MRI gadolinium enhancement precedes neuroradiological findings in acute necrotizing encephalopathy

• DOI: 10.1016/j.braindev.2012.11.011
• Publication Date: 2012-12-21T00:24:13Z
• Authors: Takeshi Yoshida; Takuya Tamura; Yuhki Nagai; Hiroyuki Ueda; Tomonari Awaya; Minoru Shibata; Takeo Kato; Toshio Heike
• Keywords: Male; Herpesvirus 6, Human; Brain; Contrast Media; Gadolinium; Magnetic Resonance Imaging; 3. Good health; Leukoencephalitis, Acute Hemorrhagic; Radiography; 03 medical and health sciences; 0302 clinical medicine; Asian People; Child, Preschool; Humans; Encephalitis, Viral

We report a 2-year-old Japanese boy with acute necrotizing encephalopathy (ANE) triggered by human herpes virus-6, who presented insightful magnetic resonance imaging (MRI) findings. He was admitted due to impaired consciousness and a convulsion, 2 days after the onset of an upper respiratory infection. At admission, cranial MRI showed marked gadolinium enhancement at the bilateral thalami, brainstem and periventricular white matter without abnormal findings in noncontrast MRI sequences. On the following day, noncontrast computed tomography demonstrated homogeneous low-density lesions in the bilateral thalami and severe diffuse brain edema. The patient progressively deteriorated and died on the 18th day of admission. The pathogenesis of ANE remains mostly unknown, but it has been suggested that hypercytokinemia may play a major role. Overproduced cytokines cause vascular endothelial damage and alter the permeability of the vessel wall in the multiple organs, including the brain. The MRI findings in our case demonstrate that blood-brain barrier permeability was altered prior to the appearance of typical neuroradiological findings. This suggests that alteration of blood-brain barrier permeability is the first step in the development of the brain lesions in ANE, and supports the proposed mechanism whereby hypercytokinemia causes necrotic brain lesions. This is the first report demonstrating MRI gadolinium enhancement antecedent to typical neuroradiological findings in ANE.