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

被引:20
作者
Engelhardt, K [1 ]
Trinka, E [1 ]
Franz, G [1 ]
Unterberger, I [1 ]
Spiegel, M [1 ]
Beer, R [1 ]
Pfausler, B [1 ]
Kampfl, A [1 ]
Schmutzhard, E [1 ]
机构
[1] Univ Innsbruck Hosp, Dept Neurol, A-6020 Innsbruck, Austria
关键词
abortion; acute hepatic porphyria; haem arginate; pregnancy; status epilepticus;
D O I
10.1111/j.1468-1331.2004.00876.x
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:693 / 697
页数:5
相关论文
共 14 条
[1]   PREGNANCY AND ACUTE PORPHYRIAS [J].
BRODIE, MJ ;
MOORE, MR ;
THOMPSON, GG ;
GOLDBERG, A ;
LOW, RAL .
BRITISH JOURNAL OF OBSTETRICS AND GYNAECOLOGY, 1977, 84 (10) :726-731
[2]   Epidemiology and clinical characteristics of seizures in patients with acute intermittent porphyria [J].
Bylesjo, I ;
Forsgren, L ;
Lithner, F ;
Boman, K .
EPILEPSIA, 1996, 37 (03) :230-235
[3]   The acute porphyrias [J].
Elder, GH ;
Hift, RJ ;
Meissner, PN .
LANCET, 1997, 349 (9065) :1613-1617
[4]   Oxcarbazepine in focal epilepsy and hepatic porphyria: A case report [J].
Gaida-Hommernick, B ;
Rieck, K ;
Runge, U .
EPILEPSIA, 2001, 42 (06) :793-795
[5]   The acute porphyrias [J].
Gordon, N .
BRAIN & DEVELOPMENT, 1999, 21 (06) :373-377
[6]  
Kappas A., 1995, METABOLIC MOL BASES, P2103
[7]   TREATMENT OF THE PORPHYRIAS [J].
KAUPPINEN, R ;
TIMONEN, K ;
MUSTAJOKI, P .
ANNALS OF MEDICINE, 1994, 26 (01) :31-38
[8]  
Keung Y K, 2000, J Am Board Fam Pract, V13, P76
[9]   MRI REVEALS MULTIPLE REVERSIBLE CEREBRAL-LESIONS IN AN ATTACK OF ACUTE INTERMITTENT PORPHYRIA [J].
KING, PH ;
BRAGDON, AC .
NEUROLOGY, 1991, 41 (08) :1300-1302
[10]   MANAGEMENT OF ATTACKS OF ACUTE PORPHYRIA [J].
LAIWAH, ACY ;
MCCOLL, KEL .
DRUGS, 1987, 34 (05) :604-616