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• Vol.54 No.11
• CLINICA NEUROL, 54: 882−887, 2014
• Vol.54 No.11 contents

Case Report

Effectiveness of midazolam for L-arginine-resistant headaches during stroke-like episodes in MELAS: a case report

Koyo Tsujikawa, M.D.¹⁾, Satoshi Yokoi, M.D.²⁾, KeizoYasui, M.D.¹⁾, Yasuhiro Hasegawa, M.D.¹⁾, Minoru Hoshiyama, M.D.³⁾ and Tsutomu Yanagi, M.D.⁴⁾

¹⁾Department of Neurology, Nagoya Daini Red Cross Hospital
²⁾Department of Neurology, Nagoya University Graduate School of Medicine
³⁾Brain & Mind Research Center, Nagoya University
⁴⁾Obu Dementia Care Research and Training Center

A 14-year-old girl was referred to us with severe migraine-like headaches associated with vomiting and right homonymous hemianopsia. On admission, MRI examination showed high signals in the left occipital cortex and subcortex on T₂-weighted images, without reduction of apparent diffusion coefficient suggestive of cerebral infarction. Her EEG demonstrated periodic sharp waves in the left posterior region, and laboratory tests revealed she had increased levels of lactic and pyruvic acid both in blood plasma and CSF. Gene analysis confirmed mitochondrial DNA A3243G mutation. Based on this data, we diagnosed her as having mitochondrial myopathy, encephalopathy, lactic acidosis and a stroke-like episode (MELAS). L-arginine infusion was unsuccessful for her severe headaches, which remained prolonged. She received a low dose (0.05 mg/kg/h) midazolam infusion, resulting in immediate improvement and the disappearance of headaches and abnormal EEG findings. By the age of 18, she had been readmitted eight times for stroke-like episodes accompanied by headaches. While L-arginine infusions alleviated her headaches when administered on day 1 of her episodes, they were not effective when started on or after day 2. Her L-arginine-resistant headaches were relieved by midazolam. Although the pathogenesis of headaches in MELAS is still unknown, neuronal hyperexcitability and trigeminovascular activation are considered important. Midazolam may play a role in suppressing neuronal hyperexcitability and trigeminovascular activation. Treatment with midazolam is advisable for headaches in patients with MELAS, in the event that L-arginine therapy is unsuccessful.
Full Text of this Article in Japanese PDF (5048K)

(CLINICA NEUROL, 54: 882−887, 2014)
key words: MELAS, stroke-like episode, headache, midazolam, electroencephalogram

(Received: 18-Nov-13)