Giant cell angiitis of the central nervous system with atypical presentation

Abstract

Giant cell angiitis of the CNS is an uncommon form of vasculitis. Neurological manifestations, both of the peripheral and CNS, are common. The most frequent manifestations are visual loss and stroke. Hemorrhagic onset is uncommon. Most cases have a fatal outcome and a tissue diagnosis is rarely established in life. We describe an unusual case of giant cell angiitis beginning as a hemorrhagic tumoral-like lesion. The results of the histological and ultrastructural analysis have also been reported. Our case illustrates that giant cell angiitis should be considered as a cause of intracerebral hemorrhage, particularly when associated with a relapsing and remitting disease of the CNS.

MMP‐3 mediates psychosine‐induced globoid cell formation: Implications for leukodystrophy pathology

“…Formation of multinucleated giant cells are pathological hallmarks of many neurological diseases including amyotropic lateral sclerosis (ALS) (Fendrick et al, 2007), HIV encephalitis (Budka, 1986), Alzheimer’s disease, acute disseminated encephalomyelitis, giant cell angiitis [associated with vasculitis in the CNS (Ciappetta et al, 2010)], gliomas and neuroepithelial tumors (Adamek et al, 2008) and other leukodystrophies as well (Budka, 1986; Elleder, 1984). Among these other diseases in which giant multinucleated microglia are found, elevated expression of MMP-3 has been reported in Alzheimer’s disease (Yoshiyama et al, 2000).…”