Abstracts

Rationale: Epilepsy with catamenial exacerbation is common and described in one third of patients with refractory partial epilepsy1 but catamenial status epilepticus is rarely reported.2 Catamenial epilepsy is the occurrence of a cluster of seizures in relation to the menstrual cycle. Neuroactive properties of reproductive hormones (neurosteroids) and their fluctuations in the menstrual cycle are thought to be the cause. Three types of catamenial seizure pattern have been described [1]: perimenstrual (C1, the most common type), periovulatory (C2), and inadequate luteal-phase (C3). Methods: We report two cases with recurrent episodes of catamenial status epilepticus refractory to antiepileptic medications (AED). Results: Case 1: A 33 year old right- handed female with a history of idiopathic generalised epilepsy with the seizure onset at the age of 28 years presented with recurrent episodes of convulsive and non-convulsive status epilepticus (SE) occurring on day 1 of her menstrual cycle over the last six months. A routine EEG and brain imaging were normal and she was refractory to three AEDs. Video-EEG (vEEG) for further diagnostic clarification showed periods of semi-continuous generalised spike and wave activity lasting up to 40 seconds with corresponding behavioural arrests, excessive blinking and facial twitching and brief inter burst intervals. These recurrent periods of absence status epilepticus were inter-mixed by generalised tonic clonic (GTC) seizures throughout the day. AEDs were changed and a combination including zonisamide and clobazam stopped the catamenial SE.Case 2: A 22 year old right-handed female with a history of cystic fibrosis and focal epilepsy from childhood re-presented with seizures aged 21 years after a long period of seizure freedom. Typical events were clusters of GTC seizures, with post ictal aphasia and right hemiparesis. Although her menstrual cycles were irregular, there was a very strong peri-menstrual distribution (C1). She was admitted to ICU on at least four occasions following episodes of SE and was refractory to multiple AEDs and hormonal treatment including mirena coil insertion. Extensive metabolic, autoimmune work-up and neuroimaging were normal.  vEEG confirmed frontal lobe epilepsy and an adjunctive hormonal treatment with Triptorelin was added. Triptorelin is a synthetic long acting agonist of gonadotropin releasing hormone and is a potent inhibitor of oestrogen.3 Triptorelin caused medical oophorectomy and our patient became seizure free at her last follow-up (at one year) and did not require further hospitalisation. Conclusions: Catamenial SE is rare and should be suspected in pre-menopausal women presenting with recurrent episodes of unresponsiveness, seizure clusters or SE in both generalised and focal epilepsy. Our case report highlights the need for documenting the menstrual cycles, identifying a pattern and perimenstrual Video-EEG for diagnosis. Although the treatment of refractory catamenial SE can be challenging, the options for treatment are expanding. Our second case also demonstrates the successful use of Triptorelin in refractory catamenial exacerbation of seizures/catamenial SE.References:[1] Herzog AG, Klein P, Ransil BJ. Three patterns of catamenial epilepsy. Epilepsia.1997;38(10):1082-8[2] Ramanujam B, Arora A, Malhotra V, Dash D, Mehta S, Tripathi M. A case of recurrent status epilepticus and successful management with progesterone. Epileptic Disord. 2016;18(1):101-5 doi: 10.1684/epd.2016.0792. [3] Bauer J, Wildt L, Flugel D, Stefan H. The effect of a synthetic GnRH analogue on catamenial epilepsy: a study in ten patients. J Neurol. 1992;239(5):284-6 Funding: No funding towards this work.