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Transient Cortical Blindness and Bioccipital Brain Lesions in Two Patients with Acute Intermittent Porphyria

Hugo Kupferschmidt, MD; Adriano Bont, MD; Hans Schnorf, MD; Theodor Landis, MD; Eike Walter, MD; Jurg Peter, MD; Stephan Krahenbuhl, MD, PharmD; and Peter J. Meier, MD
[+] Article and Author Information

From University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland. Requests for Reprints: Peter J. Meier, MD, Division of Clinical Pharmacology and Toxicology, Department of Medicine, University Hospital CH-8091, Zurich, Switzerland. Current Author Addresses: Drs. Kupferschmidt, Krahenbuhl, and Meier: Division of Clinical Pharmacology and Toxicology, Department of Medicine, University Hospital, CH-8091, Zurich, Switzerland.


Copyright ©2004 by the American College of Physicians


Ann Intern Med. 1995;123(8):598-600. doi:10.7326/0003-4819-123-8-199510150-00006
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The most common neurologic manifestations of acute intermittent porphyria are autonomic visceral neuropathy, peripheral motor neuropathy, and central nervous system dysfunctions, including seizures and neuropsychiatric disturbances [1]. In rare instances, however, patients with acute intermittent porphyria have presented with acute cortical blindness [25], for which deleterious vasospasm in both posterior cerebral arteries has been inferred [3, 6].

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Figure 1.
T2-weighted axial magnetic resonance images in Patient 1.

Large confluent areas of signal hyperdensities are present in the occipital cortex, including the calcarine cortex, and in the adjacent white matter.

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Figure 2.
T2-weighted axial magnetic resonance images in Patient 2.1right panel

Patchy hyperdensities are present in both occipital lobes and in the precentral and frontal subcortical white matter. Additionally, right paracentral white matter hyperdensities are present (left and middle panels). T -weighted coronal sections show gadolinium enhancement along cortical gyri and sulci in the occipital paracentral cortex. Slight contrast enhancement of the left calcarine cortex is also present ( ).

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