Clinicopathological investigation of vascular parkinsonism, including clinical criteria for diagnosis

Authors

  • Jan C.M. Zijlmans MD, PhD,

    Corresponding author
    1. Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom
    2. Department of Neurology, VU University Medical Center, Amsterdam, The Netherlands
    • Department of Neurology, VU University Medical Centre, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands
    Search for more papers by this author
  • Susan E. Daniel MD, FRCPath,

    1. Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom
    Search for more papers by this author
  • Andrew J. Hughes MD, FRACP,

    1. Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom
    2. Austin and Repatriation Medical Centre, Heidelberg West, Victoria, Australia
    Search for more papers by this author
  • Tamas Révész MD, FRCPath,

    1. Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom
    Search for more papers by this author
  • Andrew J. Lees MD, FRCP

    1. Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom
    Search for more papers by this author

Abstract

Vascular parkinsonism (VP) is difficult to diagnose with any degree of clinical certainty. We investigated the importance of macroscopic cerebral infarcts and pathological findings associated with microscopic “small vessel disease” (SVD) in the aetiology of VP. The severity of microscopic SVD pathology (perivascular pallor, gliosis, hyaline thickening, and enlargement of perivascular spaces) and the presence of macroscopically visible infarcts were assessed in 17 patients with parkinsonism and no pathological evidence of either Parkinson's disease or any histopathological condition known to be associated with a parkinsonian syndrome, and compared with age-matched controls. Microscopic SVD pathology was significantly more severe in the parkinsonian brains. Most patients presented with bilateral bradykinesia and rigidity together with a gait disorder characterised predominantly by a shuffling gait. Four patients presented acutely with hemiparesis and then progressed to develop a parkinsonian syndrome. They could be distinguished from the remaining VP patients by the presence at autopsy of macroscopically visible lacunar infarcts in regions where contralateral thalamocortical drive might be reduced. The clinical features at presentation varied according to the speed of onset and the underlying vascular pathological state. New clinical criteria for a diagnosis of VP are proposed based on the clinicopathological findings of this study. © 2004 Movement Disorder Society

Ancillary