Pallidopyramidal disease: a misnomer?

Movement Disorders : Official Journal of the Movement Disorder Society
M W HorstinkBart P van De Warrenburg

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.

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Citations

Jul 3, 2013·Current Opinion in Neurology·Eleanna KaraHenry Houlden
Jul 31, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Victor S C FungMarie Vidailhet
Aug 4, 2016·Arquivos de neuro-psiquiatria·Rubens Paulo Araújo SalomãoOrlando G P Barsottini
Jan 24, 2020·Movement Disorders Clinical Practice·Christine Tranchant
Jan 24, 2020·Movement Disorders Clinical Practice·Hugo Morales-Briceño, Victor S C Fung
Aug 8, 2021·Journal of Neural Transmission·Kurt A Jellinger

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