Cerebral amyloid angiopathy

Creutzfeldt-Jakob disease (CJD) Alzheimer s disease (AD) Hemodialysis-related amyloidosis Primary systemic amyloidosis Secondary systemic amyloidosis Familial amyloid polyneuropathy I Familial amyloid polyneuropathy III Cerebral amyloid angiopathy Finnish hereditary systemic amyloidosis Type II diabetes Injection-localized amyloidosis Medullary thyroid carcinoma Atrial amyloidosis... 

Olichney, J.M., Hansen, L.A., Galasko, D., et al. (1996) The apolipoprotein E epsylon-4 allele is associated with increased neuiific plaques and cerebral amyloid angiopathy in Alzheimer s disease and Lewy body variant. Neurology, 47, 190-196. 

Tian, J., Shi, J., Bailey, K., Lendon, C.L., Pickering-Brown, S.M., Mann, D.M.A. (2004) Association between apolipoprotein E E4 allele and arteriosclerosis, cerebral amyloid angiopathy, and cerebral white matter damage in Alzheimer s disease. J. Neurol. Neurosurg. Psychiatry, 75, 696-699. 

The massive amyloid deposition in the form of parenchymal plaques and/or in cerebrovascular amyloid (cerebral amyloid angiopathy) is associated with neuronal loss and dysfunction. In particular the cholinergic neurons of the basal forebrain, which are involved in the memory processes, are affected and neuron loss in these nuclei accounts for some of the AD symptoms. 

Barelli, H Lebeau, A., Vizzavona, J., et al. (1997) Characterization of new polyclonal antibodies specific for 40 and 42 amino acid-long amyloid beta peptides their use to examine the cell biology of presenilins and the immunohistochemistry of sporadic Alzheimer s disease and cerebral amyloid angiopathy cases. Mol. Med. 3, 695-707. 

Fig. 13.5. T2 -weighted gradient echo images in a patient with biopsy proven cerebral amyloid angiopathy (CAA). Extensive low signal abnormality is noted on the brain surfaces and in the parenchyma indicating the previous hemorrhages. Periventricular high intensity lesions, a common finding in CAA is also noted...

Greenberg SM, Briggs ME, Hyman BT et al (1996b) Apoli-poprotein E e4 is associated with the presence and earlier onset of hemorrhage in cerebral amyloid angiopathy. Stroke 27 1333-1337... 

Greenberg SM, O Donnell HC, Schaefer PW, Kraft E (1999) MRI detection of new hemorrhages potential marker of progression in cerebral amyloid angiopathy. Neurology 53 1135-1138... 

Itoh Y, Yamada M, Hayakawa M, Otomo E, Miyatake T (1993) Cerebral amyloid angiopathy a significant cause of cerebellar as well as lobar cerebral hemorrhage in the elderly. J Neurol Sci 116 135-141... 

All. Anders, K. H., Wang, Z. Z., Kornfeld, M., Gray F., Soontomniyomkij, V., et al., Giant cell arteritis in association with cerebral amyloid angiopathy Immunohistochemical and molecular studies [published erratum appears in Hum. Pathol. 29(2), 205] (1998). Hum. Pathol. 28(11), 1237-1246 (1997). 

G6. Greenberg, S. M., Cerebral amyloid angiopathy Prospects for clinical diagnosis and treatment. Neurology 51(3), 690-694 (1998). 

M6. Maruyama, K., Kametani, F., Ikeda, S., Ishihara, T., and Yanagisawa, N., Characterization of amyloid fibril protein from a case of cerebral amyloid angiopathy showing immunohistochemical reactivity for both 3 protein and cystatin C. Neurosci. Lett. 144(1-2), 38-42 (1992). 

M8. McCarron, M. O., Nicoll, J. A. R., Stewart, J., Ironside, J. W., Mann, D. M. A., etal., Absence of cystatin C mutation in sporadic cerebral amyloid angiopathy-related hemorrhage. Neurology 54, 242-244 (2000). 

Nl. Nagai, A., Kobayashi, S., Shimode, K., Imaoka, K., Umegae, N., etal., No mutations in cystatin C gene in cerebral amyloid angiopathy with cystatin C deposition. Mol. Chem. Neuropathol. 33(1), 63-78 (1998). 

Shimode, K., Fujihara, S., Nakamura, M., Kobayashi, S., and Tsunematsu, T., Diagnosis of cerebral amyloid angiopathy by enzyme-linked immunosorbent assay of cystatin C in cerebrospinal fluid. Stroke 22(7), 860-866 (1991). 

Primary intracerebral hemorrhage is more common than subarachnoid hemorrhage, and its incidence increases with age (see Fig. 1.1). It is more frequent in Southeast Asian, Japanese and Chinese populations than in whites. The most common causes are intracranial small vessel disease, which is associated with hypertension, cerebral amyloid angiopathy and intracranial vascular malformations (Sutherland and Auer 2006). Rarer causes include saccular aneurysms, hemostatic defects, particularly those induced by anticoagulation or therapeutic thrombolysis, antiplatelet drugs, infective endocarditis, cerebral vasculitis and recreational drug use (Neiman et al. 2000 O Connor et al. 2005). 

The site of primary intracerebral hemorrhage provides information as to the cause hypertensive hemorrhages (Fig. 7.1a) tend to occur in the basal ganglia, thalamus, and pons, while lobar hemorrhages are more often caused by cerebral amyloid angiopathy, vascular malformations and hemostatic failure (Dickinson 2001 Smith and Eichler 2006 Sutherland and Auer 2006) (Table 7.1) (Fig. 7.1b). Multiple hemorrhages suggest certain specific causes ... 

Cerebral microbleeds are seen frequently in patients with primary intracerebral hemorrhage, less commonly in patients with ischemic stroke and rarely in healthy controls (Cordonnier et al. 2007). Risk factors for cerebral microbleeds include hypertension, increasing age, diabetes, cerebral amyloid angiopathy and, less commonly, cerebral autosomal dominant arteriopathy with silent infarcts and leukoaraiosis (CADASIL) (Cordoimier et al. 2007). It is unclear whether previous use of antiplatelet agents or anticoagulants is a risk factor for cerebral microbleeds. 

Cerebral amyloid angiopathy is an organ-specific form of amyloid deposition in small and medium-sized arteries, and less commonly veins, of the cerebral cortex and meninges. 

Fig. 7.4. Brain CT scans (top) from a patient with cerebral amyloid angiopathy showing only leukoaraiosis, whereas gradient echo MRI performed on the same day (bottom) shows evidence of several previous intracerebral...

Maia LF, MacKenzie IR, Feldman HH (2007). Clinical phenotypes of cerebral amyloid angiopathy. Journal of the Neurological Sciences 257 23-30... 

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Smith EE, Eichler F (2006). Cerebral amyloid angiopathy and lobar intracerebral hemorrhage. Archives of Neurology 63 148-151... 

Thanvi B, Robinson T (2006). Sporadic cerebral amyloid angiopathy an important cause of cerebral haemorrhage in older people. Age Ageing 35 565-571... 

Greene GM, Godersky JC Biller J et al. (1990). Surgical experience with cerebral amyloid angiopathy. Stroke 21 1545-1549... 

Izumihara A, Ishihara T, Iwamoto N et al. (1999). Postoperative outcome of 37 patients with lobar intracerebral hemorrhage related to cerebral amyloid angiopathy. Stroke 30 29-33... 

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