Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome.
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.
Weeks
Robert A
RA
Department of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Scaravilli
Francisco
F
Department of Neuropathology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Lees
Andrew J
AJ
Department of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.|Reta Lila Weston Institute of Neurological Studies, Royal Free and UCL Medical School, Windeyer Institute of Medical Science, London, United Kingdom.
Carroll
Camille
C
Department of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Husain
Masud
M
Department of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Rudge
Peter
P
Department of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
D000368:Aged; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016472:Motor Neuron Disease / Q000150:complications / Q000175:diagnosis*; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis / Q000209:etiology*; D013577:Syndrome