Document Detail


Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?
MedLine Citation:
PMID:  15469454     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
K Engelhardt; E Trinka; G Franz; I Unterberger; M Spiegel; R Beer; B Pfausler; A Kampfl; E Schmutzhard
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of neurology : the official journal of the European Federation of Neurological Societies     Volume:  11     ISSN:  1351-5101     ISO Abbreviation:  Eur. J. Neurol.     Publication Date:  2004 Oct 
Date Detail:
Created Date:  2004-10-07     Completed Date:  2004-12-20     Revised Date:  2006-09-05    
Medline Journal Info:
Nlm Unique ID:  9506311     Medline TA:  Eur J Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  693-7     Citation Subset:  IM    
Affiliation:
Department of Neurology, University Hospital Innsbruck, Austria. klaus.engelhardt@uibk.ac.at
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MeSH Terms
Descriptor/Qualifier:
Abortion, Induced / methods*
Adult
Arginine / therapeutic use
Brain Mapping
Diffusion Magnetic Resonance Imaging / methods
Electroencephalography / methods
Female
Frontal Lobe / metabolism,  pathology,  physiopathology
Heme / therapeutic use
Humans
Porphyrias, Hepatic / complications*,  therapy
Porphyrins / urine
Pregnancy*
Status Epilepticus / etiology*,  therapy
Time Factors
Chemical
Reg. No./Substance:
0/Porphyrins; 100438-92-4/heme arginate; 14875-96-8/Heme; 74-79-3/Arginine
Comments/Corrections
Comment In:
Eur J Neurol. 2006 Jun;13(6):668-9   [PMID:  16796597 ]

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