Get access

Neuropathological findings in benign tremulous Parkinsonism

Authors

  • Marianna Selikhova MD,

    1. Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, United Kingdom
    2. Department of Neurology, Russian State Medical University, Moscow, Russia
    Search for more papers by this author
  • Peter A. Kempster MD,

    1. Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, United Kingdom
    2. Reta Lila Weston Institute of Neurological Studies, University College London, London, United Kingdom
    3. Neurosciences Department, Monash Medical Centre and Department of Medicine, Monash University, Melbourne, Australia
    Search for more papers by this author
  • Tamas Revesz MD,

    1. Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, United Kingdom
    Search for more papers by this author
  • Janice L. Holton PhD,

    1. Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, United Kingdom
    2. Reta Lila Weston Institute of Neurological Studies, University College London, London, United Kingdom
    Search for more papers by this author
  • Andrew J. Lees MD

    Corresponding author
    1. Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, United Kingdom
    2. Reta Lila Weston Institute of Neurological Studies, University College London, London, United Kingdom
    • Correspondence to: Professor Andrew J. Lees, Reta Lila Weston Institute of Neurological Studies, 1 Wakefield Street, London, WC1N 1PJ, UK; andrew.lees@ucl.ac.uk

    Search for more papers by this author

  • Funding agencies: J.L.H. and T.R. are supported by Parkinson's UK, the Multiple System Atrophy Trust, Alzheimer's Research UK, and the Progressive Supranuclear Palsy (Europe) Association. J.L.H. is supported by the Reta Lila Weston Institute for Neurological Studies. Part of this work was undertaken at UCLH/UCL, which received a proportion of funding from the Department of Health's NIHR Biomedical Research Centres funding scheme.

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

Abstract

Benign tremulous parkinsonism, a tremor dominant syndrome with a relatively slow rate of deterioration, is recognized by clinicians although its pathological basis is not well understood. A systematic review of Queen Square Brain Bank donors was carried out to determine the natural history and pathology of individuals who had tremor dominant parkinsonism with mild non-tremor components and minimal gait disability for at least 8 years. We identified 16 cases of pathologically proved benign tremulous Parkinson's disease (PD); another 5 individuals conformed to the definition but did not have the pathology of PD. Patients with verified benign tremulous PD had less severe neuronal loss in the substantia nigra than controls (χ2: P = .003). Twelve of these had been correctly diagnosed with PD at their first neurological evaluation, whereas the other 4 were originally thought to have another tremor disorder. The only consistent distinguishing feature of the 5 pathologically disproved cases, who may have had either essential tremor with associated rest tremor or dystonic tremor, was a failure to develop unequivocal bradykinesia within a decade of onset of tremor at rest. Our findings support the existence of a distinct subgroup of benign tremulous PD. The slower rate of clinical progression correlates with less severe nigral cell loss at postmortem, although many of these patients transgress the benign tremulous parkinsonism definition by the final third of their disease course and develop the common features of advanced PD. © 2012 Movement Disorder Society

Get access to the full text of this article

Ancillary