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Two newly proposed infectious encephalitis/encephalopathy syndromes
1Department of Pediatrics, Kameda Medical Center, 929 Higashi-cho, Kamogawa-shi, Chiba 296-8602, Japan. jtaka@kameda.jp
Abstract:
Two newly proposed infectious encephalitis/encephalopathy syndromes, in which magnetic resonance imaging (MRI) is essential for the diagnosis, have been reviewed. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is reported only in East Asian infants, characterized by a febrile seizure (usually >30 min) as the initial neurological symptom on day 1, followed by secondary seizures at day 4 to 6; affected children display variable levels of neurological sequelae. MRI shows no acute abnormality during the first two days; reduced diffusion appears in the frontal or fronto-parietal subcortical white matter during days 3 to 9, then disappears between days 9 and 25. Excitotoxic injury with delayed neuronal death is hypothesized as a possible mechanism based on MR spectroscopic findings. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is characterized by a reversible lesion with homogeneously reduced diffusion in the corpus callosum (at least involving the splenium), sometimes associated with symmetrical white matter lesions. The most common neurological symptom is delirious behavior, followed by consciousness disturbance, and seizures, all of which completely recover within a month. The reason for the transiently reduced diffusion within the lesions is unknown; possibilities that have been postulated include intramyelinic edema, interstitial edema in tightly packed fibers, and a transient inflammatory infiltrate.
Insights
Two infant encephalopathy syndromes, Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion (AESD) and Clinically Mild Encephalitis/Encephalopathy with a Reversible Splenial Lesion (MERS), are reviewed. MRI is crucial for diagnosing these conditions, which present distinct clinical and imaging features.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Two novel syndromes of infectious encephalitis/encephalopathy require magnetic resonance imaging (MRI) for diagnosis.
- Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion (AESD) and Clinically Mild Encephalitis/Encephalopathy with a Reversible Splenial Lesion (MERS) are distinct neurological conditions.
Purpose of the Study:
- To review the diagnostic criteria and clinical characteristics of AESD and MERS.
- To highlight the essential role of MRI in differentiating these encephalopathy syndromes.
Main Methods:
- Review of existing literature on AESD and MERS.
- Analysis of clinical presentations and MRI findings associated with each syndrome.
Main Results:
- AESD, exclusive to East Asian infants, presents with biphasic seizures and characteristic white matter diffusion changes on MRI.
- MERS involves a reversible splenial lesion in the corpus callosum, often with transient diffusion restriction, and typically resolves within a month.
Conclusions:
- MRI is indispensable for the diagnosis and characterization of AESD and MERS.
- Understanding the unique imaging patterns and clinical courses of these syndromes aids in accurate diagnosis and management.
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