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Letters |1 September 1996

Cortical Brain Lesions in Acute Intermittent Porphyria

Gordon Sze, MD

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  • Yale University School of Medicine, New Haven, CT 06520
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TO THE EDITOR:
Kupferschmidt and colleagues [1] described two patients with acute intermittent porphyria who presented with cortical blindness. Magnetic resonance imaging showed bilateral occipital lesions, and the authors speculated that these lesions were caused by vasospasm-induced ischemia due to unopposed cerebral vasoconstriction resulting from a deficiency of nitrous oxide synthase, a major vascular dilator. The striking feature of the magnetic resonance findings in these cases and in those of acute intermittent porphyria described in the literature is that the lesions are bioccipital and partially or totally reversible [2]. These characteristics are typical of magnetic resonance findings seen in patients with hypertensive encephalopathy. It is also notable that vasospasm alone, often a sequela of subarachnoid hemorrhage, does not result in this clinical appearance.
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Sze G. Cortical Brain Lesions in Acute Intermittent Porphyria. Ann Intern Med. ;125:422–423. doi: 10.7326/0003-4819-125-5-199609010-00023

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Published: Ann Intern Med. 1996;125(5):422-423.

DOI: 10.7326/0003-4819-125-5-199609010-00023

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Print ISSN: 0003-4819 | Online ISSN: 1539-3704

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