Central nervous system granulomastous phlebitis with limited extracranial involvement of the heart and lungs: An autopsy case

Abstract

Primary angiitis of the central nervous system is a rare condition, usually with an insidious onset. There is a wide variety of histological types (granulomatous, lymphocytic or necrotizing vasculitis) and types of vessel involved (arteries, veins or both). Most cases are idiopathic. We describe a first case of idiopathic granulomatous central nervous system phlebitis with additional limited involvement of the heart and lung, exclusively affecting small and medium sized veins in a 22-year-old woman, presenting as a sub acute headache. The reasons for this peculiar limitation of inflammation to the veins and the involvement of the heart and lungs are unknown.

A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis

“…Immunohistochemically, the infiltrated lymphocytes of the present case were mostly CD45-, CD3-, CD8- and CD20-positive and scarcely CD68- and CD4-positive. Consistent with these findings, the three above-mentioned histological types of PCNSV cases have all been reported to be CD45-, CD68-, CD8- and CD20-positive (18-25). Based on these findings, cytotoxic T lymphocytes and humoral immunity may be associated with the pathogenesis of PCNSV.…”