Acute intermittent porphyria leading to posterior reversible encephalopathy syndrome (PRES): a rare cause of abdominal pain and seizures.
Acute intermittent porphyria (AIP) is an inherited deficiency in the haem biosynthesis pathway. AIP is rare, affecting around 1 in 75 000 people. Acute attacks are characterised by abdominal pain associated with autonomic, neurological and psychiatric symptoms. AIP is rarely associated with posterior reversible encephalopathy syndrome (PRES). PRES is a clinicoradiological condition caused by the failure of the posterior circulation to autoregulate, resulting in cerebral oedema, headaches, nausea and seizures. This association is important because drugs used in the management of seizures may worsen an attack of AIP. This article describes a case of AIP and PRES in a young woman.
Department of Acute Medicine, Chelsea and Westminster Hospital NHS Foundation Trust, London, UK.
Andrew
A
Dagens
Department of Acute Medicine, Moorfields Eye Hospital, London, UK.
Michael James
http://orcid.org/0000-0003-4109-5336
MJ
Gilhooley
D015746:Abdominal Pain / Q000209:etiology*; D000328:Adult; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D017118:Porphyria, Acute Intermittent / Q000150:complications / Q000175:diagnosis*; D011166:Porphyrins / Q000652:urine; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D012640:Seizures / Q000209:etiology*
D011166:Porphyrins