We report on an 18-year-old male patient with
dentatorubral-pallidoluysian atrophy (DRPLA) (number of CAG repeats: 68) with
progressive myoclonus epilepsy (PME), who showed a dramatic response to
levetiracetam in terms of the intensity of
myoclonus. He began to have convulsive
seizures and
myoclonus at 7 and 10 years of age, respectively, and his intelligence deteriorated from 12 years of age. EEG showed multifocal and diffuse spike-and-wave complexes. His convulsive
seizures were suppressed from 13 years of age.
At 17 years of age, the patient showed gradual intensification of erratic segmental positive
myoclonus as well as frequent atonic falls that were probably attributable to negative
myoclonus. Back averaging of EEG data revealed cortical discharges associated with positive
myoclonus. Photosensitive
myoclonic seizures were also observed. The administration of
levetiracetam alleviated positive
myoclonus and suppressed atonic falls, resulting in a remarkable improvement in the patient's quality of daily life. Reports on the efficacy of
levetiracetam for
myoclonus in DRPLA are still rare, though its effect on PME is known in the context of other
neurological disorders. Thus
levetiracetam should be subjected to clinical trials as a means of disabling
myoclonus in DRPLA.