Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease

Journal Article · Sat Aug 01 00:00:00 EDT 1987 · Proc. Natl. Acad. Sci. U.S.A.; (United States)

DOI:https://doi.org/10.1073/pnas.84.16.5991· OSTI ID:5209443

van Duinen, S G; Castano, E M; Prelli, F; Bots, G T.A.B.; Luyendijk, W; Frangione, B

Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related ..beta..-protein. Silver stain-positive, senile plaque-like structures were also labeled by the antiserum, yet these lesions lacked the dense amyloid cores present in typical plaques of Alzheimer disease. No neurofibrillary tangles were present. Amyloid fibrils were purified from the leptomeningeal vessels of one patient who clinically had no signs of dementia. The protein had a molecular weight of approx. 4000 and its partial amino acid sequence to position 21 showed homology to the ..beta..-protein of Alzheimer disease and Down syndrome. These results suggest that hereditary cerebral hemorrhage with amyloidosis of Dutch origin is pathogenetically related to Alzheimer disease and support the concept that the initial amyloid deposition in this disorder occurs in the vessel walls before damaging the brain parenchyma. Thus, deposition of ..beta..-protein in brain tissue seems to be related to a spectrum of diseases involving vascular syndromes, progressive dementia, or both.

• Research Organization: New York Univ. Medical Center, NY (USA)
• OSTI ID: 5209443
• Journal Information: Proc. Natl. Acad. Sci. U.S.A.; (United States), Vol. 84:16
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
CEREBRUM
CYTOCHEMISTRY
HEREDITARY DISEASES
PATHOGENESIS
NERVOUS SYSTEM DISEASES
PROTEINS
AMINO ACID SEQUENCE
HEMORRHAGE
IMMUNOLOGY
MENINGES
PATIENTS
VASCULAR DISEASES
BIOCHEMISTRY
BODY
BRAIN
CENTRAL NERVOUS SYSTEM
CHEMISTRY
DISEASES
MEMBRANES
MOLECULAR STRUCTURE
NERVOUS SYSTEM
ORGANIC COMPOUNDS
ORGANS
PATHOLOGICAL CHANGES
SYMPTOMS
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