Progressive myoclonus in a child with a deep cerebellar mass

Myoclonus is often associated with pathology of the cerebellum. However, the site of neurons responsible for the generation of myoclonus in cerebellar disease is not known. The authors report a young child with myoclonus associated with a ganglioglioma in the region of the deep cerebellar white matter and cerebellar nuclei. They hypothesize that abnormal paroxysmal discharge of neurons in the cerebellar nuclei can generate myoclonus.