MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome

Autor: B Kinkel, N Utz, H Bewermeyer, J P Hedde
Rok vydání: 2001
Předmět:
Zdroj: Neuroradiology. 43:1059-1062
ISSN: 1432-1920
0028-3940
DOI: 10.1007/s002340100616
Popis: 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.
Databáze: OpenAIRE