Takata Tadayuki, Kume Kodai, Kokudo Yohei, Ikeda Kazuyo, Kamada Masaki, Touge Tetsuo, Deguchi Kazushi, Masaki Tsutomu
Department of Neurology, Kagawa University Hospital, Japan.
Intern Med. 2017;56(6):713-717. doi: 10.2169/internalmedicine.56.7654. Epub 2017 Mar 17.
A 20-year-old Japanese woman had an attack of acute intermittent porphyria (AIP). Magnetic resonance imaging (MRI) revealed symmetrical lesions in the cerebrum and cerebellar hemisphere, corresponding to posterior reversible encephalopathy syndrome (PRES). Our administration of heme arginate gradually improved the clinical condition associated with AIP and the level of metabolite of nitric oxide (NO), which is a vascular dilator. Repeated MRI and magnetic resonance angiography revealed exacerbated PRES, part of which showed a small infarction, accompanied by progressive vasoconstriction. These findings suggest that the recovery of NO by heme replacement alone is insufficient for preventing brain damage during an AIP attack.
一名20岁的日本女性患了急性间歇性卟啉病（AIP）。磁共振成像（MRI）显示大脑和小脑半球有对称性病变，符合后部可逆性脑病综合征（PRES）。我们给予的血红素精氨酸逐渐改善了与AIP相关的临床状况以及作为血管扩张剂的一氧化氮（NO）的代谢物水平。重复进行的MRI和磁共振血管造影显示PRES加重，部分表现为小梗死，并伴有进行性血管收缩。这些发现表明，仅通过血红素替代来恢复NO不足以预防AIP发作期间的脑损伤。
