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Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome

Weeks, RA; Scaravilli, F; Lees, AJ; Carroll, C; Husain, M; Rudge, P; (2003) Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome. MOVEMENT DISORD , 18 (3) 331 - 336. 10.1002/mds.10347.

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Abstract

We describe a 68-year-old woman who presented with falls, mild limb bradykinesia, axial rigidity, and a severe supranuclear gaze palsy, which failed to benefit from levodopa. She subsequently developed severe apraxia, progressive dysarthria, dysphagia, and a frontal cognitive impairment. Pyramidal weakness with fasciculations and widespread chronic partial denervation appeared shortly before her death from bronchopneumonia, 6 months after disease onset. A severe cerebral amyloid angiopathy diffusely involving the cerebral hemispheres and cerebellum was present at autopsy as well as a second pathological condition indicative of motor neurone disease. Cerebral amyloid angiopathy may rarely present with a progressive supranuclear palsy-like phenotype. (C) 2002 Movement Disorder Society.

Type: Article
Title: Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome
DOI: 10.1002/mds.10347
Keywords: progressive supranuclear palsy, Steele-Richardson-Olszewski, cerebral amyloid angiopathy, ALS, motor neurone disease, MND, AMYOTROPHIC-LATERAL-SCLEROSIS, RICHARDSON-OLSZEWSKI-SYNDROME, DEMENTIA, CLASSIFICATION, HEMORRHAGE, PATHOLOGY, SPECTRUM, TANGLES
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
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 Institute of Prion Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > MRC Prion Unit at UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
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
URI: http://discovery.ucl.ac.uk/id/eprint/3826
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