A SYNDROME OF PARALYSIS OF DOWNWARD GAZE, DYSARTHRIA, PSEUDOBULBAR PALSY, AXIAL RIGIDITY OF NECK AND TRUNK AND DEMENTIA

Abstract

Three cases are described which represent an unusually stereotyped syndrome of progressive manifestations of pseudo-ophthalmoplegia, with characteristics of paralysis of downward gaze, limitation only of upper gaze and preserved function in lateral gaze, pseudobulbar palsy, dysarthria, mild dementia and progressive axial rigidity involving mainly the neck and trunk, without significant cog-wheeling and no tremor. The syndrome eventually reaches a stage manifested by gross akinesia and apraxia of gait. Death occurred in 5 to 7 years. The etiology remains uncertain, but a degenerative disorder is suggested.