Acute encephalopathy with biphasic seizures and late reduced diffusion

Abstract

Rationale: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) has been reported almost exclusively in the Japanese population. Patient concerns: A 17-month-old male patient presented with fever and seizures, and subsequently fell into a coma. On the second day, he recovered consciousness. On the fourth day, he developed complex partial seizures and fell into a coma again. On day 10, the fever and seizures subsided. Head computed tomography on the first day showed no abnormalities. Brain diffusion-weighted images on the fourth day revealed reduced diffusion in the bilateral subcortical white matter. Diagnosis: A diagnosis of AESD was made. Interventions: The patient was treated with corticosteroids and intravenous immunoglobulin. Outcomes: At the 4-month follow-up, the patient was able to walk independently, and the epileptic seizures were well controlled. Lessons: AESD is a rare entity, and treatment with corticosteroids and intravenous immunoglobulin can lead to a favorable prognosis. Clinicians should be aware of this condition, and clinicoradiological features can suggest the diagnosis.

Acute encephalopathy with biphasic seizures and late reduced diffusion with concurrent transverse myelitis

“…Spine MRI is a reasonable investigation in patients with AESD, as signs and symptoms of transverse myelitis could be easily missed. Steroid treatment may have had some positive impact on our patient's outcome, and others have advocated to consider the use of additional immunomodulatory interventions such as intravenous immunoglobulins and tocilizumab 10,11 . There remains no strong evidence supporting such treatment in patients with AESD; however, potential benefits may outweigh the risks in severely affected patients.…”