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Pallidopyramidal disease: A misnomer?

Authors

  • Martin W.I.M. Horstink MD, PhD,

    1. Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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    • Deceased

  • Marieke C. Dekker MD, PhD,

    1. Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • Pasquale Montagna MD,

    1. Department of Neurological Sciences, University of Bologna, Bologna, Italy
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  • Vincenzo Bonifati MD, PhD,

    1. Department of Clinical Genetics, Erasmus Medical Centre, Rotterdam, The Netherlands
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  • Bart P. van De Warrenburg MD, PhD

    Corresponding author
    1. Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    • Department of Neurology Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, PO Box 9101, Nijmegen 6500 HB, The Netherlands
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  • Potential conflict of interest: None reported.

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. © 2010 Movement Disorder Society

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