Abstract
A clinical study was performed on 8 cases of sudden death (9.0%) selected from a series of 89 patients with severe handicaps who died at the Metropolitan Medical Center of Severely Handicapped during the period from 1968 through 1986. 1) All suffered from profound mental retardation with motor disturbance, but were able to react to environmental stimuli. 2) Most had mixed quadriplegia showing athetosis-related hypertonus. 3) Severe physical deformation and chronic respiratory symptoms were present in 6 of 8 patients. 4) Most of them were adolescent. 5) Autopsy revealed no particular findings to which the expected death could be attributed except for the right cardiac enlargement in 5 cases.
Four of the 8 patients were recovering from respiratory infection or were in the process of having their anticonvulsant doses tapered. These four cases had shown recent improvement in their general systemic condition. Sudden death occurred mainly during limited time periods in the early morning or in the evening and was not related to sleep. The authors suggest that the changes in the autonomic nervous system may precipitate them into sudden death.
