A case of recurrent status epilepticus and successful management with progesterone

Abstract

Catamenial epilepsy (CE) is a commonly observed phenomenon among women with epilepsy, the management of which is both hormonal and non‐hormonal. Progesterone therapy has been tried in these patients, as the possible mechanism of CE is withdrawal of progesterone and a higher oestrogen/progesterone ratio in the perimenstrual and periovulatory periods. Here, we describe a 24‐year‐old lady with multiple seizure types since childhood, which were refractory to adequate antiepileptic drug therapy after menarche with catamenial clustering of seizures. She went on to have several episodes of non‐convulsive status epilepticus also with similar periodicity, which would abate only with midazolam infusion, without the need for ventilatory support. She was tried on acetazolamide, progesterone vaginal pessaries, and maximum tolerated doses of antiepileptic medications, but finally responded to intramuscular and oral progesterone, and has been seizure‐free for more than a year.

Efficacy and Safety of Oral Micronized Progesterone That Converts to Allopregnanolone as an Adjunctive Treatment in Refractory Status Epilepticus: A Prospective Interventional Phase IIa Study

“…Few neurosteroids, including allopregnanolone, tetrahydrodeoxycorticosterone, and 3-alpha androstanediol, are inhibitory neurosteroids, meaning that they augment GABAergic signaling [17]. Only allopregnanolone showed in vitro and in vivo e cacy in controlling seizures in women with catamenial epilepsy as well as adult and pediatric RSE patients [9,10,13]. Our results show concordant ndings with other previous animal and human studies demonstrating that allopregnanolone improves control of seizures.…”

“…Allopregnanolone, a reduced progesterone metabolite and a positive allosteric modulator of γaminobutyric acid type A (GABA A ) receptor-mediated conduction, can reduce neuronal excitabilities and increase seizure thresholds, thus yielding a reduction of epileptiform discharges [7,8]. Previous clinical studies showed that both oral and parenteral forms of progesterone can improve seizure control as well as suppress seizure discharges in electroencephalography (EEG) [9,10]. These studies indicate that progesterone contains antiepileptic properties.…”

Low levels of progesterone and derivatives in cerebrospinal fluid of patients affected by status epilepticus

“…Recently, a successful treatment of SE in a woman with recurrent SE episodes was also reported (Ramanujam et al . ).…”

“…Intravenous progesterone for critically ill and injured patients has been demonstrated safe and inexpensive (Wigginton et al 2010). Recently, a successful treatment of SE in a woman with recurrent SE episodes was also reported (Ramanujam et al 2016).…”

Progesterone in Treatment of Catamenial Epilepsy

“…In one of these, 14 women with refractory partial seizures and normal ovulatory cycles were given parenteral depomedroxyprogesterone at 120-150 mg every 6-12 weeks, and had 39% seizure reduction. However, it is unclear whether the effect was due to direct anticonvulsant activity of medroxyprogesterone or to the hormonal consequences of the induced amenorrhea [15][16][17][18] .…”