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Publication year
2010Source
Movement Disorders, 25, 9, (2010), pp. 1109-15ISSN
Publication type
Article / Letter to editor
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Organization
Neurology
Journal title
Movement Disorders
Volume
vol. 25
Issue
iss. 9
Page start
p. 1109
Page end
p. 15
Subject
DCN 2: Functional NeurogenomicsAbstract
The combination of recessive early-onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa-responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early-onset nigral parkinsonism or dopa-responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski ("striatal toe"). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico-pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description "pallidopyramidal" seems to be a misnomer.
This item appears in the following Collection(s)
- Academic publications [238586]
- Electronic publications [122847]
- Faculty of Medical Sciences [90409]
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