| Title | A severe neurological sequela in acute intermittent porphyria: presentation of a case from encephalopathy to quadriparesis. | | Author(s) | Gürses C, Durukan A, Sencer S, Akça S, Baykan B, Gökyiğit A | | Institution | Istanbul University Faculty of Medicine, Istanbul, Millet Caddesi Capa 34390, Istanbul, Turkey. candan.gurses@gmail.com | | Source | Br J Radiol 2008 May; 81(965):e135-40. | | Abstract | Porphyrias present themselves with autonomic or peripheral neuropathy or central nervous system dysfunction. They are a varied group of inborn errors of metabolism that are characterized by specific inherited enzyme defects in haem biosynthesis. A patient whose mother was diagnosed as having porphyria was admitted to hospital because of her abdominal pains and dysuria. She had three generalized convulsions and, in a few hours, she lost the vision in both of her eyes. As the seizures continued, she became quadriparetic and fell into a coma after gradually losing consciousness. She improved but with sequelae; her serial MRIs, including apparent diffusion coefficient map imaging, diffusion-weighted imaging and angiography, showed ischaemic lesions that were both unlike and more severe than the ones reported in the literature. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 18440935 |
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