Acute Necrotizing Encephalopathy: Diffusion MR Imaging and Localized Proton MR Spectroscopic Findings in Two Infants

In this report, we describe the findings of diffusion MR imaging and proton MR spectroscopy in two infants with acute necrotizing encephalopathy in which there was characteristic symmetrical involvement of the thalami. Diffusion MR images of the lesions showed that the observed apparent diffusion coefficient (ADC) decrease was more prominent in the first patient, who had more severe brain damage and a poorer clinical outcome, than in the second. Proton MR spectroscopy detected an increase in the glutamate/glutamine complex and mobile lipids in the first case but only a small increase of lactate in the second. Diffusion MR imaging and proton MR spectroscopy may provide useful information not only for diagnosis but also for estimating the severity and clinical outcome of acute necrotizing encephalopathy.

Hemorrhagic Shock and Encephalopathy Syndrome: A Case Report

Hemorrhagic shock and encephalopathy syndrome (HSES) is a sudden-onset symptom complex that in-volves multisystem failure and includes encephalopathy, shock, coma, convulsions, prerenal azotemia, hepatic dysfunction, and bleeding coagulopathy and progressive thrombocytopenia in previously healthy infants and children. Its radiologic findings have rarely been reported, and it has not been described in Korea. We present a case of clinically diagnosed HSES, and include the CT and MRI findings.

Desmoplastic Infantile Ganglioglioma: A Case Report

Desmoplastic infantile ganglioglioma is an uncommon variety of ganglioglioma that shows evidence of glial andganglionic differentiation accompanied by an extreme desmoplastic reaction. A 16-month-old girl was ad-mitted witha six-day history of left hemiparesis. MR imaging demonstrated a large multiseptated cystic mass, with a solidportion, in the white matter of the right frontotemporoparietal lobe. After contrast injections, the solid portionwas clearly enhanced. The presence of desmoplastic infantile ganglioglioma was confirmed by surgical resection. Wedescribe the characteristic radiologic and pathologic features of desmoplastic infantile ganglioglioma, andinclude a a review of the literature.

Linear Sebaceous Nevus Syndrome with Hemimegalencephaly: A case report

Linear sebaceous nevus syndrome, which is the association of nevus sebaceous of Jadassohn in the midfacial area with seizure and mental deficiency, has been reported in at least 100 cases. Hemimegalencephaly is a rare malformation consisting of unilateral hypertrophy of the brain, an abnormal gyral pattern, thickened cortex, white matter abnormality, and lateral ventricular dilatation. The authors report a case of linear sebaceous nevus syndrome with hemimegalencephaly, diagnosed on the basis of clinical data and brain MRI.