Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Low-dose perampanel improved cortical myoclonus and basophobia in a patient with Unverricht-Lundborg disease: a case report

Yuki Oi, M.D.1)2), Katsuya Kobayashi, M.D., Ph.D.1), Takefumi Hitomi, M.D., Ph.D.1)3), Riki Matsumoto, M.D., Ph.D.1), Akio Ikeda, M.D., Ph.D.4) and Ryosuke Takahashi, M.D., Ph.D.1)

1)Department of Neurology, Kyoto University Graduate School of Medicine
2)Department of Neurology, Otowa Hospital
3)Department of Clinical Laboratory Medicine, Kyoto University Graduate School of Medicine
4)Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine

We report a 32-year-old female who presented myoclonus and generalized tonic-clonic seizure since she was 9 yearold. Thereafter, she was diagnosed as Unverricht-Lundborg disease by gene analysis. Although the epileptic seizures were controlled by multiple antiepileptic drugs, her cortical myoclonus remained intractable, which severely interfered her activity of daily living. On admission to our hospital, she presented mild cognitive impairment, dysarthria, severe postural and action myoclonus in all the limbs, severe impairment of coordinative movements, inability of standing and walking by herself, and severe basophobia. After administration of perampanel, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antagonist, with initial dose of 1 mg/day, and then 16 days later it was increased up to 2 mg/day, the myoclonus dramatically improved and the basophobia also lessened about in 30 days since it started. Moreover, abnormally enlarged amplitudes of short latency somatosensory evoked potentials by median nerve stimulation decreased, which suggested the reduction of abnormal cortical hyperexcitability mainly in the primary sensori-motor cortices. We presented that perampanel is the effective drug for treating the refractory cortical myoclonus and basophobia even with small dosage.
Full Text of this Article in Japanese PDF (402K)

(CLINICA NEUROL, 58: 622|625, 2018)
key words: Unverricht-Lundborg disease, cortical myoclonus, basophobia, perampanel, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor

(Received: 25-Apr-18)