Pallido-pyramidal degeneration, supranuclear upgaze paresis and dementia: Kufor-Rakeb syndrome

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Abstract

An unusual neurological syndrome in an Arab family with five affected siblings, is reported. Autosomal recessive inheritance is suggested by having multiple affected siblings born to phenotypically normal consanguineous parents. Similar to Davison's Pallido-pyramidal syndrome, they presented with the clinical signs and symptoms of severe parkinsonism as well as evidence of cortico-spinal tract disease. In addition, they had dementia and supranuclear upgaze paresis. MRI studies showed significant atrophy of the globus pallidus and the pyramids, as well as generalized brain atrophy in later stages. Therapy with levodopa resulted in significant improvement in the extrapyramidal dysfunction. We suggest that this probably represents a new syndrome which is closely related but not identical to the pallido-pyramidal syndrome.

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The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

Emily A Lees, Molly Maher (1986)

We analyzed the clinical features and natural history of 52 consecutive patients with the Steele-Richardson-Olszewski syndrome. The most common symptoms at onset were unsteady gait, backward falls because of poor balance, visual disturbances, slurred speech, and forgetfulness. The median duration from onset to death was 5.9 years, with a median survival after diagnosis of only 1.8 years. Bronchopneumonia was the usual cause of death recorded on death certificates.