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

  • Takata Tadayuki
    Department of Neurology, Kagawa University Hospital, Japan
  • Kume Kodai
    Department of General Internal Medicine, Kagawa University Hospital, Japan
  • Kokudo Yohei
    Department of Intractable Neurological Research, Kagawa University Faculty of Medicine, Japan
  • Ikeda Kazuyo
    Department of Neurology, Kagawa University Hospital, Japan
  • Kamada Masaki
    Department of Intractable Neurological Research, Kagawa University Faculty of Medicine, Japan
  • Touge Tetsuo
    Department of Health Sciences, Kagawa University Faculty of Medicine, Japan
  • Deguchi Kazushi
    Department of Neurology, Kagawa University Hospital, Japan
  • Masaki Tsutomu
    Department of Gastroenterology, Kagawa University Hospital, Japan

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Description

<p>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. </p>

Journal

  • Internal Medicine

    Internal Medicine 56 (6), 713-717, 2017

    The Japanese Society of Internal Medicine

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