Rinsho Shinkeigaku (Clinical Neurology)

Original Article

Two cases of acute onset of focal cortical reflex myoclonus following acute aseptic meningoencephalitis with positive anti-glutamate receptor autoantibody

Maya Tojima, M.D.1), Takefumi Hitomi, M.D., Ph.D.1)2), Naoto Jingami, M.D.1), Kosuke Tanioka, M.D.1), Hodaka Yamakado, M.D., Ph.D.1), Riki Matsumoto, M.D., Ph.D.1)3), Yukitoshi Takahashi, M.D.4), Akio Ikeda, M.D., Ph.D.1)3) and Ryosuke Takahashi, M.D., Ph.D.1)

1)Department of Neurology, Kyoto University Hospital
2)Department of Respiratory Care and Sleep Control Medicine & Department of Clinical Laboratory Medicine, Kyoto University Graduate School of Medicine
3)Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine
4)National Epilepsy Center, Shizuoka institute of Epilepsy and Neurological Disorders

Patient 1 was a 40-year-old man, who suffered from right leg myoclonus 1 week after an episode of fever and headache. Myoclonus disappeared 4 months after administration of clonazepam. Patient 2 was a 42-year-old man, who suffered from right leg myoclonus, attacks of speech arrest and a generalized tonic-clonic seizure. His symptoms disappeared after steroid-pulse therapy, but right leg myoclonus and episodic impairment of consciousness recurred within a month. He underwent another steroid-pulse therapy and his symptoms disappeared. In both patients, cerebrospinal fluid (CSF) study showed pleocytosis and elevated protein level, electrophysiological study showed cortical reflex by stimulation of the right tibial nerve, and brain MRI showed the high intensity area in the left parietal lobe. In addition, on electroencephalogram (EEG) spikes at vertex preceded myoclonic jerk of the right tibialis anterior muscle in both patients. These findings indicate that focal cortical reflex myoclonus was accompanied by acute central nervous system (CNS) infection. Furthermore, in both patients, autoantibody against glutamate receptor subunits ε2 was detected both in serum and CSF, which also suggest that autoimmune mechanism contributed in the pathophysiology of acute development of focal cortical reflex myoclonus.
Full Text of this Article in Japanese PDF (2677K)

(CLINICA NEUROL, 54: 543|549, 2014)
key words: cortical reflex myoclonus, anti-glutamate receptor autoantibody, non-bacterial meningoencephalitis, negative motor seizure

(Received: 28-Aug-13)