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Progressive Supranuclear Palsy

Published online by Cambridge University Press:  05 January 2016

Joseph Jankovic  and
Haydee Rohaidy
Joseph Jankovic*
Affiliation:
Department of Neurology, Baylor College of Medicine, Houston, Texas
Haydee Rohaidy
Affiliation:
Department of Neurology, Baylor College of Medicine, Houston, Texas
*
Department of Neurology, Baylor College of Medicine, 1 Baylor Plaz, Houston, Texas 77030 U.S.A.
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Abstract:

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Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed.

Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur.

CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvment of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue S3 , August 1987 , pp. 547 - 554
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

References

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