Ohya Takashi, Yamashita Yushiro, Shibuya Ikuhiko, Hara Munetsugu, Nagamitsu Shinichiro, Kaida Hayato, Kurata Seiji, Ishibashi Masatoshi, Matsuishi Toyojiro
Department of Pediatrics and Child Health, Kurume University School of Medicine, Japan.
Department of Pediatrics and Child Health, Kurume University School of Medicine, Japan.
Eur J Paediatr Neurol. 2014 Jul;18(4):536-9. doi: 10.1016/j.ejpn.2014.03.001. Epub 2014 Mar 12.
We describe a 15-year-old girl with subacute sclerosing panencephalitis (SSPE) in stage II who was treated with isoprinosine, intraventricular interferon alpha (IFN-α), and ribavirin for 3 years. She is alive at three years from onset and studies at school with the assistance of a special educational teacher. To assess residual brain function, serial (18)FDG-positron emission tomography (PET) was performed three times to measure cortical metabolism: at onset, a year later, and three years later. At onset, PET study revealed preserved glucose metabolism of the cerebral cortex. In serial PET study, glucose metabolism of the cerebral cortex was also preserved even after three years. Although SSPE is a progressive disease of the neuronal system, and typically leads to death in approximately 2-3 years, the neurological prognosis of our case was good. We consider that combination therapy in the very early stage without hypometabolism in the cerebral cortex may be effective for SSPE.
我们描述了一名15岁处于亚急性硬化性全脑炎(SSPE)II期的女孩,她接受了3年的异丙肌苷、脑室内注射干扰素α(IFN-α)和利巴韦林治疗。自发病起三年后她仍然存活,并在一名特殊教育教师的协助下在学校学习。为评估残留脑功能,连续三次进行(18)氟代脱氧葡萄糖正电子发射断层扫描(PET)以测量皮质代谢:发病时、一年后和三年后。发病时,PET研究显示大脑皮质葡萄糖代谢保持正常。在连续的PET研究中,即使三年后大脑皮质的葡萄糖代谢也保持正常。尽管SSPE是一种神经系统的进行性疾病,通常在大约2至3年内导致死亡,但我们这个病例的神经学预后良好。我们认为在大脑皮质无代谢减低的极早期进行联合治疗可能对SSPE有效。