Clinical and Radiological Outcomes of Angiographically Proven Central Nervous System Arteriopathy

Childhood stroke is a significant contributor to neurological morbidity often leading to debilitating outcomes. This study aimed to assess the clinical and radiological outcomes of angiographically proven central nervous system (CNS) arteriopathy in children.

This prospective cohort study was conducted from June 2023 to May 2024 at The Children's Hospital, Lahore, Pakistan. The clinical outcomes were assessed using the Pediatric National Institute of Health Stroke Scale (PedNIHSS) at baseline and compared at six-month follow-up, while radiological outcomes were categorized as stable, regressive, or progressive disease. Clinical presentation, etiology, baseline PedNIHSS score, and arterial involvement were correlated with motor outcomes.

Out of the 38 patients, 63.2% were female patients with a mean age of 4.27±2.43 years. The most common age group was 2-5 years (50.0%), followed by >5-10 years (31.6%). Global clinical presentation was seen in 60.5%, while 29.0% had a recurrence of stroke. Moyamoya disease (21.1%) was the most common etiological factor, followed by primary CNS angiitis (13.0%), infections (8.0%), and post-varicella focal cerebral arteriopathy (8.0%). Bilateral infarcts (55.3%) and anterior circulation involvement (68.4%) were the most affected areas, with the middle cerebral artery (MCA) being the most affected with complete involvement in 18.4% and partial involvement in 81.5% of cases. The mean baseline PedNIHSS score was 29±6.4, which improved to 19±4.8 at the six-month follow-up. Radiological outcomes showed in 42.2% of cases, with a progression of the disease on follow-up MRA; 31.6% had stable disease, while 21.0% had regressive disease. Significant factors associated with poor motor outcomes included global presentation (p=0.000008), etiological factors (p=0.047), bilateral infarcts (p=0.050), severe baseline PedNIHSS (p=0.000019), and progressive radiological disease (p=0.003).

This study highlights the significant neurological impact of pediatric stroke. Early diagnosis, investigation of underlying causes, and identification of recurrence risk factors are crucial in preventing immediate and long-term complications.