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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.

Original publication

DOI

10.1002/mds.10347

Type

Journal article

Journal

Mov Disord

Publication Date

03/2003

Volume

18

Pages

331 - 336

Keywords

Aged, Cerebral Amyloid Angiopathy, Diagnosis, Differential, Fatal Outcome, Female, Humans, Motor Neuron Disease, Supranuclear Palsy, Progressive, Syndrome