[Cerebral amyloid angiopathies].
RESULTS
Cerebral amyloid angiopathies are defined by the presence of amyloid deposits on the walls of cerebral vessels. These amyloid deposits are found in the media of arterioles of the leptomeninges and the cortex. They are sometimes associated with Alzheimer-type lesions. Overt amyloid vasculopathy characterized by lesions of the vascular wall with a media totally replaced by amyloid substance may be observed.
Lobular hemorrhage, often with recurrent episodes is the most frequent manifestation of cerebral amyloid angiopathy. Cerebral infarcts or leukoencephalopathy may also be observed. An association with cerebral angiitis has been reported in a few cases. SPORADIC OR FAMILIAL DISEASE: Several types can be distinguished depending on the sporadic or familiar nature of the disease pattern as well as the chemical make-up of the protein deposit. In sporadic cerebral amyloid angiopathy the deposit is composed of protein A beta, and in familial cases, of protein A beta, cystatin C, gelsosine or transthyretin. Mutation of the genes coding for amyloid substance constituents have been identified in diverse forms of familial cerebral amyloid angiopathy.
Masson
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C
Service de Neurologie, Hôpital Beaujon, Clichy. catherine.masson@bjn.ap-hop-paris.fr
Leys
D
D
Buée
L
L
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000473:pathology; D016564:Amyloid beta-Protein Precursor / Q000235:genetics; D001921:Brain / Q000473:pathology; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000235:genetics / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology; D002543:Cerebral Hemorrhage / Q000209:etiology; D002544:Cerebral Infarction / Q000209:etiology; D002556:Cerebrospinal Fluid Proteins / Q000235:genetics; D055316:Cystatin C; D015891:Cystatins / Q000235:genetics; D015853:Cysteine Proteinase Inhibitors / Q000235:genetics; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D009154:Mutation; D011228:Prealbumin / Q000235:genetics; D013702:Temporal Lobe / Q000473:pathology; D014657:Vasculitis / Q000473:pathology
D016564:Amyloid beta-Protein Precursor; C525030:CST3 protein, human; D002556:Cerebrospinal Fluid Proteins; D055316:Cystatin C; D015891:Cystatins; D015853:Cysteine Proteinase Inhibitors; D011228:Prealbumin