• Neuroradiology · Dec 2001

    Case Reports

    MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome.

    • N Utz, B Kinkel, J P Hedde, and H Bewermeyer.
    • Department of Neurology, City Hospital of Merheim, Cologne, Germany. NUtz695191@aol.com
    • Neuroradiology. 2001 Dec 1; 43 (12): 1059-62.

    AbstractReversible 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.

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