Acute Intermittent Porphyria presenting with posterior reversible encephalopathy syndrome and lateralized periodic discharges plus fast activity on cEEG

We report a 20years-old patient with 6-months history of recurrent abdominal pain, and 3-days history of vomiting, hypertension, seizures and encephalopathy. The brain MRI showed posterior reversible encephalopathy syndrome and the cEEG monitoring showed lateralized periodic discharges plus fast activity. Comprehensive CSF studies were negative. Due to severe abdominal pain without definite etiology we requested urine porphobilinogen, and serum and fecal porphyrins, which were suggestive of acute intermittent porphyria (AIP). Patient had complete resolution of her symptoms with carbohydrate loading and highly caloric diet. AIP is potentially life threatening without proper management and prevention of triggers.
Source: Epilepsy and Behavior Case Reports - Category: Neurology Source Type: research