Journal of Womens Health, Issues and Care ISSN: 2325-9795

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Case Report, J Womens Health Issues Care Vol: 4 Issue: 6

Refractory Status Epilepticus in a Pregnant Woman Affected by Clivus Chordoma

Candelieri M1* , Maragno AM1, Galoppi P1, Masselli G2,Brunelli R1 and Perrone G1
1Department of Gynaecology, Obstetrics and Urological Sciences, University of Rome “Sapienza”, Italy
2Department of Radiology, University of Rome “Sapienza”, Italy
Corresponding author : Miriam Candelieri
Dipartimento di Ginecologia, Ostetricia e Scienze Urologiche, Via del Policlinico 155, 00161 Roma, Italy
Tel: +393295892958
E-mail: [email protected]
Received: June 24, 2015 Accepted: November 26, 2015 Published: November 28, 2015
Citation: Candelieri M , Maragno AM, Galoppi P, Masselli G, Brunelli R, et al. (2015) Refractory Status Epilepticus in a Pregnant Woman Affected By Clivus Chordoma. J Womens Health, Issues Care 4:6. doi:10.4172/2325-9795.1000213

Abstract

Status epilepticus (SE) is a rare but potentially life threatening complication that women with epilepsy may experience during pregnancy. Refractory status epilepticus (RSE) is defined as an epileptic status which is unresponsive to early standard antiepileptic medical treatments; it has been reported that in pregnant women affected by other diseases (systemic lupus erythematosus, porphyria, vitamin B6 deficiency and cavernous angioma) this status spontaneously resolved only after delivery. We report the case of a primigravida 29-year-old epileptic woman who was admitted to the Emergency Room at 25 weeks of gestation with RSE and a confused mental state. Her Electroencephalography documented repeated seizures not controlled by medical treatments, for this reason she was then sent to the Intensive Care Unit. The patient had no known risk factors. Her cranial magnetic resonance revealed a clivus chordoma, a rare, slow-growing neoplasm, characterized by locally aggressive growth patterns and high local recurrence rates. Spontaneous premature delivery at 26 weeks and three days brought an almost immediate cessation of seizures.

Keywords:

Keywords

Epilepsy; Pregnancy; Refractory status epilepticus; Clivus chordoma

Introduction

Status epilepticus (SE) is a condition resulting either from the failure of the mechanisms responsible for seizure termination, or from the initiation of mechanisms which lead to abnormally, prolonged seizures. It is a condition, which requires urgent medical attention and is a rare but potentially life threatening complication that women with epilepsy may experience during pregnancy [1]. When a convulsive epileptic status occurs, the patient does not get back to a normal alert state between repeated tonic-clonic attacks [1]. Pregnancy-related risk factors for SE are: a seizure- free period lasting less than 12 months before pregnancy, altered hormonal levels, changed metabolism induced by medication and many other factors (compliance, stress, and emotional changes) [2,3]. Indeed, the marked increases in sex hormone levels during pregnancy may also modify seizure activity. The hormonal influence may be twofold. Hormones may interact with the pharmacokinetics (PK) of Antiepileptic Drugs (AEDs). They may also have a direct pharmacodynamic effect on the seizure threshold [3]. A range of physiologic changes during gestation may alter the PK of AEDs. Increased plasma volume and/or increased total body water may lead to increased volume of distribution, and thus reduced AED serum concentrations. Furthermore also worries and psychosocial problems related to the pregnant state may contribute to the occurrence of seizures [3]. SE occurs in about 2% of pregnant women affected by epilepsy and its occurrence tends to be distributed evenly over the 3 trimesters [2]. During delivery it occurs in 1% of these women [4]. SE mortality rates range from 6 to 30% [1]. Refractory status epilepticus (RSE) is defined as an epileptic status which is unresponsive to early standard antiepileptic medical treatments. There have been only a few case reports of RSE during pregnancy in patients affected by systemic lupus erythematosus (SLE), porphyria, vitamin B6 deficiency, and cavernous angioma [5,6]. We discuss the case of a 29-year-old primigravida epileptic patient affected by clival chordoma. This woman presented a RSE which had a positive outcome after a spontaneous premature delivery.

Case Report

A no smoker primigravida, 29-year-old woman, affected by epilepsy since her childhood, came to the Department of Gynaecology and Obstetrics of Policlinico Umberto I, University Hospital of Rome, at 25 weeks of gestation for recurrent seizures. Her medical history was characterized by monthly seizures treated with lamotrigine (425 mg /die) and clonazepam (1 ml /die). Serum lamotrigine levels were always within the therapeutic range (3-15 μg/mL). During initial screening a diagnosis of preeclampsia was excluded because her blood pressure was normal, there was no proteinuria and no oedemas. She was immediately treated with high doses of benzodiazepines (midazolam 2 mg and diazepam 20 mg i.v.) . An obstetric ultrasound examination indicated that the foetus was alive with normal amniotic fluid index (AFI) and normal spontaneous foetal movements. The Electroencephalogram (EEG) showed the presence of short periods of diffuse cortical depression constituting a framework of burst-suppression. The patient developed tonic-clonic seizures, psychomotor agitation, and visual and auditory hallucinations with no recovery to a normal alert state between attacks. She was moved to the Intensive Care Unit where she was intubated, kept in an induced coma and checked with continuous EEG. The track laid showed a status epilepticus affecting the right and frontal-temporal hemisphere. When doing the daily obstetric ultrasound scan, we found out that the foetus was alive, having normal fluximetric indices and AFI, but no spontaneous foetal movements were observed. The patient was treated with progesterone for tocolysis and corticosteroids for lung maturation. In order to find out potential underlying causes of RSE (for example, focal or diffuse lesions of the central nervous system), a Magnetic Resonance Imaging (MRI) of the brain was performed; this revealed the presence of a chordoma of the clivus, whose major diameter was of 31 mm (Figure 1a). This is a rare, slow-growing intracranial tumour, characterized by locally aggressive growth patterns and high local recurrence rates. It is surgically resectable even if, given the location and invasive nature of this tumour, complete resection is difficult [7]. Induced coma was interrupted on day 4 (25 weeks + 4 days), but EEG findings and clinical seizures were not successfully controlled by the therapy. The health conditions of the woman were unstable, until the 10th day of hospitalization (26 weeks +3 days) when, after a seizure, despite tocolysis, a premature labour started and the patient was moved to the delivery room. A female baby, morphologically normal, weighing 812 gr with Apgar score of 4/8 was born. The baby developed a severe respiratory depression and an intraventricular haemorrhage (grade II) which was probably due to prematurity. However, she did not show any other neonatal problems and was discharged in good health conditions after 2 months. After childbirth, the RSE of the patient immediately reversed. The patient was treated pharmacologically (lamotrigine 450 mg/die, carbamazepine 800 mg/die, promazine ½ fl if necessary) and did not present any attacks in the first three days postpartum and the EEG gradually improved. On day 4 postpartum, she presented an episode of absence which disappeared spontaneously. Six months after childbirth, the patient was asymptomatic and continued her antiepileptic treatment; a second brain RMI was performed and the clivus chordoma appeared reduced in size (25 mm) (Figure1b).
Figure 1a: Brain MRI sagittal section; highlighted clivus chordoma in pregnancy (31 mm diameter).
Figure 1b: Brain MRI sagittal section; clivus chordoma after 6 months of pregnancy (25 mm diameter).

Discussion

SE in the pregnant state is usually unpredictable and unpreventable. In addition to this, delivery may have beneficial effects and it is the best treatment option in pregnant patients affected with RSE. In women with epilepsy during pregnancy SE may occur in up to 1.3% of the cases [1,8]. Few cases of RSE in pregnancy are associated with vitamin deficiency, SLE, cavernous angioma and cortical dysplasia [5,6,9]. Our patient had an unplanned pregnancy and the seizure-free interval before pregnancy had a duration of less than twelve months: her disease appeared not to be well controlled, which may increase the risk of seizures in pregnancy. She did not smoke, assumed regularly antiepileptic drugs, had no detectable vitamin deficiency or metabolic abnormality and no other medical problems. When RSE was diagnosed, we scheduled a brain MRI to investigate the possible presence of an unknown disorder causing seizures unresponsive to standard therapy. The MRI showed a clivus chordoma and no other hypoxic/ischemic vascular disorder (Figure 1a). There is no evidence of specific effects of the presence of clivus chordoma in pregnancy nor are there reports on the presence of seizures in patients affected by clivus chordoma. Despite therapy, normalization of clinical situation was not achieved until after the occurrence of spontaneous premature delivery. Similarly other cases of RSE in pregnancy recovered only after the delivery [6,7,9].

Conclusion

We described for the first time a case of RSE in a pregnant epileptic patient affected by chordoma of the clivus. Clival chordoma has very low incidence in patients younger than 40 years; it is painless and slow-growing, therefore it is often clinically silent until the late stages of the disease. Skull-base chordomas often grow in the clivus and may sometime cause cranial-nerve palsies. Depending on their size and involvement of the sella, endocrinopathy can also occur [7]. Other rare occurrences include epistaxis and intracranial haemorrhage [8]. We can only hypothesize that the presence of clivus chordoma in pregnancy contributed to the triggering of RSE in our epileptic patient. MRI scheduled at six months after delivery, showed a reduction of the chordoma size (20 % decrease). We suggest that MRI is a safe, non-invasive imaging technique in pregnancy that can often provide useful information about brain diseases, as in our case. It is impossible to determine which factors precipitated the onset of seizures in this patient; the only certainty is the immediate cessation of seizures following the ending of her pregnancy. The therapeutic effect of delivery strongly suggests that pregnancy-induced hormonal changes were likely to be responsible for the patient’s RSE [9]. Given the lack of adequate clinical trials, epileptic pregnant women or those planning a pregnancy should be followed-up in specialized centres for the management and treatment of potential complications.

References

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  4. Nakken KO, Lillestølen KM, Taubøll E, Engelsen B, Brodtkorb E (2006) [Epilepsy and pregnancy--drug use, seizure control, and complications]. TidsskrNorLaegeforen 126: 2507-2510.

  5. Schulze-Bonhage A, Kurthen M, Walger P, Elger CE (2004) Pharmacorefractory status epilepticus due to low vitamin B6 levels during pregnancy. Epilepsia 45: 81-84.

  6. Aladdin Y, Gross DW (2008) Refractory status epilepticus during pregnancy secondary to cavernous angioma. Epilepsia 49: 1627-1629.

  7. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT (2012) Chordoma: current concepts, management, and future directions.Lancet Oncol. 13:e69–76.

  8. Kaplan PW, Norwitz ER, Ben-Menachem E, Pennell PB, Druzin M, et al. (2007) Obstetric risks for women with epilepsy during pregnancy. Epilepsy Behav 11: 283-291.

  9. Jeong HS, Oh ES, Lee JH, Kim JM (2011) Refractory status epilepticus spontaneously resolved by parturition. J Epilepsy Res 1: 29-31.

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