私共の研究室から小児期に発症する新しい急性脳症の概念として複数の脳症症候群(MES)が提唱されました。名称は東京女子医科大学八千代医療センターの高梨潤一教授より授かり、サイトカインプロファイルは東京都医学総合研究所の佐久間啓先生に解析いただきました。この脳症は、既存の急性壊死性脳症(ANE)、けいれん重積型二相性脳症(AESD)、脳梁膨大部脳症(MERS)、急性小脳炎(Cerebellitis)の4つの急性脳症を併発する小児期に発症する最も重篤な予後の小児急性脳症と考えられます。症例は当院ICUに収容され、軽度脳脳低温療法により救命され、その後の後遺症も日常生活が可能なまでに劇的な回復を得ました。

　＊小児急性脳症に対する軽度脳脳低温療法は、私が商標特許(登録番号5958525)を有する高度先進医療です。

　詳細はイタリアの総合医学誌European review for Medical and phermacological Sciences (IF 3.784: 2021)に掲載されました。https://www.europeanreview.org/article/29509　https://pubmed.ncbi.nlm.nih.gov/36066146/

Multiple encephalopathy syndrome: a case of a novel radiological subtype of acute encephalopathy in childhood

Y Fujita1, G Imataka1, H Sakuma2, J-I Takanashi3, S Yoshihara1

1 Department of Pediatrics, Dokkyo Medical University, Tochigi, Japan

2 Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan 

3 Department of Pediatrics, Tokyo Women’s Medical University Yachiyo Medical Center, Chiba, Japan 

Introduction: This report presents the case of a novel subtype of acute encephalopathy syndrome in childhood found in a patient with influenza type A infection; the patient exhibited evident magnetic resonance imaging (MRI) findings.

Case report: A 4-year-old boy was transferred to our hospital for prolonged (lasting 60 min) status epilepticus with influenza encephalopathy. Mild brain hypothermia therapy was applied for 72 h, followed by targeted temperature management for 96 h with mechanical ventilation in the intensive care unit. Moreover, methylprednisolone pulse therapy and immunoglobulin therapy were administered. One month after the treatment, his physical status recovered such that he was able to run, take food orally, communicate verbally, and successfully return to kindergarten. Interestingly, serial MRI studies revealed findings that were compatible with 1) acute necrotizing encephalopathy (ANE), 2) mild encephalitis/encephalopathy with a reversible splenial lesion (MERS type II), 3) acute cerebellitis, and 4) acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) on days 2, 4, 7, and 16, respectively.

Conclusions: To the best of our knowledge, these significant MRI findings associated with acute encephalopathy have never been reported. Thus, herein, we propose the new term radiological "multiple encephalopathy syndrome (MES)" based on our case of acute encephalopathy in childhood.