Abstract
We report a case of refractory tremulous myoclonus associated with benign adult familial myoclonus epilepsy (BAFME), which was controlled by the addition of primidone. A 39 year-old woman had a rich family history of epilepsy and tremulous myoclonus. Tremor in her hand appeared at 17 years of age, and generalized convulsion started at age 24. Epilepsy was diagnosed at age 29. Valproic acid and clonazepam were started, but hand tremor did not improve, and convulsions continued at yearly frequency. At age 36, levetiracetam was added and generalized convulsion disappeared. Therefore after, hand tremor worsened, and tremor in lower limbs and the trunk appeared. She was referred to our hospital at age 39. Detailed examinations led to a definitive diagnosis of BAFME. After primidone was added by referring to the American Academy of Neurology guideline on the treatment of essential tremor, tremulous tremor was controlled. This case suggests that primidone known to be effective for essential tremor may also be expected to control tremulous myoclonus.
