THE RIGID FORM OF HUNTINGTON'S DISEASE

Abstract

The elder of two brothers in the 4th generation of a family in which classical Huntington''s chorea has been traced showed signs of a Parkinsonian rigity at age 10. He began to have major epileptic fits and choreiform movements in the limbs at age 13. Advanced pseudo-bulbar palsy was the cause of death at age 17. Neuropathological examination showed (1) widespread cerebral cortical atrophy with maximal involvement of the postcentral gyri and the occipital lobes; (2) atrophied centrum ovale; (3) enlarged lateral ventricles; (4) symmetrical atrophy of the caudate nuclei and putamina; (5) a shrunken globus pallidus; and (6) slight general shrinkage of the compact zone of the substantia nigra. There were no lesions in the corpora Luysii or dentato-rubral system. It is suggested that the akinetic form of Huntington''s disease is primarily characterized by the severity of the putaminal lesions, while the choreic manifestations of this disorder are due to involvement of the caudate nuclei and their cerebral cortical connections.