5TH NATIONAL CONFERENCE OF PEDIATRIC RHEUMATOLOGY, KOLKATA, 29TH & 30TH SEPTEMBER 2007

*Priyankar Pal,**Debosree Ganguly,***Dipankar Das,**Vikram Patra
* Asst. Professor, ** PGT, *** Asst. Professor, Dept of Radiology
Institute Of Child Health, Kolkata

Primary angiitis of the central nervous system (PACNS) is a rare group of vasculitis limited to the brain and its overlying meninges. Described about 40 years back, the disease had always been perceived to have a relapsing and uniformly fatal course. In the 1990s, a subset was realised to have a better outcome and renamed as Benign Angiopathy of the CNS (BACNS).

A seven-year-old boy presented with severe headache and complete ophthalmoplegia. MR angiography showed segmental narrowing of bilateral internal carotid, anterior, middle and posterior cerebral arteries, as well as of vertebral and basilar arteries. A provisional diagnosis of PACNS was made. He was treated with steroids and showed good recovery. A follow up MR angio after 6 months showed a normal pattern. A 4 year follow up did not show any neurological abnormality.

Considering the stormy onset, monophasic course, angiographically demonstrable diffuse vasculitis with subsequent complete resolution and overall good prognosis clinches the final diagnosis as BACNS.

5th National Conference of Pediatric Rheumatology - Conference Abstracts. Pediatric Oncall [serial online] 2007 [cited 15 December 2007(Supplement 12)];4. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/ncpr/
central_nervous_system.asp