A case of palatal myoclonus is reported and the clinical and neuropathological aspects are studied. The patient, a woman aged eighty two, during the clinical course of the disease showed stupor, right brachial monoplegia, aphasia, epileptic seizures and myoclonus involving the palate, pharynx and diaphragm (palatopharyngodiaphragmatic myoclonus). The movements were not influenced by sleep and remained during the coma. The post-mortem findings revealed generalized arteriosclerotic vascular changes with scattered areas of softening in the cortex, brain stem and cerebellum. The significance of a pathway directly linking the dentate nucleus of the cerebellum and the contralateral inferior olive (the dentato-olivary pathway) is stressed.