WordNet

of or relating to the cerebrum or brain; "cerebral hemisphere"; "cerebral activity"
involving intelligence rather than emotions or instinct; "a cerebral approach to the problem"; "cerebral drama" (同)intellectual
(pathology) a waxy translucent complex protein resembling starch that results from degeneration of tissue
a non-nitrogenous food substance consisting chiefly of starch; any substance resembling starch
any disease of the blood vessels or lymph ducts

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大脳の,脳の / 頭脳的な,知的な

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/06/12 07:51:06」(JST)

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Cerebral amyloid angiopathy (CAA), also known as congophilic angiopathy,^[1] is a form of angiopathy in which amyloid deposits form in the walls of the blood vessels of the central nervous system.^[2] The term congophilic is used because the presence of the abnormal aggregations of amyloid can be demonstrated by microscopic examination of brain tissue after application of a special stain called Congo red. The amyloid material is only found in the brain and as such the disease is not related to other forms of amyloidosis.^[3]

Signs and symptoms

Since this can be caused by the same amyloid protein that is associated with Alzheimer's dementia, brain bleeds^[4] are more common in people who have a diagnosis of Alzheimer's Disease, however they can also occur in those who have no history of dementia. The bleeding within the brain is usually confined to a particular lobe^[5] and this is slightly different compared to brain bleeds which occur as a consequence of high blood pressure (hypertension) - a more common cause of a hemorrhagic stroke (or bleeding in the brain).^[6]

Causes

CAA has been identified as occurring either sporadically (generally in elderly populations)^[7] or in familial forms such as Flemish, Iowa, and Dutch types. In all cases, it is defined by the deposition of Aβ in the leptomeningal and cerebral vessel walls.^[8] CAA occurring in the Flemish type has been observed to be linked to large dense-core plaques observed in this pedigree.^[9]

The reason for increased deposition of Aβ in sporadic CAA is still unclear with both increased production of the peptide and abnormal clearance having been proposed as potential causes.^[10] Under normal physiology Aβ is cleared from the brain by four pathways: (1) endocytosis by astrocytes and microglial cells, (2) enzymatic degradation by neprilysin or insulysin(3) cleared by way of the blood brain barrier or (4) drained along periarterial spaces. Abnormalities in each of these identified clearance pathways have been linked to CAA.^[11]^[12]

In familial forms of CAA, the cause of Aβ build up is likely due to increased production rather than poor clearance.^[13] Mutations in the amyloid precursor protein (APP), Presenilin (PS) 1 and PS2 genes can result in increased rates of cleavage of the APP into Aβ. An immune mechanism has also been proposed.^[14]^[15]

Diagnosis

MRI showing low signal foci in cerebral amyloid angiopathy. Conventional gradient echo T2*-weighted image (left, TE=20ms), susceptibility weighted image (SWI) and SWI phase image (center and right, respectively, TE=40ms) at 1.5 Tesla.^[16]

Susceptibility weighted imaging has been proposed as a tool for identifying CAA-related microhemorrhages.^[17] Biopsies also play a role in diagnosing the condition.^[18]

Types

It is usually associated with amyloid beta.^[19]

However, there are other types:

the "Icelandic type" is associated with Cystatin C^[20]
the "British type" is associated with ITM2B^[21]

Research is currently being conducted to determine if there is a link between cerebral amyloid angiopathy and ingestion of excessive quantities of aluminum.^[22]

Management

The aim in cerebral amyloid angiopathy is to treat the symptoms, as there is no current cure. Physical and/or speech therapist may be helpful in the management process of this condition.^[2]

References

^ Exley C, Esiri MM (July 2006). "Severe cerebral congophilic angiopathy coincident with increased brain aluminium in a resident of Camelford, Cornwall, UK". J. Neurol. Neurosurg. Psychiatr. 77 (7): 877–9. PMC 2117501 . PMID 16627535. doi:10.1136/jnnp.2005.086553.
^ ^a ^b "Cerebral amyloid angiopathy: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-05-27.
^ Newfoundland, FRCP William Pryse-Phillips MD, FRCP(C) Faculty of Medicine Health Sciences Centre Memorial University of Newfoundland St John's (2009-05-06). Companion to Clinical Neurology. Oxford University Press, USA. ISBN 9780199710041.
^ Godefroy, Olivier (2013-02-28). The Behavioral and Cognitive Neurology of Stroke. Cambridge University Press. ISBN 9781107310896.
^ Coleman, William B.; Tsongalis, Gregory J. (2010-02-16). Essential Concepts in Molecular Pathology. Academic Press. ISBN 9780080922188.
^ "Brain Basics: Preventing Stroke: National Institute of Neurological Disorders and Stroke (NINDS)". www.ninds.nih.gov. Retrieved 2015-05-27.
^ Sirven, Joseph I.; Malamut, Barbara L. (2008). Clinical Neurology of the Older Adult. Lippincott Williams & Wilkins. ISBN 9780781769471.
^ Ettinger, Alan B.; Weisbrot, Deborah M. (2014-04-17). Neurologic Differential Diagnosis: A Case-Based Approach. Cambridge University Press. ISBN 9781107014558.
^ Kumar-Singh, S.; Cras, P.; et al. (2002). "Dense-core senile plaques in the Flemish variant of Alzheimer's disease are vasocentric". American Journal of Pathology. 161: 507–20. PMC 1850756 . PMID 12163376. doi:10.1016/S0002-9440(10)64207-1.
^ Tolnay, Markus; Probst, Alphonse (2012-12-06). Neuropathology and Genetics of Dementia. Springer Science & Business Media. ISBN 9781461512493.
^ Subramaniam, Rathan; Barrio, Jorge (2013-10-15). Novel Imaging Techniques in Neurodegenerative and Movement Disorders, an Issue of Pet Clinics. Elsevier Health Sciences. ISBN 9780323227353.
^ Brain Extracellular Matrix in Health and Disease. Elsevier. 2014-10-30. ISBN 9780444634948.
^ Hall, Tim (2013-09-17). PACES for the MRCP: with 250 Clinical Cases. Elsevier Health Sciences. ISBN 0702054666.
^ Mental Disorders: New Insights for the Healthcare Professional: 2011 Edition. ScholarlyEditions. 2012-01-09. ISBN 9781464900853.
^ Advances in Dementia Research and Treatment: 2012 Edition. ScholarlyEditions. 2012-12-26. ISBN 9781464991721.
^ Mittal, S.; Wu, Z.; Neelavalli, J.; Haacke, E. M. (2009). "Susceptibility-Weighted Imaging: Technical Aspects and Clinical Applications, Part 2". American Journal of Neuroradiology. 30 (2): 232–252. ISSN 0195-6108. PMID 19131406. doi:10.3174/ajnr.A1461.
^ Ellenbogen, Richard G.; Abdulrauf, Saleem I.; Sekhar, Laligam N. (2012). Principles of Neurological Surgery. Elsevier Health Sciences. ISBN 1437707017.
^ Verbeek, M. M.; Waal, R. M. de; Vinters, Harry V. (2013-06-29). Cerebral Amyloid Angiopathy in Alzheimer’s Disease and Related Disorders. Springer Science & Business Media. ISBN 9789401710077.
^ Dickson, Dennis; Weller, Roy O. (2011-09-09). Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders. John Wiley & Sons. ISBN 9781444341232.
^ Larner, A. J. (2008-04-24). Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders. Cambridge University Press. ISBN 9780521717922.
^ Dening, Tom; Thomas, Alan (2013-09-26). Oxford Textbook of Old Age Psychiatry. OUP Oxford. ISBN 9780199644957.
^ Kawahara, Masahiro; Kato-Negishi, Midori (2011). "Link between Aluminum and the Pathogenesis of Alzheimer's Disease: The Integration of the Aluminum and Amyloid Cascade Hypotheses". International Journal of Alzheimer's Disease. 2011: 1–17. ISSN 2090-0252. PMC 3056430 . PMID 21423554. doi:10.4061/2011/276393.

UpToDate Contents

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1. 脳アミロイドアンギオパチー cerebral amyloid angiopathy
2. 脳卒中評価の概要 overview of the evaluation of stroke
3. 脳血管性認知症の病因、臨床症状、および診断 etiology clinical manifestations and diagnosis of vascular dementia
4. アミロイド心筋症の治療 treatment of amyloid cardiomyopathy
5. 特発性脳出血：病因、臨床的特徴、および診断 spontaneous intracerebral hemorrhage pathogenesis clinical features and diagnosis

English Journal

Sex Dimorphism Profile of Alzheimer's Disease-Type Pathologies in an APP/PS1 Mouse Model.

Jiao SS1, Bu XL1, Liu YH1, Zhu C1, Wang QH1, Shen LL1, Liu CH1, Wang YR1, Yao XQ1, Wang YJ2.
Neurotoxicity research.Neurotox Res.2016 Feb;29(2):256-66. doi: 10.1007/s12640-015-9589-x. Epub 2015 Dec 26.
Alzheimer's disease (AD) is the most common form of dementia among the elderly, characterized by parenchymal and vascular beta-amyloid (Aβ) burden, tau pathology, neuroinflammation, and loss of neurons and synapses. There is a clear sex difference in the prevalence of AD. However, sex differences i
PMID 26707129

Statin Therapy and the Development of Cerebral Amyloid Angiopathy-A Rodent in Vivo Approach.

Reuter B1, Venus A2, Grudzenski S3, Heiler P4, Schad L5, Staufenbiel M6, Hennerici MG7, Fatar M8.
International journal of molecular sciences.Int J Mol Sci.2016 Jan 19;17(1). pii: E126. doi: 10.3390/ijms17010126.
BACKGROUND: Cerebral amyloid angiopathy (CAA) is characterized by vascular deposition of amyloid β (Aβ) with a higher incidence of cerebral microbleeds (cMBs) and spontaneous hemorrhage. Since statins are known for their benefit in vascular disease we tested for the effect on CAA.METHODS: APP23-tr
PMID 26797603

Detection of cerebral amyloid angiopathy by 3-T magnetic resonance imaging and amyloid positron emission tomography in a patient with subcortical ischaemic vascular dementia.

Kida H1, Satoh M1, Ii Y2, Fukuyama H3, Maeda M4, Tomimoto H1,2.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society.Psychogeriatrics.2016 Jan 18. doi: 10.1111/psyg.12187. [Epub ahead of print]
The patient was an 81-year-old man who had been treated for hypertension for several decades. In 2012, he developed gait disturbance and mild amnesia. One year later, his gait disturbance worsened, and he developed urinary incontinence. Conventional brain magnetic resonance imaging using T2 -weighte
PMID 26782037

Japanese Journal

血管性認知障害 : 高血圧性脳小血管病とアミロイド血管症をめぐって (特集 The Border-Land of Dementia)

冨本秀和
Brain and nerve : 神経研究の進歩 64(12), 1377-1386, 2012-12
NAID 40019510521

副腎皮質ステロイドが奏功した脳アミロイドアンギオパチー関連白質脳症の1例

岩永健,兼子宜之,西村広健 [他]
臨床神経学 52(8), 585-588, 2012-08
NAID 40019405797

Small-vessel diseaseと脳梗塞 (特集 Small-vessel disease)

三輪佳織,北川一夫
循環器内科 71(2), 125-132, 2012-02
NAID 40019227959

Related Pictures

★リンクテーブル★

リンク元	「脳アミロイドアンギオパチー」「congophilic angiopathy」「CAA」
拡張検索	「familial cerebral amyloid angiopathy」
関連記事	「cerebral」「amyloid」「amyloid angiopathy」

「脳アミロイドアンギオパチー」

Cerebral amyloid angiopathy
	Congo red stain
Classification and external resources
Specialty	cardiology
ICD-10	I68.0
DiseasesDB	32874
MedlinePlus	000719
eMedicine	neuro/628
MeSH	D016657
	[edit on Wikidata]

　　[★]

英: cerebral amyloid angiopathy, CAA
同: 脳アミロイド血管症、脳アミロイド血管障害。アミロイドアンギオパチーアミロイド・アンギオパチー amyloid angiopathy AA アミロイド血管症？
関: コンゴーレッド親和性血管障害

[show details]

脳アミロイド血管症 CAA : 55 件
脳アミロイドアンギオパチー CAA : 73 件
脳アミロイド血管障害 CAA : 18 件

脳アミロイド血管症 : 約 20,900 件
脳アミロイドアンギオパチー : 約 21,000 件
脳アミロイド血管障害 : 50 件

「congophilic angiopathy」

　　[★]

コンゴーレッド親和性血管障害、コンゴレッド親和性血管障害

関: cerebral amyloid angiopathy

「CAA」

　　[★] 脳アミロイドアンギオパチー cerebral amyloid angiopathy

「familial cerebral amyloid angiopathy」

　　[★]

家族性脳アミロイド血管症

「cerebral」

　　[★]

adj.

大脳の、大脳性の、脳性の、脳の

関: brain、cerebri、cerebro、cerebrum、encephalic、encephalo、encephalon

「amyloid」

　　[★]

アミロイド

関: amyloid fibril、amyloid protein

「amyloid angiopathy」

　　[★]

同: アミロイド・アンギオパチー

[pmid16627535-1] Exley C, Esiri MM (July 2006). "Severe cerebral congophilic angiopathy coincident with increased brain aluminium in a resident of Camelford, Cornwall, UK". J. Neurol. Neurosurg. Psychiatr. 77 (7): 877–9. PMC 2117501 . PMID 16627535. doi:10.1136/jnnp.2005.086553.

[nih-2] "Cerebral amyloid angiopathy: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-05-27.

[3] Newfoundland, FRCP William Pryse-Phillips MD, FRCP(C) Faculty of Medicine Health Sciences Centre Memorial University of Newfoundland St John's (2009-05-06). Companion to Clinical Neurology. Oxford University Press, USA. ISBN 9780199710041.

[4] Godefroy, Olivier (2013-02-28). The Behavioral and Cognitive Neurology of Stroke. Cambridge University Press. ISBN 9781107310896.

[5] Coleman, William B.; Tsongalis, Gregory J. (2010-02-16). Essential Concepts in Molecular Pathology. Academic Press. ISBN 9780080922188.

[6] "Brain Basics: Preventing Stroke: National Institute of Neurological Disorders and Stroke (NINDS)". www.ninds.nih.gov. Retrieved 2015-05-27.

[7] Sirven, Joseph I.; Malamut, Barbara L. (2008). Clinical Neurology of the Older Adult. Lippincott Williams & Wilkins. ISBN 9780781769471.

[8] Ettinger, Alan B.; Weisbrot, Deborah M. (2014-04-17). Neurologic Differential Diagnosis: A Case-Based Approach. Cambridge University Press. ISBN 9781107014558.

[9] Kumar-Singh, S.; Cras, P.; et al. (2002). "Dense-core senile plaques in the Flemish variant of Alzheimer's disease are vasocentric". American Journal of Pathology. 161: 507–20. PMC 1850756 . PMID 12163376. doi:10.1016/S0002-9440(10)64207-1.

[10] Tolnay, Markus; Probst, Alphonse (2012-12-06). Neuropathology and Genetics of Dementia. Springer Science & Business Media. ISBN 9781461512493.

[11] Subramaniam, Rathan; Barrio, Jorge (2013-10-15). Novel Imaging Techniques in Neurodegenerative and Movement Disorders, an Issue of Pet Clinics. Elsevier Health Sciences. ISBN 9780323227353.

[12] Brain Extracellular Matrix in Health and Disease. Elsevier. 2014-10-30. ISBN 9780444634948.

[13] Hall, Tim (2013-09-17). PACES for the MRCP: with 250 Clinical Cases. Elsevier Health Sciences. ISBN 0702054666.

[14] Mental Disorders: New Insights for the Healthcare Professional: 2011 Edition. ScholarlyEditions. 2012-01-09. ISBN 9781464900853.

[15] Advances in Dementia Research and Treatment: 2012 Edition. ScholarlyEditions. 2012-12-26. ISBN 9781464991721.

[16] Mittal, S.; Wu, Z.; Neelavalli, J.; Haacke, E. M. (2009). "Susceptibility-Weighted Imaging: Technical Aspects and Clinical Applications, Part 2". American Journal of Neuroradiology. 30 (2): 232–252. ISSN 0195-6108. PMID 19131406. doi:10.3174/ajnr.A1461.

[17] Ellenbogen, Richard G.; Abdulrauf, Saleem I.; Sekhar, Laligam N. (2012). Principles of Neurological Surgery. Elsevier Health Sciences. ISBN 1437707017.

[18] Verbeek, M. M.; Waal, R. M. de; Vinters, Harry V. (2013-06-29). Cerebral Amyloid Angiopathy in Alzheimer’s Disease and Related Disorders. Springer Science & Business Media. ISBN 9789401710077.

[19] Dickson, Dennis; Weller, Roy O. (2011-09-09). Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders. John Wiley & Sons. ISBN 9781444341232.

[20] Larner, A. J. (2008-04-24). Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders. Cambridge University Press. ISBN 9780521717922.

[21] Dening, Tom; Thomas, Alan (2013-09-26). Oxford Textbook of Old Age Psychiatry. OUP Oxford. ISBN 9780199644957.

[22] Kawahara, Masahiro; Kato-Negishi, Midori (2011). "Link between Aluminum and the Pathogenesis of Alzheimer's Disease: The Integration of the Aluminum and Amyloid Cascade Hypotheses". International Journal of Alzheimer's Disease. 2011: 1–17. ISSN 2090-0252. PMC 3056430 . PMID 21423554. doi:10.4061/2011/276393.

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案内

cerebral amyloid angiopathy