Hemiparkinsonism-hemiatrophy syndrome: Clinical and neuroradiologic features

N. Giladi, R. E. Burke, V. Kostic, S. Przedborski, M. Gordon, A. Hunt, S. Fahn

Research output: Contribution to journalArticlepeer-review

Abstract

We evaluated 11 patients with hemiparkinson-hemiatrophy syndrome, 6 with body and contralateral cerebral hemispheric hemiatrophy, 4 with only body hemiatrophy, and 1 with just brain hemiatrophy. The mean age of symptom onset was 38.1 years (range, 18 to 54) with 5.2 ± 3.1 (mean ± SD) years of illness until the last follow-up visit. The presenting symptom was unilateral tremor in 6 patients, hand dystonia in 2, bradykinesia in 2, and abnormal gait in 1 patient. Three patients had a good response to levodopa, 4 had moderate response, and 2 patients had a poor response. During a mean follow-up period of 1.7 years (range, 4 months to 5 years), the Hoehn and Yahr score changed in only 3 patients: 2 gained 1.5 points and 1 gained 3 points over 2.5 years. We discuss the association between hemiparkinsonism-body hemiatrophy and contralateral hemispheric hemiatrophy, and raise the possibility of early childhood brain insult with delayed-onset parkinsonism.

Original languageEnglish
Pages (from-to)1731-1734
Number of pages4
JournalNeurology
Volume40
Issue number11
DOIs
StatePublished - Nov 1990
Externally publishedYes

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