感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症の1例
感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症（spinal and bulbar muscular atrophy，以下SBMAと略記）を報告する．症例は50歳日本人男性．11日前からの感冒症状に続き急性に嗄声と呼吸困難が出現した．喉頭鏡検査で両側声帯麻痺を認め，気管切開を行った．追加の問診で，以前からの手指の姿勢時振戦や口周囲顔面筋のcontraction fasciculation，嚥下障害が判明した．血清CK高値，胸部computed tomography（CT）で両側乳腺腫大，針筋電図で神経原性変化を認めた．Androgen受容体遺伝子のCAG反復回数延長（47）を認め，...
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| Published in | 臨床神経学 p. cn-002090 |
|---|---|
| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2025
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.cn-002090 |
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| Abstract | 感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症（spinal and bulbar muscular atrophy，以下SBMAと略記）を報告する．症例は50歳日本人男性．11日前からの感冒症状に続き急性に嗄声と呼吸困難が出現した．喉頭鏡検査で両側声帯麻痺を認め，気管切開を行った．追加の問診で，以前からの手指の姿勢時振戦や口周囲顔面筋のcontraction fasciculation，嚥下障害が判明した．血清CK高値，胸部computed tomography（CT）で両側乳腺腫大，針筋電図で神経原性変化を認めた．Androgen受容体遺伝子のCAG反復回数延長（47）を認め，SBMAと診断した．声帯麻痺を生じるSBMAは稀だが鑑別に挙げる必要があり，詳細な病歴聴取や所見評価が求められる． |
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| AbstractList | 感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症（spinal and bulbar muscular atrophy，以下SBMAと略記）を報告する．症例は50歳日本人男性．11日前からの感冒症状に続き急性に嗄声と呼吸困難が出現した．喉頭鏡検査で両側声帯麻痺を認め，気管切開を行った．追加の問診で，以前からの手指の姿勢時振戦や口周囲顔面筋のcontraction fasciculation，嚥下障害が判明した．血清CK高値，胸部computed tomography（CT）で両側乳腺腫大，針筋電図で神経原性変化を認めた．Androgen受容体遺伝子のCAG反復回数延長（47）を認め，SBMAと診断した．声帯麻痺を生じるSBMAは稀だが鑑別に挙げる必要があり，詳細な病歴聴取や所見評価が求められる． |
| Author | 酒井 直樹 柴田 一馬 吉見 亘弘 小関 昭仁 森下 直樹 鈴木 洋司 |
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| References | 5) 中村毅，三枝英人，愛野威一郎ら．一側声帯麻痺をきたしたKennedy-Alter-Sung症候群の1症例とその筋電図所見．耳喉頭頸 2006;78:887-890. 19) 伊藤恵子，千年紘子，小林麻．Floppy epiglottisにより上気道閉塞を来した筋萎縮性側索硬化症の2症例．日耳鼻 2009;112:660-664. 10) 滝山嘉久，澤田幹雄，中野今治．球脊髄性筋萎縮症と声帯麻痺．神経内科 2003;58:442-446. 17) Sperfeld AD, Hanemann CO, Ludolph AC, et al. Laryngospasm. An underdiagnosed symptom of X―linked spinobulbar muscular atrophy. Neurology 2005;64:753-754. 18) Holley D, Mendez A, Donald C. Paroxysmal laryngospasm: Episodic closure of the upper airway. JAAPA 2019;32:31-34. 15) Hayashi M, Isozaki E, Oda M, et al. Loss of large myelinated nerve fibers of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy. J Neurol Neurosurg Psychiatry 1997;62:234-238. 8) 緒方明彦，長嶋敏子，長嶋和郎．球脊髄性筋萎縮症における中枢神経の病理．神経内科 1989;30:16-23. 12) Finsterer J, Soraru G. Onset manifestations of spinal and bulbar muscular atrophy (Kennedy’s disease). J Mol Neurosci 2016;58:321-329. 11) 近藤直英，佐橋健太郎，飯田円ら．球脊髄性筋萎縮症の病態と治療法の開発．神経治療 2017;34:101-106. 7) 大森孝一．喉頭の臨床解剖．日本耳鼻咽喉科学会会報 2009;112:86-89. 9) Nagashima T, Seko K, Hirose K, et al. Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome): autopsy case report of two brothers. J Neurol Sci 1988;87:141-152. 16) Hillel A, Dray T, Miller R, et al. Presentation of ALS to the otolaryngologist head and neck surgeon: getting to the neurologist. Neurology 1999;53(Suppl 5):S22-S25. 3) 冨保和宏，斎藤豊和，糠澤達志ら．両側声帯麻痺をきたした球脊髄性筋萎縮症例．臨床神経 1996;36:683-686. 2) 水地智基，三澤園子．Guillain-Barré症候群の診断と治療overview．脳神経内科 2023;99:289-294. 6) Niki M, Tachikawa T, Sano Y, et al. Previously undiagnosed spinal and bulbar muscular atrophy as a cause of airway obstruction after robot-assisted laparoscopic prostatectomy. Case Rep Anesthesiol. 2017;2017:9780265. 13) Fratta P, Nirmalananthan N, Masset L, et al. Correlation of clinical and molecular features in spinal bulbar muscular atrophy. Neurology 2014;82:2077-2084. 1) Kennedy WR, Alter M, Sung JH. Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology 1968;18:671-680. 14) 磯崎英治，林理之，林田哲郎ら．神経変性疾患における内喉頭筋病変：声帯麻痺との関連において．臨床神経 1998;38:711-718. 20) Shimohata T, Shinoda H, Nakayama H, et al. Daytime hypoxemia, sleep-disordered breathing, and laryngopharyngeal findings in multiple system atrophy. Arch Neurol 2007;64:856-861. 4) 澤田幹雄，滝山嘉久，中野今治．球脊髄性筋萎縮症と声帯麻痺．医のあゆみ 1999;191:833-835. |
| References_xml | – reference: 19) 伊藤恵子，千年紘子，小林麻．Floppy epiglottisにより上気道閉塞を来した筋萎縮性側索硬化症の2症例．日耳鼻 2009;112:660-664. – reference: 3) 冨保和宏，斎藤豊和，糠澤達志ら．両側声帯麻痺をきたした球脊髄性筋萎縮症例．臨床神経 1996;36:683-686. – reference: 14) 磯崎英治，林理之，林田哲郎ら．神経変性疾患における内喉頭筋病変：声帯麻痺との関連において．臨床神経 1998;38:711-718. – reference: 12) Finsterer J, Soraru G. Onset manifestations of spinal and bulbar muscular atrophy (Kennedy’s disease). J Mol Neurosci 2016;58:321-329. – reference: 2) 水地智基，三澤園子．Guillain-Barré症候群の診断と治療overview．脳神経内科 2023;99:289-294. – reference: 18) Holley D, Mendez A, Donald C. Paroxysmal laryngospasm: Episodic closure of the upper airway. JAAPA 2019;32:31-34. – reference: 20) Shimohata T, Shinoda H, Nakayama H, et al. Daytime hypoxemia, sleep-disordered breathing, and laryngopharyngeal findings in multiple system atrophy. Arch Neurol 2007;64:856-861. – reference: 13) Fratta P, Nirmalananthan N, Masset L, et al. Correlation of clinical and molecular features in spinal bulbar muscular atrophy. Neurology 2014;82:2077-2084. – reference: 8) 緒方明彦，長嶋敏子，長嶋和郎．球脊髄性筋萎縮症における中枢神経の病理．神経内科 1989;30:16-23. – reference: 10) 滝山嘉久，澤田幹雄，中野今治．球脊髄性筋萎縮症と声帯麻痺．神経内科 2003;58:442-446. – reference: 17) Sperfeld AD, Hanemann CO, Ludolph AC, et al. Laryngospasm. An underdiagnosed symptom of X―linked spinobulbar muscular atrophy. Neurology 2005;64:753-754. – reference: 5) 中村毅，三枝英人，愛野威一郎ら．一側声帯麻痺をきたしたKennedy-Alter-Sung症候群の1症例とその筋電図所見．耳喉頭頸 2006;78:887-890. – reference: 15) Hayashi M, Isozaki E, Oda M, et al. Loss of large myelinated nerve fibers of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy. J Neurol Neurosurg Psychiatry 1997;62:234-238. – reference: 6) Niki M, Tachikawa T, Sano Y, et al. Previously undiagnosed spinal and bulbar muscular atrophy as a cause of airway obstruction after robot-assisted laparoscopic prostatectomy. Case Rep Anesthesiol. 2017;2017:9780265. – reference: 7) 大森孝一．喉頭の臨床解剖．日本耳鼻咽喉科学会会報 2009;112:86-89. – reference: 4) 澤田幹雄，滝山嘉久，中野今治．球脊髄性筋萎縮症と声帯麻痺．医のあゆみ 1999;191:833-835. – reference: 11) 近藤直英，佐橋健太郎，飯田円ら．球脊髄性筋萎縮症の病態と治療法の開発．神経治療 2017;34:101-106. – reference: 16) Hillel A, Dray T, Miller R, et al. Presentation of ALS to the otolaryngologist head and neck surgeon: getting to the neurologist. Neurology 1999;53(Suppl 5):S22-S25. – reference: 1) Kennedy WR, Alter M, Sung JH. Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology 1968;18:671-680. – reference: 9) Nagashima T, Seko K, Hirose K, et al. Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome): autopsy case report of two brothers. J Neurol Sci 1988;87:141-152. |
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| Snippet | 感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症（spinal and bulbar muscular atrophy，以下SBMAと略記）を報告する．症例は50歳日本人男性．11日前からの... |
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| SubjectTerms | 両側声帯麻痺 呼吸困難 嗄声 球脊髄性筋萎縮症 |
| Title | 感染を契機に急性に両側声帯麻痺が顕在化した球脊髄性筋萎縮症の1例 |
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