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Cerebrovascular Amyloidoses

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Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

The most recognizable and common cerebral nervous system amyloidosis is Alzheimer’s disease (AD) in its familiar or sporadic forms. In AD, amyloid is formed from the processing of the amyloid β precursor protein (AβPP), termed amyloid β (Aβ). There are also other types of Aβ pathology in the central nervous system that can be distinct from AD and dementia. Moreover, a number of other proteins (prions, cystatin C, gelsolin, transthyretin, immunoglobulin light chains, and BriPP) can lead to amyloidosis of the cerebral nervous system, although they are rare. Some of these proteins have mutations that cause cerebral amyloid angiopathy, resulting in hemorrhages with or without dementia. Amyloid derived from immunoglobulin light chains, AL, can be produced by intracerebral amyloidomas mimicking tumors. Finally, there are systemic amyloidoses that secondarily can involve the superficial leptomeningeal vessels and the eye. Amyloid deposition in the eyes may cause vitreous opacity, keratoconjunctivitis, glaucoma, or lattice corneal dystrophy. Amyloid deposits in the pituitary are associated with aging or adenomas.

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Correspondence to Maria M. Picken M.D., Ph.D., F.A.S.N. .

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Lee, J.M., Picken, M.M. (2012). Cerebrovascular Amyloidoses. In: Picken MD, PhD, FASN, M., Dogan, M.D., Ph.D., A., Herrera, M.D., G. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press. https://doi.org/10.1007/978-1-60761-389-3_7

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  • DOI: https://doi.org/10.1007/978-1-60761-389-3_7

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  • Publisher Name: Humana Press

  • Print ISBN: 978-1-60761-388-6

  • Online ISBN: 978-1-60761-389-3

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