• Internal medicine · Jan 2017

    Case Reports

    Acute Intermittent Porphyria Presenting with Posterior Reversible Encephalopathy Syndrome, Accompanied by Prolonged Vasoconstriction.

    • Tadayuki Takata, Kodai Kume, Yohei Kokudo, Kazuyo Ikeda, Masaki Kamada, Tetsuo Touge, Kazushi Deguchi, and Tsutomu Masaki.
    • Department of Neurology, Kagawa University Hospital, Japan.
    • Intern. Med. 2017 Jan 1; 56 (6): 713-717.

    AbstractA 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.

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