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Neuropediatrics 1982; 13(3): 152-154
DOI: 10.1055/s-2008-1059614
© Georg Thieme Verlag KG Stuttgart · New York

Fluctuating Dystonia and Allied Syndromes

N.  Gordon
  • Booth Hall Children's Hospital, Charlestown Road, Blackley, Manchester, England
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Publication History

Publication Date:
05 May 2008 (online)

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Abstract

Dystonia musculorum deformans is a descriptive diagnosis. A number of other conditions such as Hallervorden-Spatz disease and juvenile paralysis agitans have to be excluded. Then the history of a child's illness may suggest a particular syndrome such as the progressive dystonia with marked diurnal fluctuations. Two case reports are given of children who show fluctuation of symptoms but their histories varied from those previously described. Problems of treatment are discussed and it is suggested that fluctuation of symptoms may indicate a response to levadopa while those with a relentless progression of symptoms may respond to other drugs such as orphenadrine.

Key words

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

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