Acute Necrotizing Encephalopathy
ANE is a rare brain disorder primarily affecting children, characterized by multiple symmetric brain lesions--particularly in the thalami--and rapid neurologic deterioration, including changes in consciousness, seizures, and coma. Patients diagnosed with ANE often have a recent history of viral infection, including influenza and SARS-CoV-2.
Neuroimaging of patients indicates symmetric, bilateral lesions in gray matter, often seen in the thalami, along with other abnormalities, including edema and inflammation.
While the pathogenesis of the disease is unknown, there is a possible genetic component involving mutations associated with cytokine storm and immune dysregulation.
The condition is thought to be fatal in about one-third of patients, and survivors often have permanent brain damage due to their illness. The best treatment for patients with ANE is still under investigation, but management typically involves modulating immune responses, including anti-cytokine therapies (e.g., tocilizumab), corticosteroids, and plasmapheresis.
