Diagnosis and Treatment of a Case of Acute Onset Childhood Primary Angiitis of the CNS

Childhood primary angiitis of the CNS (cPACNS) is a rare cause of neurologic dysfunction in children. Diagnosis requires angiographic or pathologic evidence of vasculitis in the brain or spinal cord in the absence of systemic vasculitis and after exclusion of other causes of CNS dysfunction. Onset is usually insidious in angiography-negative cases. We present a case of angiography-negative PACNS causing rapid onset of CNS dysfunction mimicking meningoencepahlitis, which was successfully treated with immune suppression.