Amyloid angiopathy combined with granulomatous angiitis of the central nervous system: report on two patients

Abstract

We report here two cases of isolated angiitis of the central nervous system associated with congophilic angiopathy. The clinical history lasted 9 months in the first patient (65 years old) and 9 years in the second patient (59 years old). It was characterized by progressive intellectual deterioration, increased protein content of the CSF and evidence of focal brain lesions in the CT scan. One patient showed chronic intracranial hypertension. Vascular lesions were limited to the brain and were characterized by granulomatous and necrotizing angiitis of the small leptomeningeal and intracortical vessels. Amyloid deposits were present in large amounts along vascular segments showing vasculitis, in foreign body giant cells, in plaque-like structures surrounding diseased perforating arterioles, along cortical microvessels and in many neuritic plaques. Close proximity and topographic overlap of vasculitis and amyloid changes suggest a possible pathogenetic relationship.