Recurrent acute necrotizing encephalopathy. A 2-year-old boy with previous influenza, hyperpyrexia, ataxia, episodes of prolonged stupor. A mutation in ran-binding protein 2 was confirmed. Characteristic distribution of lesions involve the pons, midbrain, thalamus and external capsule bilaterally (D). T2 hyperintense and T1 hypointense lesions of the pontine transverse fibers (A) and of the ventro-lateral portions of the pons-midbrain (B and C) are demonstrated.