Figure 1.

Acute Necrotizing Encephalopathy (Local case). (Personal case of APN and JMW): Axial T-2 weighted MRI of a 9-year-old girl with who presented with classical clinical features of ANE and was admitted to the local paediatric intensive care unit. The MRI shows the classical symmetrical involvement of both thalami (with a target appearance) and symmetrical external capsular white matter affected. She had brainstem involvement (not shown) and was treated with intravenous methylprednisolone early. She survived with mild to moderate neurological sequelae. She was the first in her family to be genetically confirmed as positive for a RANBP2 mutation, with two of her cousins having been previously affected. The genetic result assisted with identification of at-risk family members, counseling and subsequent preventative measures including vaccination and early ANE ‘crisis’ management.