Treatment and outcome of children with acute encephalopathy depend on the cause, prompt treatment of the underlying cause, and use of adequate supportive measures. Many novel causes of acute encephalopathy are emerging where lumbar puncture, computed tomography of the head, and routine biochemical testing can be normal such as acute disseminated encephalomyelitis and febrile infection-related refractory epilepsy syndrome. Magnetic resonance imaging (MRI) plays an important role in the workup of children with acute leukoencephalopathy. Despite this in few cases, a correct diagnosis is not possible and novel conditions have been described in the last decade. One such condition is acute encephalopathy with biphasic seizures and restricted diffusion also called as acute leukoencephalopathy with restricted diffusion. Here, the routine MRI sequences such as T1, T2, and fluid-attenuated inversion recovery sequences can be normal. Here, we have reviewed the etiology, types, clinicoradiological features, and treatment of this condition.
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