Abstract
The precise clinical characteristics of acute encephalopathy with bilateral reduced diffusion are not fully understood. We compared clinical, laboratory, and neuroimaging findings according to the patterns of brain lesions among children with reduced diffusion in the bilateral hemispheres. Nine patients were analyzed. The patterns of brain lesions were divided into diffuse lesions and central-sparing lesions. Diffuse lesions were defined as reduced diffusion in the whole cortex and/or subcortical white matter. Central-sparing lesions were defined as the lack of reduced diffusion in the areas around the bilateral Sylvian fissures. Clinical, laboratory, and neuroimaging findings were compared between groups. Five patients showed diffuse lesions and 4 showed central-sparing lesions. Coma was significantly more common in patients with diffuse lesions, whereas a biphasic clinical course was more common in those with central-sparing lesions. Outcome was worse in patients with diffuse lesions. Maximal aspartate aminotransferase, alanine aminotransferase, and kinase levels were also significantly higher in patients with diffuse lesions. In 2 patients with diffuse lesions, diffusion-weighted images during the acute phase revealed reduced diffusion in the bilateral frontal and occipital areas, followed by diffuse lesions. No patient with central-sparing lesions showed MR imaging abnormalities during the acute phase. Clinical manifestations in patients with diffuse lesions were severe, whereas those in patients with central-sparing lesions were relatively mild.
Highlights
AND PURPOSE: The precise clinical characteristics of acute encephalopathy with bilateral reduced diffusion are not fully understood
Acute encephalopathy in association with infectious disease has attracted the attention of pediatricians and pediatric neurologists in Japan since the outbreak of influenza-associated encephalopathy during the 1997/1998 winter season
It is estimated that hundreds of Japanese children die or experience neurologic sequelae due to acute encephalopathy of infectious causes, which has prompted studies on acute encephalopathy in Japan
Summary
Central-sparing lesions were defined as the lack of reduced diffusion in the areas around the bilateral Sylvian fissures. Acute encephalopathy was defined as a condition characterized by decreased consciousness with or without other neurologic symptoms, lasting for Ͼ24 hours in children with infectious symptoms, such as fever, cough, and diarrhea. We carefully excluded patients with sustained decreased consciousness after a febrile seizure, and those with delirious behavior without obviously reduced consciousness. We excluded patients who were clinically diagnosed with status epilepticus by the attending physician. Coma was defined as a condition in which the patient was not arousable with maximal painful stimulation, which is consistent with a score of 3–5 on the Glasgow Coma Scale, modified for children, or a score of 100 –300 on the Japan Coma Scale
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