- Grubb, Anders, et al.
Abnormal Metabolism of γ-Trace Alkaline Microprotein : The Basic Defect in Hereditary Cerebral Hemorrhage with Amyloidosis
Ingår i: New England Journal of Medicine. - Massachusetts Medical Society. - 0028-4793. ; 311:24, s. 1547-1549
- ALTHOUGH the total incidence of cerebral hemorrhage is high, comparatively few reports concerning the familial occurrence of this disease have been published.1,2 In 1935 Arnason described 10 families with a high incidence of cerebral hemorrhage and concluded that a hereditary form of the disease was present in these families.3 Further clinicopathological investigations of the disease revealed an autosomal dominant inheritance and a connection between the disease and a special form of amyloidosis confined to the cerebral vasculature.4 This type of cerebral hemorrhage is therefore generally referred to as hereditary cerebral hemorrhage with amyloidosis. Recently, the fibrillar components of the amyloid.