Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) with CNS involvement in a child

We describe the case of a 9-year-old boy with encephalitis associated with histiocytic necrotizing lymphadenitis (HNL), also known as Kikuchi-Fujimoto disease. The child presented with unilateral cervical lymphadenopathy and fever that evolved to encephalitis in 3 weeks. Brain MRI showed bilateral temporal lobe hyperintense signal on T2 and FLAIR, hyperintense FLAIR signal in the periaqueductal gray matter, medial walls of the third ventricle, and mammillary bodies, multiple diffusion restriction foci in a central perivascular distribution and central perivascular enhancement. The perivascular distribution and nodularity of the diffusion restriction seen in this case has not been previously reported in HNL encephalitis.