A CASE OF PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM (PACNS)

PACNS, a rare vasculitis limited to the brain, spinal cord and overlying leptomeninges. Atypical PACNS is the most common subset of PACNS. We report a 16 year old male who suffered young stroke (Right ganglio- capsular infarct) with headache. CT angiography showed occlusion of Rt.MCA segments. Takayasu arteritis, Fibromuscular dyplasia and PACNS were considered as differential diagnosis. Patient was diagnosed as Isolated CNS angiitis after excluding hypercoagulable state, infection and embolic etiologies. Patient was treated with corticosteroids for 5 months and repeat CT angiography showed persistence of the pre-existing lesion with no further progression. Hence, the case was diagnosed as an atypical subset of PACNS. This constitutes the group of CNS vasculitis which could not be entitled as granulomatous PACNS or benign angiopathy of CNS.