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

Abstract

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.