Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome
✍ Scribed by Robert A. Weeks; Francisco Scaravilli; Andrew J. Lees; Camille Carroll; Masud Husain; Peter Rudge
- Publisher
- John Wiley and Sons
- Year
- 2003
- Tongue
- English
- Weight
- 92 KB
- Volume
- 18
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
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.