A-3 Small-Vessel Childhood Primary Angiitis of the CNS: A Longitudinal Case Study

Abstract

Abstract Childhood primary angiitis of the central nervous system (cPACNS) is a rare autoimmune and cerebrovascular inflammatory disease associated with cognitive, behavioral, psychiatric, motor, sensory and/or visual symptoms. This longitudinal case study describes deficits associated with a rare course of refractory small vessel cPACNS (sv-cPACNS) with presentation uncharacteristically confined to the right hemisphere. Improved understanding of sv-cPACNS may promote diagnostic clarity and subsequent intervention for a rare and often refractory presentation that is scientifically underserved. Records indicate onset of acute events (paralysis, migraines, stroke-like symptoms) with diagnosis of sv-cPACNS based on brain biopsy. MRI revealed abnormalities restricted to the right cerebral hemisphere. Treatments included high-dose steroids, chemotherapy, IVIg, and other immunomodulatory agents. Treatment course was refractory with many failed therapies until remission on current monoclonal antibody regimen (Natalizumab). Patient was referred due to poor academic and social progression. Neuropsychological results indicated a profile congruent with nonverbal learning disorder, including dysfunction in visuospatial reasoning, fine motor, mathematics, and social judgement. Compared to prior evaluation (age 11), reduced overall intellectual functioning, working memory, and processing speed were observed. This case study examines an 18-year-old patient with sv-cPACNS and cognitive difficulties. Results were consistent with limited research demonstrating low average intellectual functioning with longitudinal declines in working memory and processing speed. Results enhance the current understanding of sv-cPACNS by illustrating a nonverbal learning disorder profile, coinciding with right hemispheric involvement. Unfortunately, the long-term impact on cognitive functioning, especially in small-vessel disease subtype, is still not well understood. Findings add to phenotypic variability and developmental course of previously refractory sv-cPACNS.