SUPER REFRACTORY STATUS EPILEPTICUS: CASE REPORT. Vignali A, Viviani R, Bardini A, Morelli G, Maggiani M, Mos G, Monfroni A, Roni C, Tonelli E.
SUPER REFRACTORY STATUS EPILEPTICUS: CASE REPORT Vignali A, Viviani R, Bardini A, Morelli G, Maggiani M, Mos@ G, Monfroni A, Roni C, Tonelli E Anestesia Rianimazione ed Elisoccorso - Ospedale Apuane - Azienda USL Toscana nord ovest
INTRODUCTION Late or super refractory status epilep0cus (LRSE) can be defined as status epilep0cus that con0nues or recurs
24hours or more a>er the onset of anaesthe0c and an0convulsivant therapy. It is a severe variant of refractory status epilep0cus (RSE), which is defined as con0nuous or repe00ve seizures las0ng longer than 60 minutes despite treatment with a benzodiazepine together with any other standard an0convulsant. It is a fairly uncommon clinical problem that should be treated promptly to prevent the high morbidity and mortality rates of up to 50%. For pa0ents who do recover, the prognosis can be guardedly posi0ve given sufficient 0me, par0cularly for young pa0ents. Mirski et al. reported recovery of consciousness and significant neurological func0on a>er more than 53 days in therapeu0c barbiturate coma. Protocols for treatment at the onset of status epilep0cus are well defined. Treatment for LRSE is less clear and scarce evidence is available to support the choice of specific treatments. Most of these treatment modali0es are based on either observa0onal studies or case reports. It’s clear that addi0onal research on how best to treat RSE is needed. Midazolam(MDZ), propofol and barbiturates represent the most used alterna0ves. Several other treatments, such as addi0onal anaesthe0cs, ketamine, halogenate, lidocaine, magnesium, phenobarbital, phenytoin or other an0epilep0c, immunomodulatory compounds, steroids, gammaglobulin, plasmapheresis electroconvulsive therapy, hypothermia, ketogenic-diet, brain or nerve s0mula0on and neurosurgery have been discussed. EEG monitoring is crucial, as many pa0ents (20%) whose clinical symptoms are controlled con0nue to experience electrographic seizures. Mortality and morbidity are significantly higher when such non-convulsive seizures are present. The longer the dura0on of status epilep0cus, the higher the risk of progression to refractory status epilep0cus, which may result in either death or debilita0ng sequelae. Female gender and acute underlying causes including acute infec0ons of the central nervous system have both been iden0fied as strong factors for developing refractory illness. CASE REPORT We report the case of a 28 years old woman with severe LRSE requiring more than 3 months of iatrogenic coma and 5 months of ICU stay. She was well un0l the days prior to hospital admission when she developed dizziness, headache, pharestesia, fever 39°C, agita0on and confusion. In the emergency room, she developed generalized tonic-clonic seizures with prolonged loss of consciousness. General exam was unremarkable, with no meningismus, rash or 0cs. Neurological exam showed coma with intact cranial nerves and no obvious focal finding, with a nega0ve CT scan. She had addi0onal clinical seizures not responding to standard therapy requiring transfer to the ICU. Despite high dose of phenytoin, propofol and midazolam in drip she con0nued having frequent, convulsive and non-convulsive seizures . She was monitored with con0nuous EEG and thiopental(TPS) drip was started and 0trated to suppression burst. EEG seizures arising from mul0ple foci con0nued to occur from suppressionburst, and TPS was increased further. All tests obtained were nega0ve: CT scan acquired at admission in emergency room and 1 month later; MRI scan with contrast was obtained in the first day a>er admission and repeated 1 week, 1 month, 3 months and 5 months later; PET was performed 2 months a>er admission. Laboratory values, cerebrospinal fluid, test of viral or bacterial encephali0s, research for autoimmune or neoplas0c syndrome was uniformly nega0ve. There was not known hypoxia or other secondary injury at any point. Opinions on prognosis and clinical management were solicited from a number of experienced neuro-intensivist and neurologist at mul0ple 0me points during the clinical course. Over the first 2 months the pa0ents was maintained on TPS drip in combina0on with MDZ. All afempts at reduc0on of an0convulsivant medica0on resulted in the reappearance of frequent electrographic seizures ac0vity. If le> untreated, this would eventually evolve into 2-4hz generalized spike-and-wave discharges, accompanied by face jerking as well, especially a>er s0mula0on such as suc0oning. This exacerba0on of periodic or ictal EEG ac0vity with s0mula0on is not unusual in cri0cally ill pa0ents and has termed SIRPID (s0mula0on induced rhythmic, periodic, or ictal discharged). The following therapies have been tried while maintain the seizures control with TPS at maximum dose of 3,3 g/die: phenobarbital, MDZ, propofol, phenytoin, valproate, topiramate, leve0racetam, carbamazepine, gabapen0n, lacosamide, pregabalin, intravenous immunoglobulin, lidocaine, ketamine, cor0sone, magnesium and ketogenic-diet. A>er 3 months was feasible to slowly decrease the daily dose of TPS un0l the complete withdrawal in 20 days. The EEG monitoring showed progressive improvement, with the occurrence of par0al seizure followed by pos0ctal period of short dura0on. During the 5 months ICU stay early percutaneous tracheostomy and gastrostomy were performed, occurred infec0ve complica0on by KPC and MRSA, and deep-vein thrombosis; both resolved. A>er 5 months the pa0ents was discharged in rehabilita0on, orientated free from seizures in therapy with phenytoin, leve0racetam, and diazepam.
RosseK AO, Lowenstein DH. Management of refractory status epilep@cus in adults: s@ll more ques@ons than answers. Lancet Neurol 2011;10:922-30 Onya CU et Al. Super-Refractory Status Epilep@cus: An Atypical Presenta@on and Case -Based Review. Epilepsi 2015;21(2):89-94
