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

Abstract

Brief Communications15 October 1995Transient Cortical Blindness and Bioccipital Brain Lesions in Two Patients with Acute Intermittent PorphyriaHugo 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, MDHugo Kupferschmidt, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Adriano Bont, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Hans Schnorf, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Theodor Landis, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Eike Walter, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Jurg Peter, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., Stephan Krahenbuhl, MD, PharmDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland., and Peter J. Meier, MDFrom University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-123-8-199510150-00006 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail 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 [2-5], for which deleterious vasospasm in both posterior cerebral arteries has been inferred [3, 6].We describe two patients in whom cortical blindness was the first symptom of acute intermittent porphyria. Magnetic resonance imaging showed extensive lesions involving primarily white matter of both occipital lobes consistent with vasospasm-induced ischemic lesions. However, the pathogenesis of the suspected vasospasm in ...References1. Bonkowsky HL, Schady W. Neurologic manifestations of acute porphyria. Semin Liv Dis. 1982; 2:108-24. Google Scholar2. Goldstein NP, Martin WJ, Brunsting LA, Kirby TJ. Blindness of cerebral origin in porphyria: report of a case. Mayo Clin Proc. 1957; 32:82-7. Google Scholar3. Lai CW, Hung TP, Lin WS. Blindness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination. Arch Neurol. 1977; 34:310-2. Google Scholar4. Sato M, Tomizaki Y. A case of transient cerebral blindness in acute intermittent porphyria. Folia Ophthalmol Jpn. 1992; 4:1378-81. Google Scholar5. King PH, Bragdon AC. MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent porphyria. Neurology. 1991; 41:1300-2. Google Scholar6. Hierons R. Changes in the nervous system in acute porphyria. Brain. 1957; 80:176-92. Google Scholar7. White KA, Marletta MA. Nitric oxide synthase is a cytochrome P-450 type hemoprotein. Biochemistry. 1992; 31:6627-31. Google Scholar8. Lowenstein CJ, Snyder SH. Nitric oxide, a novel biologic messenger. Cell. 1992; 70:705-7. Google Scholar9. Anton G. Ueber Herderkrankungen des Gehirns, welche vom Patienten selbst nicht wahrgenommen werden. Wiener Klin Wochenschr. 1898; 11:227-9. Google Scholar10. Riddoch G. Dissociation of visual perceptions due to occipital injuries, with special reference to appreciation of movement. Brain 1917; 40:15-57. Google Scholar11. Woods RP, Iacoboni M, Mazziotta JC. Brief report: bilateral spreading cerebral hypoperfusion during spontaneous migraine headache. N Engl J Med 1994; 331:1689-92. Google Scholar12. Kuhn MJ, Shekar PC. A comparative study of magnetic resonance imaging and computed tomography in the evaluation of migraine. Comput Med Imaging Graph. 1990; 14:149-52. Google Scholar Author, Article, and Disclosure InformationAuthors: Hugo Kupferschmidt, MD; Adriano Bont, MD; Hans Schnorf, MD; Theodor Landis, MD; Eike Walter, MD; Jurg Peter, MD; Stephan Krahenbuhl, MD, PharmD; Peter J. Meier, MDAffiliations: From University Hospital, Zurich; University Hospital, Basel; and University Hospital, Geneva, Switzerland.Corresponding Author: 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. 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A population-based studyCould attacks of abdominal pain in cases of acute intermittent porphyria be due to intestinal angina?Síndrome de leucoencefalopatía posterior reversibleVisual disturbances and seizures associated with pregnancy: a diagnostic dilemma and the role of radiological techniques as an aid to diagnosisA Reversible Posterior Leukoencephalopathy Syndrome 15 October 1995Volume 123, Issue 8Page: 598-600KeywordsBlindnessEpileptic seizuresLesionsMagnetic resonance imagingNeuropathyNitric oxidePeripheral neuropathyPorphyriaVasoconstrictionVision ePublished: 15 August 2000 Issue Published: 15 October 1995 Copyright & PermissionsCopyright © 1995 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...