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Pallidopyramidal disease: A misnomer?
Movement disorders, 2010-07, Vol.25 (9), p.1109-1115
Horstink, Martin W.I.M.
Dekker, Marieke C.
Montagna, Pasquale
Bonifati, Vincenzo
van De Warrenburg, Bart P.
2010
Volltextzugriff (PDF)
Details
Autor(en) / Beteiligte
Horstink, Martin W.I.M.
Dekker, Marieke C.
Montagna, Pasquale
Bonifati, Vincenzo
van De Warrenburg, Bart P.
Titel
Pallidopyramidal disease: A misnomer?
Ist Teil von
Movement disorders, 2010-07, Vol.25 (9), p.1109-1115
Ort / Verlag
Hoboken: Wiley Subscription Services, Inc., A Wiley Company
Erscheinungsjahr
2010
Quelle
MEDLINE
Beschreibungen/Notizen
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. © 2010 Movement Disorder Society
Sprache
Englisch
Identifikatoren
ISSN: 0885-3185
eISSN: 1531-8257
DOI: 10.1002/mds.23118
Titel-ID: cdi_proquest_miscellaneous_734011648
Format
–
Schlagworte
Biological and medical sciences
,
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
,
Female
,
Globus Pallidus - pathology
,
History, 19th Century
,
History, 20th Century
,
Humans
,
juvenile parkinsonism
,
Male
,
Medical sciences
,
Neurodegenerative Diseases - history
,
Neurodegenerative Diseases - pathology
,
Neurodegenerative Diseases - physiopathology
,
Neurology
,
pallidal atrophy
,
pallidopyramidal
,
Parkinson Disease - pathology
,
pseudobabinski
,
pyramidal syndrome
,
Pyramidal Tracts - pathology
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