Reversible Cerebral Vasospasm in Acute Intermittent Porphyria: A Case Report and Review of the Literature

  • Attout Hassene Internal Medicine Unit, General Hospital Les Cevennes, Alés, France
  • Stephane Guez Internal Medicine Unit, Pellegrin Teaching Hospital, Bordeaux, France

Keywords

Porphyria, cerebral infarction, vasospasm

Abstract

The porphyrias are rare inherited diseases of heme biosynthesis which can involve the nervous system. The most common neurological manifestations of acute intermittent porphyria are autonomic visceral neuropathy, peripheral motor neuropathy, and central nervous system dysfunction. In rare cases, patients with acute intermittent porphyria have presented with cerebral infarction, suggested to be due to vasospasm in cerebral arteries. We report a case of reversible vasospasm in porphyric encephalopathy demonstrated by both magnetic resonance and conventional angiography. Unexplained abdominal pain occurred before the onset of neurological symptoms.

VIEW THE ENTIRE ARTICLE

References

  • Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ 2000;320:1647–1651.
  • King PH, Bragdon AC. MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent porphyria. Neurology 1991;41:1300–1302.
  • Aggarwal A, Quint DJ, Lynch JP. MR imaging of porphyric encephalopathy. Am J Roentgenol 1994;162:1218–1220.
  • Kuperschmidt H, Bont A, Schnorf H, Landis T, Walter E, Peter J, et al. Transient cortical blindness and bioccipital brain lesions in two patients with acute intermittent porphyria. Ann Intern Med 1995;123:598–600.
  • Black K, Mirsky P, Kalina P, Woldenberg R, Drehobl KE, et al. Angiographic demonstration of reversible cerebral vasospasm in porphyric encephalopathy. AJNR 1995;16:1650–1652.
  • Lithner F. Could attacks of abdominal pain in cases of acute intermittent porphyria be due to intestinal angina? J Intern Med 2000;247(3):407–409.
  • Olivier P, Van Melkebeke D, Honore PJ, Defrayne L, Hemelsoet D. Cerebral vasospasm in porphyria. Eur J Neurol 2017;24(9):1183–1187.
  • Meyer UA, Schuurmans MM, Lindberg RL.Acute porphyrias: pathogenesis of neurological manifestations. Semin Liver Dis 1998;18(1):43-52.
  • Published: 2022-09-12

    Issue: 2022: LATEST ONLINE (view)

    Section: Articles

    How to cite:
    1.
    Hassene A, Guez S. Reversible Cerebral Vasospasm in Acute Intermittent Porphyria: A Case Report and Review of the Literature. EJCRIM 2022;doi:10.12890/2022_003543.

    Most read articles by the same author(s)