title: Pallidopyramidal disease: a misnomer?
abstract: 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.
authors.affiliation: Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Martin W I M
authors.initials: MW
authors.lastname: Horstink
authors.forename: Marieke C
authors.initials: MC
authors.lastname: Dekker
authors.forename: Pasquale
authors.initials: P
authors.lastname: Montagna
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Bonifati
authors.forename: Bart P
authors.initials: BP
authors.lastname: van De Warrenburg
mesh_terms: D005260:Female; D005917:Globus Pallidus / Q000473:pathology*; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans; D008297:Male; D019636:Neurodegenerative Diseases / Q000266:history / Q000473:pathology* / Q000503:physiopathology; D010300:Parkinson Disease / Q000473:pathology; D011712:Pyramidal Tracts / Q000473:pathology*