Progressive supranuclear palsy

History In 1955, Canadian neurologist J. Clifford Richardson was consulted by a good friend, a 52-year-old business executive, complaining of clumsiness, trouble in seeing and mild forgetfulness. Over the next four years his friend progressively developed an unusual constellation of symptoms including vertical supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, dystonic rigidity-in-extension of the neck and mild dementia [1]. In the next few years more patients were identified. By the early 1960s, seven of them had died. Neuropathologists Linnel and Tom at the Banting Institute examined the brains and diagnosed postencephalitic parkinsonism. Richardson disagreed with their conclusion because none of these patients had a history of encephalitis, parkinsonism or oculogyric crises [2]. In 1962, Richardson assisted by John Steele, one of his neurology residents, and Jersy Olszewski, a neuropathologist, reevaluated and reported the pathology of these intriguing cases [2]. In June 1963, at the American Neurological Association meeting in Atlantic City, Richardson presented the first clinical report of the disease. All the reported cases had defects in ocular gaze, “spasticity of the facial musculature” (we suspect dystonia), dysarthria, dysphagia, extensor rigidity of the neck with head retraction and dementia. In a companion report at the meeting of the American Association of Neuropathology, Olszewski described the cardinal neuropathological features of this disease, mainly characterized by the presence of neurofibrillary tangles without senile plaques especially in the brain stem. In April 1964, the report of nine patients, seven of whom had died, was published in the Archives of Neurology [1].