title: MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome.
abstract: Reversible posterior leukoencephalopathy syndrome (PLS) is characterized by headache, altered mental function, visual disturbances and seizures. Neuroimaging studies suggest a white-matter oedema, predominantly in the posterior parietal-temporal-occipital regions of the brain. We present the case of a 30-year-old woman who had suffered her first attack of acute intermittent porphyria (AIP). Following 1 week of abdominal pain she developed several generalized seizures, and hallucinations, and exhibited a progressive deterioration of the consciousness. T2-weighted images, especially fluid-attenuated inversion recovery (FLAIR) sequences showed bilateral lesions in the posterior frontal, parietal and occipital cortex and subcortical white matter. Following treatment with haematin and a high carbohydrate diet the patient's condition improved. Follow-up magnetic resonance imaging (MRI) revealed complete resolution of the lesions. To our knowledge, this is the first report concerning a completely reversible PLS in AIP.
authors.affiliation: Department of Neurology, City Hospital of Merheim, Cologne, Germany. NUtz695191@aol.com
authors.forename: N
authors.initials: N
authors.lastname: Utz
authors.forename: B
authors.initials: B
authors.lastname: Kinkel
authors.forename: J P
authors.initials: JP
authors.lastname: Hedde
authors.forename: H
authors.initials: H
authors.lastname: Bewermeyer
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology*; D001929:Brain Edema / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D009569:Nitric Oxide / Q000378:metabolism; D017118:Porphyria, Acute Intermittent / Q000150:complications / Q000175:diagnosis*; D012640:Seizures / Q000209:etiology; D013577:Syndrome
chemical_list: D009569:Nitric Oxide