From the Departments of Neurology (R.N., H.V.V., M.L.C.M.-S., M.B., J.H.,
R.A.C.R.) and Pathology (S.G. v D.), Leiden University Medical Center, Leiden,
Netherlands; Department of Neurology, Rijnland Hospital, Leiderdorp,
Netherlands (J.H.); and Department of Pathology and Laboratory Medicine,
Section of Neuropathology, University of California, Los Angeles Medical
Center (H.V.V.).
Background and Purpose—Microvascular
changes such as microaneurysms and fibrinoid necrosis have been
found in the presence of cerebral amyloid angiopathy (CAA). These
CAA-associated microvasculopathies (CAA-AM) may contribute to the
development of CAA-associated hemorrhages and/or infarcts,
hereafter referred to as "cerebrovascular lesions." Hereditary
cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D) is
an autosomal dominant form of CAA, in which the amyloid angiopathy is
pathologically and biochemically similar to sporadic CAA associated
with aging and Alzheimer disease. To determine the significance
of CAA-AM for CAA-associated cerebrovascular complications, we
investigated the association between CAA-AM and cerebrovascular lesions
in HCHWA-D patients.
Methods—In a previous autopsy study we
semiquantitatively scored CAA-AM in 29 HCHWA-D patients. In the
present study we reviewed clinical charts and autopsy protocols of
these same patients. We investigated whether CAA-AM was associated with
age at death, number of cerebrovascular lesions, duration of clinical
illness, hypertension, and atherosclerosis.
Results—An association was found between CAA-AM and the number of
cerebrovascular lesions (P=0.009). The presence of
microaneurysmal degeneration was most strongly associated with
the number of cerebrovascular lesions (P<0.001). In
addition, we found an association between
atherosclerosis and the CAA-AM score
(P=0.047). Hypertension was not associated with
CAA-AM.
Conclusions—Our findings support previous reports suggesting an
important role of secondary microvascular degenerative changes in
CAA-associated cerebrovascular lesions and suggest an aggravating
effect of systemic atherosclerosis, but not
hypertension, on the evolution of CAA-AM. These findings may be of
relevance to understanding cerebrovascular complications of sporadic or
Alzheimer disease–associated CAA.
© 1998 American Heart Association, Inc.
Original Contributions
Microvasculopathy Is Associated With the Number of Cerebrovascular Lesions in Hereditary Cerebral Hemorrhage With Amyloidosis, Dutch Type
Key Words: Alzheimer's disease • amyloid ß protein • cerebral amyloid angiopathy • cerebral aneurysm • cerebral hemorrhage
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