A Primary Angiitis of the Central Nervous System (PACNS) is ill-defined, complex and rare in children. Clinical presentation is variable, diagnosis is challenging and it is life-threatening but treatable. The index case is a 9-year old male who presented with progressively slurred speech, progressive weakness of the limbs, hemifacial weakness and vomiting. There were no clinical or laboratory features of systemic vasculitis. Neuroimaging showed multiple subacute infarcts in both cerebral hemispheres. He responded well to immunosuppressive therapy. The case report aims to underscore the need for high index of suspicion, early neuroimaging for progressive nonspecific decline in neurologic function and the treatable nature of the condition if diagnosis is made early.
Keywords: Primary CNS vasculitis; aphasia; cerebral infarcts; childhood PACNS; progressive limb weakness.