Motor polyneuropathy might be the presenting feature of acute intermittent porphyria

Akut intermittent porfiri motor polinöropati kliniği ile başlayabilir.


Abstract views: 38 / PDF downloads: 35 / PDF downloads: 7

Authors

  • Yasemin Ekmekyapar Fırat Department of Neurology, Gaziantep University School of Medicine
  • Mehmet Ali Elçi Division of Neurology, Turkish Ministry of Health Hatay Kırıkhan State Hospital
  • Sırma Geyik Department of Neurology, Gaziantep University School of Medicine
  • Ayşe Münife Neyal Department of Neurology, Gaziantep University School of Medicine

DOI:

https://doi.org/10.5152/EurJTher.2017.03034

Keywords:

Akut intermitant porfiri, motor polinöropati, ilerleyici kuadriparezi, periferik sinir sistemi

Abstract

Acute intermittent porphyria (AIP) is a rare metabolic disorder that is the most common of the acute porphyries. Peripheral neuropathy occurs in 10%–40% of patients during an acute attack. A 25-year-old female presented with progressive quadriparesis for the last two weeks. Appendectomy and cholecystectomy operations were recorded in her past medical history because of abdominal pain attacks. She had acute motor polyneuropathy electroneuromyographic findings. Fluctuations in her liver function tests, tachycardia, high blood pressure, and hyponatremia were observed when she was staying in the hospital. She had a 24-hour urine porphobilinogen value of 48.4 mg, and a high-calorie diet with general nutritional support was started. During follow-up in the hospital, the patient’s clinical symptoms improved gradually. AIP should be kept in mind in cases presenting with motor
polyneuropathy even if the diagnosis was not done previously.

Metrics

Metrics Loading ...

References

Lin CS, Park SB, Krishnan AV. Porphyric neuropathy. Handb Clin Neurol 2013; 115: 613-27.

Solis C, Martinez-Bermejo A, Naidich TP, Kaufmann WE, Astrin KH, Bishop DF, et al. Acute intermittent porphyria: studies of the severe homozygous dominant disease provides insights into the neurologic attacks in acute porphyrias. Arch Neurol 2004; 61: 1764-70.

Lin CS, Krishnan AV, Lee MJ, Zagami AS, You HL, Yang CC, et al. Nerve function and dysfunction in acute intermittent porphyria. Brain 2008; 131: 2510-9.

Hift RJ, Meissner PN. An analysis of 112 acute porphyric attacks in Cape Town, South Africa: Evidence that acute intermittent porphyria and variegate porphyria differ in susceptibility and severity. Medicine (Baltimore) 2005; 84: 48-60.

Foran SE, Abel G. Guide to porphyrias. A historical and clinical perspective. Am J Clin Pathol 2003; 119 Suppl: S86-93.

Gross U, Sassa S, Jacob K, Deybach JC, Nordmann Y, Frank M, et al. 5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up. Clin Chem 1998; 44: 1892-6.

Albers JW, Fink JK. Porphyric neuropathy. Muscle Nerve 2004; 30: 410-22.

Hervier B, Rimbert M, Colonna F, Hamidou MA, Audrain M. Clinical significance of anti-Ro/SSA-52 kDa antibodies: a retrospective monocentric study. Rheumatology (Oxford) 2009; 48: 964-7.

Allard SA, Charles PJ, Herrick AL, McColl KE, Scott JT. Antinuclear antibodies and the diagnosis of systemic lupus erythematosus in patients with acute intermittent porphyria. Ann Rheum Dis 1990; 49: 246-8.

Anderson KE, Bloomer JR, Bonkovsky HL, Kushner JP, Pierach CA, Pimstone NR, et al. Recommendations for the diagnosis and treatment of the acuteporphyrias. Ann Intern Med 2005; 142: 439-50.

Michaels BD, Del Rosso JQ, Mobini N, Michaels JR. Erythropoietic protoporphyria: a case report and literature review. J Clin Aesthet Dermatol 2010; 3: 44-8.

Downloads

Published

2023-04-27

How to Cite

Ekmekyapar Fırat, Y., Elçi, M. A., Geyik, S., & Neyal, A. M. (2023). Motor polyneuropathy might be the presenting feature of acute intermittent porphyria: Akut intermittent porfiri motor polinöropati kliniği ile başlayabilir. European Journal of Therapeutics, 23(2), 87–90. https://doi.org/10.5152/EurJTher.2017.03034

Issue

Section

Case Reports