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A severe neurological sequela in acute intermittent porphryria: presentation of a case from encephalopathy to quadriparesis

C GÜRSES, MD, A DURUKAN, MD, S SENCER, AKÇA, B BAYKAN and A GÖKYIIT

Istanbul University Faculty of Medicine, Istanbul, Millet Caddesi Çapa 34390, Istanbul Turkey

Correspondence: Candan Gürses, Istanbul University Faculty of Medicine, Millet Caddesi Çapa 34390 Istanbul Turkey. E-mail: candan.gurses{at}gmail.com

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.