We examined histopathological changes in cerebrovascular
amyloid deposition in a patient with
cerebral amyloid angiopathy receiving
corticosteroid therapy. A 69-year-old female developed subacute onset
cognitive decline, and magnetic resonance image (MRI) showed
subarachnoid hemorrhage with leptomeningeal enhancement. She entered in an apathetic state due to
communicating hydrocephalus and a ventricle-peritoneal (V-P) shunt operation was performed. Brain biopsy disclosed multiple cortical microhemorrhages and severe
Congophilic angiopathy with positive Aβ-immunoreactivity in most vessels. Inflammatory mononuclear cells surrounded a few severe
amyloid-laden leptomeningeal vessels. She received high-dose
corticosteroid, which was slowly tapered. She gradually recovered but finally died 1.5 years later with no recurrence of CAA-related
hemorrhages. Postmortem examination of the brain showed multiple old microhemorrhages in the cortex and extensive degeneration of cerebral white matter. The cortical and leptomeningeal vascular walls showed a few Congophilic
amyloid deposits, but small deposits with Aβ-immunoreactivity were frequently seen. There was no infiltration of inflammatory cells in either leptomeninges or vascular walls. Electron microscopy revealed sparse aggregation of
amyloid fibrils in significant numbers of vascular walls. Biochemical analysis disclosed that Aβ1-40-immunoreactive
amyloid protein fractions obtained from the patient's leptomeninges were very small in amount. Comparing the previous biopsy findings with those at autopsy, the total disappearance of the inflammatory cell infiltration and diminishing of the cerebrovascular
amyloid deposits were noted.