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Childhood primary angiitis of the central nervous system: poor neurologic outcome despite treatment

Muhammad A. Malik, Nadeem Shabbir, Muhammed Saeed, Hamza Malik

Background: Childhood primary angiitis of the central nervous system (cPACNS) is one of the most formidable diagnostic and therapeutic challenges to pediatric neurologists. Understanding the long-term outcomes related to cPACNS in children is important in the development of secondary prevention strategies. Methods: A prospective, long-term, follow-up study was performed at the Brain Associates Institute Lahore. Children with cPACNS were reviewed every 12 months and neuropsychological assessment was done with the Pediatric Stroke Assessment Outcome Measurement (PSOM) between January 2008-June 2014. Results: Among 56 survivors assessed by PSOM at the time of discharge from hospital, 11 (20%) were normal; 14 (25%) had minor disabilities; 11 (20%) had moderate disabilities and 20 (35%) had severe disabilities. These patients were analyzed every 12 months and the final follow-up was at 60 months after discharge. At the final follow-up, a very high mortality rate of 39% was observed, with the highest mortality (54%), during the first 12 months post-discharge. Of the 22 patients who died, 16 (73%) died directly due to cPACNS, 6 (23%) patients died from other causes. At the final follow-up, only 28 (50%) of the discharged cPACNS were available, as 6 (11%) patients were lost in follow-up. Of these, 19 (37%) had independent lives, 7 (12.5%) required some help and 2 (3.5%) were totally care dependent. Conclusions: Long-term neurological, neuropsychological and functional impairments are common in cPACNS survivors. Outcome improvement should focus on minimizing complications and evolution of the disease should be monitored with meticulous neurorehabilitation.