Literature DB >> 15469454

Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?

K Engelhardt1, E Trinka, G Franz, I Unterberger, M Spiegel, R Beer, B Pfausler, A Kampfl, E Schmutzhard.   

Abstract

A 22-years old, 55 kg female patient in the twelfth week of pregnancy developed neuropsychiatric syndromes and in the following status epilepticus. Raised porphyrines and porphyrine precursors were found in the patient's urine. Despite intravenous glucose infusions and appropriate medication no reduction in seizure-frequency and neuropsychiatric syndromes was observed. An abortion was induced. After the interruption and starting of haem arginate therapy, seizure activity stopped and porphyrine precursors returned to normal levels, and after 6 weeks the patient was discharged in excellent clinical condition. This report describes a status epilepticus caused by acute hepatic porphyria, triggered by pregnancy, in a 22-years old woman. To our knowledge this is the first report of induced abortion as successful treatment in acute hepatic porphyria induced status epilepticus.

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Year:  2004        PMID: 15469454     DOI: 10.1111/j.1468-1331.2004.00876.x

Source DB:  PubMed          Journal:  Eur J Neurol        ISSN: 1351-5101            Impact factor:   6.089


  6 in total

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Journal:  J Clin Neurophysiol       Date:  2013-10       Impact factor: 2.177

2.  Treatment of acute intermittent porphyria during pregnancy and posterior reversible encephalopathy syndrome after delivery: A case report.

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Journal:  Exp Ther Med       Date:  2017-09-27       Impact factor: 2.447

3.  Is screening for urinary porphobilinogen useful among patients with acute polyneuropathy or encephalopathy?

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Journal:  J Neurol       Date:  2008-06-27       Impact factor: 4.849

4.  Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study.

Authors:  Mette Christophersen Tollånes; Aasne Karine Aarsand; Sverre Sandberg
Journal:  J Inherit Metab Dis       Date:  2010-10-27       Impact factor: 4.982

Review 5.  Acute porphyrias - A neurological perspective.

Authors:  Lea M Gerischer; Franziska Scheibe; Astrid Nümann; Martin Köhnlein; Ulrich Stölzel; Andreas Meisel
Journal:  Brain Behav       Date:  2021-10-17       Impact factor: 2.708

6.  Acute porphyria presenting as epilepsia partialis continua.

Authors:  Thi Phuoc Yen Tran; Karine Leduc; Martin Savard; Nicolas Dupré; Donald Rivest; Dang Khoa Nguyen
Journal:  Case Rep Neurol       Date:  2013-06-29
  6 in total

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