緊張性瞳孔で偶然発見されたRoss症候群の1例
66歳，女性．めまいを主訴に受診した際に，緊張性瞳孔と四肢腱反射消失をみとめた．また10歳代より，熱不耐症や左半身の発汗低下を自覚していた．ピロカルピン点眼試験では両側縮瞳，温熱発汗試験では顔面と左胸部に発汗低下をみとめ，Ross症候群と診断した．アセチルコリン皮内注射では鎖骨下と臍周囲の左側で発汗が低下していた．胃電図では平均周波数の低下とbradygastriaの頻度上昇，皮膚血流検査では左上肢で安静時皮膚血流増加と深呼吸刺激に対する血管反応性低下をみとめた．左上腕皮膚生検では毛包脂腺や汗腺は萎縮していた．免疫グロブリン静注療法は無効であった．本疾患では多様な自律神経障害が併存する可能性が...
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| Published in | 臨床神経学 Vol. 55; no. 3; pp. 160 - 164 |
|---|---|
| Main Authors | , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2015
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.55.160 |
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| Abstract | 66歳，女性．めまいを主訴に受診した際に，緊張性瞳孔と四肢腱反射消失をみとめた．また10歳代より，熱不耐症や左半身の発汗低下を自覚していた．ピロカルピン点眼試験では両側縮瞳，温熱発汗試験では顔面と左胸部に発汗低下をみとめ，Ross症候群と診断した．アセチルコリン皮内注射では鎖骨下と臍周囲の左側で発汗が低下していた．胃電図では平均周波数の低下とbradygastriaの頻度上昇，皮膚血流検査では左上肢で安静時皮膚血流増加と深呼吸刺激に対する血管反応性低下をみとめた．左上腕皮膚生検では毛包脂腺や汗腺は萎縮していた．免疫グロブリン静注療法は無効であった．本疾患では多様な自律神経障害が併存する可能性がある． |
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| AbstractList | 66歳，女性．めまいを主訴に受診した際に，緊張性瞳孔と四肢腱反射消失をみとめた．また10歳代より，熱不耐症や左半身の発汗低下を自覚していた．ピロカルピン点眼試験では両側縮瞳，温熱発汗試験では顔面と左胸部に発汗低下をみとめ，Ross症候群と診断した．アセチルコリン皮内注射では鎖骨下と臍周囲の左側で発汗が低下していた．胃電図では平均周波数の低下とbradygastriaの頻度上昇，皮膚血流検査では左上肢で安静時皮膚血流増加と深呼吸刺激に対する血管反応性低下をみとめた．左上腕皮膚生検では毛包脂腺や汗腺は萎縮していた．免疫グロブリン静注療法は無効であった．本疾患では多様な自律神経障害が併存する可能性がある． |
| Author | 岡田, 匡充 軸丸, 美香 安東, 由喜雄 米原, 敏郎 加藤, 勇樹 稲富, 雄一郎 大林, 光念 |
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| References | 2) Shin RK, Galetta SL, Ting TY, et al. Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin. Neurology 2000;55:1841-1846. 4) Beier C, Ernemann U, Gerloff C. Moving along with Ross syndrome — a patient with a 50 years history. J Neurol 2004;251:1402-1403. 5) Huber CG, Fischer M, Mohr M, et al. Sudden worsening of ross syndrome after glucocorticoid withdrawal emerges as panic disorder: a case report. J Neuropsychiatry Clin Neurosci 2012;24:E28-29. 9) Sommer C, Lindenlaub T, Zillikens D, et al. Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome. Ann Neurol 2002;52:247-250. 7) Kaneko H, Sakakibara M, Mitsuma T, et al. Possibility of postprandial electrogastrography for evaluating vagal/nonvagal cholinergic activity in humans, through simultaneous analysis of postprandial heart rate variability and serum immunoreactive hormone levels. Am J Gastroenterol 1995;90:603-609. 10) Nolano M, Provitera V, Perretti A, et al. Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. Brain 2006;129:2119-31. 12) Luong M, Jomir L, Labauge P, et al. Ross syndrome with sweating anomaly associated with Sjögren syndrome: An infrared thermo-graphic case study. Acta Derm Venereol 2011;91:80-81. 11) 松村美由起，渡辺弘美，竹宮敏子ら．Harlequin症候群とAdie症候群を呈したRoss syndrome plus．発汗学 2005;12:84-86. 16) 田村暢子，和田秀文，宮沢めぐみら．後天性分節性無汗症の2例．西日皮 1999;61: 27-31. 6) Nakamizo S, Miyachi Y, Kabashima K, et al. A case of cholinergic urticaria associated with acquired generalized hypohidrosis and abnormal neurological findings: association with incomplete Ross syndrome? Br J Dermatol 2010;162:903-905. 8) 磯野 理，北 耕平，福武敏夫ら．緊張性瞳孔を伴う無汗症（Ross症候群）その自律神経病変の広がりについて．自律神経 1989;26:55-62. 13) 中里良彦，田村直俊．無汗症．Brain Med 2006;18:167-172. 1) Ross AT. Progressive selective sudomotor denervation: a case with coexisting Adie’s syndrome. Neurology 1958;8:809-817. 17) Vasudevan B, Sawhney M, Vishal S. Ross syndrome with ana positivity: a clue to possible autoimmune origin and treatment with intravenous immunoglobulin. Indian J Dermatol 2010;55:274-276. 14) Péréon Y, de Ferron E, Fève JR, et al. Electrophysiological study of a case of diffuse loss of sweating with segmental hyperhidrosis associated with areflexia. Neurophysiol Clin 1993;23:363-369. 15) Nolano M, Provitera V, Donadio V, et al. Ross syndrome: a lesson from a monozygotic twin pair. Neurology 2013;80:417-418. 19) Reinauer S, Schauf G, Hölzle E. Ross syndrome: treatment of segmental compensatory hyperhidrosis by a modified iontophoretic device. J Am Acad Dermatol 1993;28:308-312. 20) Serra Mitjans M, Callejas Pérez MA, Valls Solé J, et al. Surgical treatment for compensatory hyperhidrosis in Adie syndrome. Arch Bronconeumol 2004;40:97-99. 3) 鈴木瑠美，沖山奈緒子，宮崎安洋ら．多汗を主訴としたRoss症候群とSjögren症候群，橋本病の合併例．皮病診療 2013;35:477-480. 18) Bergmann I, Dauphin M, Naumann M, et al. Selective degeneration of sudomotor fibers in Ross syndrome and successful treatment of compensatory hyperhidrosis with botulinum toxin. Muscle Nerve 1998;21:1790-1793. |
| References_xml | – reference: 1) Ross AT. Progressive selective sudomotor denervation: a case with coexisting Adie’s syndrome. Neurology 1958;8:809-817. – reference: 7) Kaneko H, Sakakibara M, Mitsuma T, et al. Possibility of postprandial electrogastrography for evaluating vagal/nonvagal cholinergic activity in humans, through simultaneous analysis of postprandial heart rate variability and serum immunoreactive hormone levels. Am J Gastroenterol 1995;90:603-609. – reference: 8) 磯野 理，北 耕平，福武敏夫ら．緊張性瞳孔を伴う無汗症（Ross症候群）その自律神経病変の広がりについて．自律神経 1989;26:55-62. – reference: 14) Péréon Y, de Ferron E, Fève JR, et al. Electrophysiological study of a case of diffuse loss of sweating with segmental hyperhidrosis associated with areflexia. Neurophysiol Clin 1993;23:363-369. – reference: 17) Vasudevan B, Sawhney M, Vishal S. Ross syndrome with ana positivity: a clue to possible autoimmune origin and treatment with intravenous immunoglobulin. Indian J Dermatol 2010;55:274-276. – reference: 6) Nakamizo S, Miyachi Y, Kabashima K, et al. A case of cholinergic urticaria associated with acquired generalized hypohidrosis and abnormal neurological findings: association with incomplete Ross syndrome? Br J Dermatol 2010;162:903-905. – reference: 3) 鈴木瑠美，沖山奈緒子，宮崎安洋ら．多汗を主訴としたRoss症候群とSjögren症候群，橋本病の合併例．皮病診療 2013;35:477-480. – reference: 13) 中里良彦，田村直俊．無汗症．Brain Med 2006;18:167-172. – reference: 18) Bergmann I, Dauphin M, Naumann M, et al. Selective degeneration of sudomotor fibers in Ross syndrome and successful treatment of compensatory hyperhidrosis with botulinum toxin. Muscle Nerve 1998;21:1790-1793. – reference: 16) 田村暢子，和田秀文，宮沢めぐみら．後天性分節性無汗症の2例．西日皮 1999;61: 27-31. – reference: 4) Beier C, Ernemann U, Gerloff C. Moving along with Ross syndrome — a patient with a 50 years history. J Neurol 2004;251:1402-1403. – reference: 5) Huber CG, Fischer M, Mohr M, et al. Sudden worsening of ross syndrome after glucocorticoid withdrawal emerges as panic disorder: a case report. J Neuropsychiatry Clin Neurosci 2012;24:E28-29. – reference: 15) Nolano M, Provitera V, Donadio V, et al. Ross syndrome: a lesson from a monozygotic twin pair. Neurology 2013;80:417-418. – reference: 2) Shin RK, Galetta SL, Ting TY, et al. Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin. Neurology 2000;55:1841-1846. – reference: 9) Sommer C, Lindenlaub T, Zillikens D, et al. Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome. Ann Neurol 2002;52:247-250. – reference: 20) Serra Mitjans M, Callejas Pérez MA, Valls Solé J, et al. Surgical treatment for compensatory hyperhidrosis in Adie syndrome. Arch Bronconeumol 2004;40:97-99. – reference: 12) Luong M, Jomir L, Labauge P, et al. Ross syndrome with sweating anomaly associated with Sjögren syndrome: An infrared thermo-graphic case study. Acta Derm Venereol 2011;91:80-81. – reference: 19) Reinauer S, Schauf G, Hölzle E. Ross syndrome: treatment of segmental compensatory hyperhidrosis by a modified iontophoretic device. J Am Acad Dermatol 1993;28:308-312. – reference: 11) 松村美由起，渡辺弘美，竹宮敏子ら．Harlequin症候群とAdie症候群を呈したRoss syndrome plus．発汗学 2005;12:84-86. – reference: 10) Nolano M, Provitera V, Perretti A, et al. Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. Brain 2006;129:2119-31. |
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| SubjectTerms | Adie症候群 Ross症候群 分節性無汗症 腱反射消失 自律神経障害 |
| Title | 緊張性瞳孔で偶然発見されたRoss症候群の1例 |
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