Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy: Report of two cases and review of the literature

Early diagnosis is essential for the effective management of primary angiitis of the CNS (PACNS), but the presence of cerebrovascular amyloid angiopathy (CAA) may complicate the pathologic diagnosis since nonvasculitic inflammatory reactions can accompany CAA. We report two patients with PACNS associated with CAA in whom the progression of symptoms ceased during combined corticosteroid/cyclophosphamide therapy. One patient had prominent eosinophilic vasculitis and eosinophilic CSF pleocytosis. Based on review of reported cases, features supporting the diagnosis of symptomatic vasculitis in these patients include subacute progression of mental status changes and multifocal deficits, elevated ESR and CSF protein, and multifocal nonhemorrhagic lesions on imaging studies. We conclude that combined disease (PACNS/CAA) is similar to PACNS and probably occurs more frequently than expected by coincidence. The presence of CAA should not alter the treatment strategy in patients presenting with symptoms and laboratory studies consistent with PACNS.