[Granulomatous angiitis of the CNS associated with cerebral amyloid angiopathy--an autopsied case with widespread involvement].

The patient was a 69-year-old woman without history of dementia who developed meningoencephalitis with initial signs of headache and disturbance of consciousness. In this time any abnormal lesions were admitted. Five weeks later, she developed bleeding from the right parietal lobe and underwent surgical removal of hematoma and brain biopsy at another hospital. The pathological specimen revealed beta-amyloid (Abeta) deposition in the pial vascular wall, perivascular invasion of multinucleated giant cells and lymphocytes, and the presence of numerous microinfarcts in the brain parenchyma. In response to pulsed steroid therapy, transient and slight improvement of level of consciousness was noted, but she died of pneumonia 12 weeks after disease onset. On post-mortem examination, widespread multinucleated giant cell granulomatous angiitis accompanied by Abeta deposition was found extensively even in the sacral cord. In addition, groups of tau-positive degenerated nerve processes without Abeta deposition were found scattered in the cerebral cortex.