[Skip to Content]
[Skip to Content Landing]
June 29, 1964


JAMA. 1964;188(13):1148. doi:10.1001/jama.1964.03060390050012

An interesting clinicopathological syndrome is described in a recent issue of the Archives of Neurology.1 Although isolated cases of a similar nature have been reported previously, this is the first indication that the condition may be a distinct syndrome. The chief clinical features reported by Steele and associates include supranuclear opthalmoplegia primarily affecting vertical gaze, pseudobulbar palsy, dysarthria, and dystonic rigidity of the neck and upper trunk. The ophthalmoplegia was expressed as a loss of conjugate vertical gaze to command; however, the eyes moved fully when gaze was fixed and the head moved passively. Downward gaze was more severely affected than upward. Dementia was usually present, but to a minor degree in most patients. All nine subjects were male; their disease began in the sixth or seventh decade. In the seven fatal cases, a steady progressive course led to death in five to seven years.

The "typical" patient presented

First Page Preview View Large
First page PDF preview
First page PDF preview