Acute Intermittent Porphyria Presenting with Posterior Reversible Encephalopathy Syndrome, Accompanied by Prolonged Vasoconstriction.
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.
Department of Neurology, Kagawa University Hospital, Japan.
Tadayuki
T
Takata
Kodai
K
Kume
Yohei
Y
Kokudo
Kazuyo
K
Ikeda
Masaki
M
Kamada
Tetsuo
T
Touge
Kazushi
K
Deguchi
Tsutomu
T
Masaki
D001120:Arginine / Q000627:therapeutic use; D005260:Female; D006418:Heme / Q000627:therapeutic use; D006801:Humans; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D017118:Porphyria, Acute Intermittent / Q000150:complications*; D054038:Posterior Leukoencephalopathy Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D055815:Young Adult
D006418:Heme; D001120:Arginine; C048849:heme arginate