Primary Central Nervous System Vasculitis in Childhood (cPACNS): A Case Series Study

Author(s): Farhad Salehzadeh MD, Adel Ahadi MD*, Omid Akbari MD

Background: Primary CNS angiitis of childhood (cPACNS) is an autoimmune process of CNS vascular structure. It is considered a novel and challenging clinical problem in pediatric neurology and rheumatology. This study discusses a significant case series study of cPACNS.

Methods: From 1994 to 2021 at pediatric rheumatology clinic, patients <18 years of age were enrolled this study with diagnosis of cPACNS if they had: a clinical symptoms compatible with primary CNS vasculitis, and MRA findings demonstrating arterial stenosis and or aneurism that are not attributable to other disease and background.

Results: There were 22 patients with mean age 10 years, 12 patients (54%) were female. The mean delayed time to diagnosis was 4 years. The most common neurologic symptoms were headache (88%) then seizure and mental disorder in (45%). Fever was in (54%) and positive ANA result in (31%) patients. 14 patients (63 %) showed abnormality in both MCA and ACA, whereas PCA showed this abnormity in 36% (8) patients. Most of patients 86 % (19) had normal EEG findings. Five patients (22%) had severe and permanent neurological damage and sequel.

Conclusion: Although cPACNS seems to be a rare and unfamiliar disorder, however it can be conceptualized much in the same way as pediatric CNS ischemic disorder, and it should be considered in any patient with unexplained headache, seizure and mental disorders.