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A case of Lewy body disease and anaplastic astrocytoma presenting with atypical parkinsonism

Leahy, Christopher B; Robinson, Andrew C; Jabbari, Edwin; Morris, Huw R; Lally, Imogen; Djoukhadar, Ibrahim; Roncaroli, Federico; (2022) A case of Lewy body disease and anaplastic astrocytoma presenting with atypical parkinsonism. Neuropathology 10.1111/neup.12848. Green open access

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Abstract

We report on a patient with atypical parkinsonism due to coexistent Lewy body disease (LBD) and diffuse anaplastic astrocytoma. The patient presented with a mixed cerebellar and parkinsonian syndrome, incomplete levodopa response, and autonomic failure. The clinical diagnosis was multiple system atrophy (MSA). Supportive features of MSA according to the consensus diagnostic criteria included postural instability and early falls, early dysphagia, pyramidal signs, and orofacial dystonia. Multiple exclusion criteria for a diagnosis of idiopathic Parkinson's disease (iPD) were present. Neuropathological examination of the left hemisphere and the whole midbrain and brainstem revealed LBD, neocortical-type consistent with iPD, hippocampal sclerosis, and widespread neoplastic infiltration by an anaplastic astrocytoma without evidence of a space occupying lesion. There were no pathological features of MSA. The classification of atypical parkinsonism was difficult in this patient. The clinical features and disease course were confounded by the coexistent tumor, leading to atypical presentation and a diagnosis of MSA. We suggest that the initial features were due to Lewy body pathology, while progression and ataxia, pyramidal signs, and falls were accelerated by the occurrence of the astrocytoma. Our case reflects the challenges of an accurate diagnosis of atypical parkinsonism, the potential for confounding co-pathology and the need for autopsy examination to reach a definitive diagnosis.

Type: Article
Title: A case of Lewy body disease and anaplastic astrocytoma presenting with atypical parkinsonism
Location: Australia
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/neup.12848
Publisher version: https://doi.org/10.1017/S1368980022001276
Language: English
Additional information: Copyright © 2022 The Authors. Neuropathology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Neuropathology. This is an open access article under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Keywords: anaplastic astrocytoma, atypical parkinsonism, Lewy body disease, multiple system atrophy, neuropathology
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
URI: https://discovery.ucl.ac.uk/id/eprint/10152844
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