Endocrine Abstracts

Posterior reversible encephalopathy syndrome (PRES) is a cliniconeuroradiological entity characterized by seizures (91.7%), headache (83.3%), visual disturbance (62.5%),encephalophathy (29.2%), and paralysis (8.3%). Co-morbidities included systemic lupus erythematous (29.2%), kidney disease (20.8%), eclampsia(20.8%), renal artery stenosis (12.5%), Takayasu arteritis (4.2%), Sheehan's syndrome(4.2%), and acute intermittent porphyria (AIP) (4.2%). We report a 31-year-old woman who had typical manifestations of AIP, including abdominal pain, tachycardia, hypertension, mental disturbances, and excessive excretion of porphyrin precursors in urine, over the last 10 years. She is currently awaiting liver transplant due to severe frequent repeated symptoms and failure to respond to other treatments. During recent admission, following 2 weeks of abdominal pain, she developed sudden visual loss preceding several generalized seizures, and exhibited a progressive deterioration of the consciousness. She was intubated and transferred to the intensive care unit. However, her symptoms reversed in the next 24 hours. T2-weighted MRI revealed increased signal in most of the gyri from the frontal lobes extending posteriorly to occipital lobes consistent with encephalopathy, which were no longer visible 2 weeks later. PRES, which has been found in patients' MRI during an acute attack of AIP with severe encephalopathy, could explain the pathogenesis of encephalopathy and seizures in AIP. To our knowledge, this is the fifth report concerning a reversible PRES in AIP.

Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.