Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion (AESD): Case Report and Review of the Literature
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Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a recently established subtype of encephalopathy syndrome. AESD is characterized by febrile status epilepticus on the first day, followed by transient recovery of consciousness. A secondary cluster of complex seizures follows on day three to six, and reduced diffusion in the subcortical white matter can be observed by magnetic resonance imaging (MRI). Affected children have neurological sequelae of varying severity. The exact pathogenesis of AESD is unclear and clinicians often empirically manage patients with steroid pulse therapy and intravenous immunoglobulin. We herein report a case of a 13-month-old girl who was transferred to our hospital because of a secondary episode of afebrile seizures five days after a brief febrile convulsion. On day seven, diffusion-weighted MRI and the calculated apparent diffusion coefficient map indicated restricted diffusion in the subcortical white matter of both frontal lobes, the posterior cingulate gyrus, and the genu of the corpus callosum. Because the patient had no neurologic abnormalities after the second cluster of seizures, she underwent close observation with no medical treatment. She recovered completely without neurological sequelae and follow-up MRI on day 36 showed resolution of the restricted diffusion.
Abstract
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a recently established subtype of encephalopathy syndrome. AESD is characterized by febrile status epilepticus on the first day, followed by transient recovery of consciousness. A secondary cluster of complex seizures follows on day three to six, and reduced diffusion in the subcortical white matter can be observed by magnetic resonance imaging (MRI). Affected children have neurological sequelae of varying severity. The exact pathogenesis of AESD is unclear and clinicians often empirically manage patients with steroid pulse therapy and intravenous immunoglobulin. We herein report a case of a 13-month-old girl who was transferred to our hospital because of a secondary episode of afebrile seizures five days after a brief febrile convulsion. On day seven, diffusion-weighted MRI and the calculated apparent diffusion coefficient map indicated restricted diffusion in the subcortical white matter of both frontal lobes, the posterior cingulate gyrus, and the genu of the corpus callosum. Because the patient had no neurologic abnormalities after the second cluster of seizures, she underwent close observation with no medical treatment. She recovered completely without neurological sequelae and follow-up MRI on day 36 showed resolution of the restricted diffusion.
참고문헌 (13)
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