Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?
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.
Department of Neurology, University Hospital Innsbruck, Austria. klaus.engelhardt@uibk.ac.at
K
K
Engelhardt
E
E
Trinka
G
G
Franz
I
I
Unterberger
M
M
Spiegel
R
R
Beer
B
B
Pfausler
A
A
Kampfl
E
E
Schmutzhard
D000028:Abortion, Induced / Q000379:methods*; D000328:Adult; D001120:Arginine / Q000627:therapeutic use; D001931:Brain Mapping; D038524:Diffusion Magnetic Resonance Imaging / Q000379:methods; D004569:Electroencephalography / Q000379:methods; D005260:Female; D005625:Frontal Lobe / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D006418:Heme / Q000627:therapeutic use; D006801:Humans; D017094:Porphyrias, Hepatic / Q000150:complications* / Q000628:therapy; D011166:Porphyrins / Q000652:urine; D011247:Pregnancy; D013226:Status Epilepticus / Q000209:etiology* / Q000628:therapy; D013997:Time Factors
D011166:Porphyrins; D006418:Heme; D001120:Arginine; C048849:heme arginate