脳卒中様発作急性期の頭痛発作に対してL-アルギニンが無効でミダゾラムが有効であったMELASの1例
症例は14歳女性である．初診時，高度の拍動性頭痛を主訴に来院した．左後頭葉の頭部MRI異常所見と遺伝子検査などから，mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes（MELAS）の脳卒中様発作と診断した．脳波では左後頭部に周期性鋭波をみとめた．頭痛はL-アルギニン（L-Arg）静注後も遷延したが，ミダゾラム（MDZ）の持続投与後に脳波異常とともに改善した．17歳時と18歳時に再発し，L-Argが無効であったためふたたびMDZを投与したところ，頭痛と脳波異常は同様に改善した．MDZが...
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| Published in | 臨床神経学 Vol. 54; no. 11; pp. 882 - 887 |
|---|---|
| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2014
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.54.882 |
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| Abstract | 症例は14歳女性である．初診時，高度の拍動性頭痛を主訴に来院した．左後頭葉の頭部MRI異常所見と遺伝子検査などから，mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes（MELAS）の脳卒中様発作と診断した．脳波では左後頭部に周期性鋭波をみとめた．頭痛はL-アルギニン（L-Arg）静注後も遷延したが，ミダゾラム（MDZ）の持続投与後に脳波異常とともに改善した．17歳時と18歳時に再発し，L-Argが無効であったためふたたびMDZを投与したところ，頭痛と脳波異常は同様に改善した．MDZが神経細胞の過剰興奮性や三叉神経血管系の活性化などを抑制し，頭痛を改善させたと考えられる．L-Arg静注が無効であるMELASの頭痛に対してMDZの投与を考慮すべきである． |
|---|---|
| AbstractList | 症例は14歳女性である．初診時，高度の拍動性頭痛を主訴に来院した．左後頭葉の頭部MRI異常所見と遺伝子検査などから，mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes（MELAS）の脳卒中様発作と診断した．脳波では左後頭部に周期性鋭波をみとめた．頭痛はL-アルギニン（L-Arg）静注後も遷延したが，ミダゾラム（MDZ）の持続投与後に脳波異常とともに改善した．17歳時と18歳時に再発し，L-Argが無効であったためふたたびMDZを投与したところ，頭痛と脳波異常は同様に改善した．MDZが神経細胞の過剰興奮性や三叉神経血管系の活性化などを抑制し，頭痛を改善させたと考えられる．L-Arg静注が無効であるMELASの頭痛に対してMDZの投与を考慮すべきである． |
| Author | 寳珠山, 稔 柳, 務 安井, 敬三 長谷川, 康博 辻河, 高陽 横井, 聡 |
| Author_xml | – sequence: 1 fullname: 横井, 聡 organization: 名古屋大学神経内科 – sequence: 1 fullname: 長谷川, 康博 organization: 名古屋第二赤十字病院神経内科 – sequence: 1 fullname: 寳珠山, 稔 organization: 名古屋大学脳とこころの研究センター – sequence: 1 fullname: 安井, 敬三 organization: 名古屋第二赤十字病院神経内科 – sequence: 1 fullname: 柳, 務 organization: 社会福祉法人仁至会認知症介護研究・研修大府センター – sequence: 1 fullname: 辻河, 高陽 organization: 名古屋第二赤十字病院神経内科 |
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| References | 3) Yatsuga S, Povalko N, Nishioka J, et al. MELAS: a nationwide prospective cohort study of 96 patients in Japan. Biochim Biophys Acta 2012;1820:619-624. 5) Sakuta R, Nonaka I. Vascular involvement in mitochondrial myopathy. Ann Neurol 1989;25:594-601. 17) 大澤真木子，金子堅一郎．てんかん重積症とmidazolam．脳と発達 2003;35:139-142. 8) Iizuka T, Sakai F. Pathogenesis of stroke-like episodes in MELAS: analysis of neurovascular cellular mechanisms. Curr Neurovasc Res 2005;2:29-45. 9) Iizuka T, Sakai F, Suzuki N, et al. Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome. Neurology 2002;59:816-824. 2) Goto Y, Horai S, Matsuoka T, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation. Neurology 1992;42:545-550. 16) Blumer JL. Clinical pharmacology of midazolam in infants and children. Clin Pharmacokinet 1998;35:37-47. 11) Koga Y, Ishibashi M, Ueki I, et al. Effects of L-arginine on the acute phase of strokes in three patients with MELAS. Neurology 2002;58:827-828. 15) 古賀靖敏．ミトコンドリア病の治療―薬物治療．Clinical Neuroscience（臨床神経科学） 2012;30:1058-1063. 12) Koga Y, Akita Y, Nishioka J, et al. L-arginine improves the symptoms of strokelike episodes in MELAS. Neurology 2005;64:710-712. 20) Olesen J, Burstein R, Ashina M, et al. Origin of pain in migraine: evidence for peripheral sensitisation. Lancet Neurol 2009;8:679-690 22) Storer RJ, Akerman S, Shields KG, et al. GABAA receptor modulation of trigeminovascular nociceptive neurotransmission by midazolam is antagonized by flumazenil. Brain Res 2004;1013:188-193. 19) 大津真優，中野和俊，前田由美ら．脳卒中様発作に対しmidazolamが奏効したMELAS（mitochondrial encephalopathy, lactic acidosis and stroke-like episodes）の2例．脳と発達 2003;35:71-74. 6) Mukoyama M, Kazui H, Sunohara N, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes with acanthocytosis: a clinicopathological study of a unique case. J Neurol 1986;233:228-232. 10) Iizuka T, Sakai F. Pathophysiology of stroke-like episodes in MELAS: neuron-astrocyte uncoupling in neuronal hyperexcitability. Future Neurology 2010;5:61-83. 4) Ohama E, Ohara S, Ikuta F, et al. Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy. Acta Neuropathol 1987;74:226-233. 13) Hirata K, Akita Y, Povalko N, et al. Effect of L-arginine on synaptosomal mitochondrial function. Brain Dev 2007;30:238-245. 21) Iizuka T, Sakai F, Endo M, et al. Response to sumatriptan in headache of MELAS syndrome. Neurology 2003;61:577-578. 18) 皆川公夫，渡邊年秀．小児のけいれん重積およびけいれん群発に対する8年間のmidazolam静注治療成績の検討．脳と発達 2003;35:484-490. 7) Sano M, Ishii K, Momose Y, et al. Cerebral metabolism of oxygen and glucose in a patient with MELAS syndrome. Acta Neurol Scand 1995;92:497-502. 14) Yoneda M, Ikawa M, Arakawa K, et al. In vivo functional brain imaging and a therapeutic trial of L-arginine in MELAS patients. Biochim Biophys Acta 2012;1820:615-618. 1) Pavlakis SG, Phillips PC, DiMauro S, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol 1984;16:481-488. |
| References_xml | – reference: 1) Pavlakis SG, Phillips PC, DiMauro S, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol 1984;16:481-488. – reference: 3) Yatsuga S, Povalko N, Nishioka J, et al. MELAS: a nationwide prospective cohort study of 96 patients in Japan. Biochim Biophys Acta 2012;1820:619-624. – reference: 22) Storer RJ, Akerman S, Shields KG, et al. GABAA receptor modulation of trigeminovascular nociceptive neurotransmission by midazolam is antagonized by flumazenil. Brain Res 2004;1013:188-193. – reference: 8) Iizuka T, Sakai F. Pathogenesis of stroke-like episodes in MELAS: analysis of neurovascular cellular mechanisms. Curr Neurovasc Res 2005;2:29-45. – reference: 19) 大津真優，中野和俊，前田由美ら．脳卒中様発作に対しmidazolamが奏効したMELAS（mitochondrial encephalopathy, lactic acidosis and stroke-like episodes）の2例．脳と発達 2003;35:71-74. – reference: 6) Mukoyama M, Kazui H, Sunohara N, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes with acanthocytosis: a clinicopathological study of a unique case. J Neurol 1986;233:228-232. – reference: 5) Sakuta R, Nonaka I. Vascular involvement in mitochondrial myopathy. Ann Neurol 1989;25:594-601. – reference: 13) Hirata K, Akita Y, Povalko N, et al. Effect of L-arginine on synaptosomal mitochondrial function. Brain Dev 2007;30:238-245. – reference: 4) Ohama E, Ohara S, Ikuta F, et al. Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy. Acta Neuropathol 1987;74:226-233. – reference: 15) 古賀靖敏．ミトコンドリア病の治療―薬物治療．Clinical Neuroscience（臨床神経科学） 2012;30:1058-1063. – reference: 20) Olesen J, Burstein R, Ashina M, et al. Origin of pain in migraine: evidence for peripheral sensitisation. Lancet Neurol 2009;8:679-690 – reference: 9) Iizuka T, Sakai F, Suzuki N, et al. Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome. Neurology 2002;59:816-824. – reference: 12) Koga Y, Akita Y, Nishioka J, et al. L-arginine improves the symptoms of strokelike episodes in MELAS. Neurology 2005;64:710-712. – reference: 7) Sano M, Ishii K, Momose Y, et al. Cerebral metabolism of oxygen and glucose in a patient with MELAS syndrome. Acta Neurol Scand 1995;92:497-502. – reference: 17) 大澤真木子，金子堅一郎．てんかん重積症とmidazolam．脳と発達 2003;35:139-142. – reference: 21) Iizuka T, Sakai F, Endo M, et al. Response to sumatriptan in headache of MELAS syndrome. Neurology 2003;61:577-578. – reference: 11) Koga Y, Ishibashi M, Ueki I, et al. Effects of L-arginine on the acute phase of strokes in three patients with MELAS. Neurology 2002;58:827-828. – reference: 14) Yoneda M, Ikawa M, Arakawa K, et al. In vivo functional brain imaging and a therapeutic trial of L-arginine in MELAS patients. Biochim Biophys Acta 2012;1820:615-618. – reference: 18) 皆川公夫，渡邊年秀．小児のけいれん重積およびけいれん群発に対する8年間のmidazolam静注治療成績の検討．脳と発達 2003;35:484-490. – reference: 16) Blumer JL. Clinical pharmacology of midazolam in infants and children. Clin Pharmacokinet 1998;35:37-47. – reference: 2) Goto Y, Horai S, Matsuoka T, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation. Neurology 1992;42:545-550. – reference: 10) Iizuka T, Sakai F. Pathophysiology of stroke-like episodes in MELAS: neuron-astrocyte uncoupling in neuronal hyperexcitability. Future Neurology 2010;5:61-83. |
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| Snippet | 症例は14歳女性である．初診時，高度の拍動性頭痛を主訴に来院した．左後頭葉の頭部MRI異常所見と遺伝子検査などから，mitochondrial myopathy, encephalopathy, lactic... |
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| SubjectTerms | MELAS ミダゾラム 脳卒中様発作 脳波 頭痛 |
| Title | 脳卒中様発作急性期の頭痛発作に対してL-アルギニンが無効でミダゾラムが有効であったMELASの1例 |
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