123I-IMP-SPECT検査で前頭頭頂葉の血流低下を認め，広汎な高次脳機能障害を呈した古典型脳表ヘモジデリン沈着症の1例

72歳男性．約10か月前から電子マネーカードのチャージや携帯電話の掛け方が分からないなどの症状が出現した．入院時には近時記憶障害，構成障害，失書，失計算など広汎な高次脳機能障害を認めた．頭部MRI T2*強調画像で脳表面をびまん性に縁取る低信号を認め，古典型脳表ヘモジデリン沈着症（cortical superficial siderosis，以下cSSと略記）と診断したが，小脳や脳幹へのヘモジデリン沈着がめだたない点が特徴的であった．123I-IMP-SPECT検査で左優位に前頭頭頂葉の血流低下を認めた．脳表限局性微小出血や脳表へのヘモジデリン沈着を認め，probable CAA（cerebr...

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Published in臨床神経学 Vol. 63; no. 8; pp. 505 - 512
Main Authors 藤井, 裕樹, 峰, 奈保子, 松島, 勇人, 井料, 崇文, 北村, 健
Format Journal Article
LanguageJapanese
Published 日本神経学会 2023
Subjects
APOE
アルツハイマー病
皮質性くも膜下出血
脳アミロイドアンギオパチー
脳表ヘモジデリン沈着症
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ISSN0009-918X
1882-0654
DOI10.5692/clinicalneurol.cn-001828

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Abstract 72歳男性．約10か月前から電子マネーカードのチャージや携帯電話の掛け方が分からないなどの症状が出現した．入院時には近時記憶障害，構成障害，失書，失計算など広汎な高次脳機能障害を認めた．頭部MRI T2*強調画像で脳表面をびまん性に縁取る低信号を認め，古典型脳表ヘモジデリン沈着症（cortical superficial siderosis，以下cSSと略記）と診断したが，小脳や脳幹へのヘモジデリン沈着がめだたない点が特徴的であった．123I-IMP-SPECT検査で左優位に前頭頭頂葉の血流低下を認めた．脳表限局性微小出血や脳表へのヘモジデリン沈着を認め，probable CAA（cerebral amyloid angiopathy：脳アミロイドアンギオパチー）と診断，CAAに伴うcSSと考えた．
AbstractList 72歳男性．約10か月前から電子マネーカードのチャージや携帯電話の掛け方が分からないなどの症状が出現した．入院時には近時記憶障害，構成障害，失書，失計算など広汎な高次脳機能障害を認めた．頭部MRI T2*強調画像で脳表面をびまん性に縁取る低信号を認め，古典型脳表ヘモジデリン沈着症（cortical superficial siderosis，以下cSSと略記）と診断したが，小脳や脳幹へのヘモジデリン沈着がめだたない点が特徴的であった．123I-IMP-SPECT検査で左優位に前頭頭頂葉の血流低下を認めた．脳表限局性微小出血や脳表へのヘモジデリン沈着を認め，probable CAA（cerebral amyloid angiopathy：脳アミロイドアンギオパチー）と診断，CAAに伴うcSSと考えた．
Author 峰, 奈保子
松島, 勇人
藤井, 裕樹
井料, 崇文
北村, 健
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References 10) Kumar N. Superficial siderosis: Associations and therapeutic implication. Arch Neurol 2007;64:491-496.
13) Charidimou A, Zonneveld HI, Shams S, et al. APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology 2019;93:e358-e371.
26) Greenberg SM, Vonsattel JP, Segal AZ, et al. Association of apolipoprotein E epsilon2 and vasculopathy in cerebral amyloid angiopathy. Neurology 1998;50:961-965.
2) Offenbacher H, Fazekas F, Schmidt R, et al. Superficial siderosis of the central nervous system; MRI findings and clinical significance. Neuroradiology 1996;38:S51-S56.
5) Knudsen KA, Rosand J, Karluk D, et al. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 2001;56:537-539.
6) 町野由佳，中山茂穂，高島慎吾ら．てんかん発作をくりかえした脳表ヘモジデリン沈着症の1例．臨床神経 2010;50:108-110.
18) Kumar N, Cohen-Gadol AA, Wright RA, et al. Superficial siderosis. Neurology 2006;66:1144-1152.
22) Joonho K, Han KN, Jeong-Hyeon S, et al. Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis. Neurology 2018;90:e1751-e1758.
14) Raposo N, Calviere L, Cazzola V, et al. Cortical superficial siderosis and acute convexity subarachnoid hemorrhage in cerebral amyloid angiopathy. Eur J Neurol 2018;25:253-259.
20) 山脇健盛．脳表ヘモジデリン沈着症の臨床．臨床神経 2012;52:947-950.
16) Herzig MC, Van Nostrand WE, Jucker M. Mechanism of cerebral beta-amyloid angiopathy: murine and cellular models. Brain Pathol 2006;16:40-54.
4) 山脇健盛，櫻井圭太．脳表ヘモジデリン沈着症の診断と治療．Brain Nerve 2013;65:843-855.
11) McCarron MO, Flynn PA, Owens C, et al. Superficial siderosis of the central nervous system many years after neurosurgical procedures. J Neurol Neurosurg Psychiatry 2003;74:1326-1328.
15) Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of theleterature. Nat Clin Pract Neurol 2007;3:54-58.
19) 迫田礼子，山下謙一郎，林田光正ら．MRI CISS（constructive interference in steady state）法により出血源の硬膜欠損部が同定でき，硬膜閉鎖術が有効であった脳表ヘモジデリン沈着症の1例．臨床神経 2017;57:180-183.
24) Aggleton JP, Brown MW. Episodic memory, amnesia, and the hippocampal-anterior thalamic axis. Behav Brain Sci 1999;22:425-444.
3) McGee DA, van Patter HJ, Morotta J, et al. Subpial cerebral siderosis, a case report of two cases. Neurology 1952;12:108-113.
7) 山脇健盛，櫻井圭太．脳表ヘモジデリン沈着症．神経内科 2010;73:147-155.
9) Kellermier H, Wang G, Wiley C. Iron localization in superficial siderosis of the central nerve system. Neuropathology 2009;29:187-195.
12) Duncan W, Fiona C, Simon FF, et al. Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol 2017;81:333-343.
17) 山田正仁．脳アミロイドアンギオパチー．認知症学3．日本臨床 2004;62:167-173.
8) Koeppen AH, Borke RC. Experimental superficial siderosis of the central nerve system. 1. Morphological observation. J Neuropathol Exp Neurol 1991;50:579-594.
21) Charidimou A, Linn J, Vernooij MW, et al. Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain 2015;138:2126-2139.
23) Papez JW. A proposed mechanism of emotion. Arch Neurol Psychiatry 1937;38:725-743.
25) Rannikmae K, Samarasekera N, Martinez-Gonzalez NA, et al. Genetics of cerebral amyloid angiopathy: systematic review and ‍meta-analysis. J Neurol Neurosurg Psychiatry 2013;84:901-908.
1) Koeppen AH, Dentinger MP. Brain hemosiderin and superficial siderosis of the central nerve system. J Neuropathol Exp Neurol 1988;47:249-270.
References_xml – reference: 5) Knudsen KA, Rosand J, Karluk D, et al. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 2001;56:537-539.
– reference: 18) Kumar N, Cohen-Gadol AA, Wright RA, et al. Superficial siderosis. Neurology 2006;66:1144-1152.
– reference: 19) 迫田礼子，山下謙一郎，林田光正ら．MRI CISS（constructive interference in steady state）法により出血源の硬膜欠損部が同定でき，硬膜閉鎖術が有効であった脳表ヘモジデリン沈着症の1例．臨床神経 2017;57:180-183.
– reference: 12) Duncan W, Fiona C, Simon FF, et al. Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol 2017;81:333-343.
– reference: 14) Raposo N, Calviere L, Cazzola V, et al. Cortical superficial siderosis and acute convexity subarachnoid hemorrhage in cerebral amyloid angiopathy. Eur J Neurol 2018;25:253-259.
– reference: 11) McCarron MO, Flynn PA, Owens C, et al. Superficial siderosis of the central nervous system many years after neurosurgical procedures. J Neurol Neurosurg Psychiatry 2003;74:1326-1328.
– reference: 24) Aggleton JP, Brown MW. Episodic memory, amnesia, and the hippocampal-anterior thalamic axis. Behav Brain Sci 1999;22:425-444.
– reference: 13) Charidimou A, Zonneveld HI, Shams S, et al. APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology 2019;93:e358-e371.
– reference: 17) 山田正仁．脳アミロイドアンギオパチー．認知症学3．日本臨床 2004;62:167-173.
– reference: 6) 町野由佳，中山茂穂，高島慎吾ら．てんかん発作をくりかえした脳表ヘモジデリン沈着症の1例．臨床神経 2010;50:108-110.
– reference: 26) Greenberg SM, Vonsattel JP, Segal AZ, et al. Association of apolipoprotein E epsilon2 and vasculopathy in cerebral amyloid angiopathy. Neurology 1998;50:961-965.
– reference: 3) McGee DA, van Patter HJ, Morotta J, et al. Subpial cerebral siderosis, a case report of two cases. Neurology 1952;12:108-113.
– reference: 21) Charidimou A, Linn J, Vernooij MW, et al. Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain 2015;138:2126-2139.
– reference: 10) Kumar N. Superficial siderosis: Associations and therapeutic implication. Arch Neurol 2007;64:491-496.
– reference: 15) Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of theleterature. Nat Clin Pract Neurol 2007;3:54-58.
– reference: 2) Offenbacher H, Fazekas F, Schmidt R, et al. Superficial siderosis of the central nervous system; MRI findings and clinical significance. Neuroradiology 1996;38:S51-S56.
– reference: 16) Herzig MC, Van Nostrand WE, Jucker M. Mechanism of cerebral beta-amyloid angiopathy: murine and cellular models. Brain Pathol 2006;16:40-54.
– reference: 9) Kellermier H, Wang G, Wiley C. Iron localization in superficial siderosis of the central nerve system. Neuropathology 2009;29:187-195.
– reference: 23) Papez JW. A proposed mechanism of emotion. Arch Neurol Psychiatry 1937;38:725-743.
– reference: 20) 山脇健盛．脳表ヘモジデリン沈着症の臨床．臨床神経 2012;52:947-950.
– reference: 8) Koeppen AH, Borke RC. Experimental superficial siderosis of the central nerve system. 1. Morphological observation. J Neuropathol Exp Neurol 1991;50:579-594.
– reference: 25) Rannikmae K, Samarasekera N, Martinez-Gonzalez NA, et al. Genetics of cerebral amyloid angiopathy: systematic review and ‍meta-analysis. J Neurol Neurosurg Psychiatry 2013;84:901-908.
– reference: 1) Koeppen AH, Dentinger MP. Brain hemosiderin and superficial siderosis of the central nerve system. J Neuropathol Exp Neurol 1988;47:249-270.
– reference: 4) 山脇健盛，櫻井圭太．脳表ヘモジデリン沈着症の診断と治療．Brain Nerve 2013;65:843-855.
– reference: 22) Joonho K, Han KN, Jeong-Hyeon S, et al. Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis. Neurology 2018;90:e1751-e1758.
– reference: 7) 山脇健盛，櫻井圭太．脳表ヘモジデリン沈着症．神経内科 2010;73:147-155.
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Snippet 72歳男性．約10か月前から電子マネーカードのチャージや携帯電話の掛け方が分からないなどの症状が出現した．入院時には近時記憶障害，構成障害，失書，失計算など広汎な...
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SubjectTerms APOE
アルツハイマー病
皮質性くも膜下出血
脳アミロイドアンギオパチー
脳表ヘモジデリン沈着症
Title 123I-IMP-SPECT検査で前頭頭頂葉の血流低下を認め，広汎な高次脳機能障害を呈した古典型脳表ヘモジデリン沈着症の1例
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