Steele-richardson-olszewski syndrome (progressive supranuclear palsy)

General: Nerve cell degenera 555j98f tion centered in the brainstem; resemblance to Lhermitte pyramidopallidal syndrome and to Jakob disease with dementia and rigidity; onset in the sixth decade of life; prominent in males.

Ocular: Supranuclear ophthalmoplegia affecting chiefly vertical gaze, especially downward.

Clinical: Pseudobulbar palsy; dysarthria; dystonic rigidity of neck and upper trunk; axial rigidity; bradykinesia; pyramidal signs; parkinsonism; frontal lobe-type dementia.

Fearnley JM, et al. Diffuse Lewy body disease presenting with a supranuclear gaze palsy. J Neurol Neurosurg Psychiatry 1991; 54:159-l61.

Mastaglia FL, et al. Progressive supranuclear palsy (the Steele-Richardson-Olszewski syndrome). Clinical and electrophysiological observations in eleven cases. Proc Aust Assoc Neurol 1973; 10:35.

Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. Arch Neurol 1964; 10:333.

Tolosa E, et al. Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). J Neural Transm Suppl 1994; 42:15-31.