. The response to L-Dopa was unequivocally favourable in one patient. The HVA-level in her CSF was significantly reduced before treatment, whereas in the other four non-responding patients the pre-treatment HVA was normal. It is postulated, that the biochemical delineation of clinical responsiveness to a neurotransmitter may provide useful clues towards the classification of extrapyramidal disease in childhood.

Key words

Dystonia - Chorea - Hallervorden Spatz - Catecholamine - Basal ganglia - Dopamine