Long-term outcomes after surgery for catastrophic epilepsy in infants: institutional experience and review of the literature

OBJECTIVEUncontrolled epilepsy is associated with serious deleterious effects on the neurological development of infants and has been described as “catastrophic epilepsy.” Recently, there has been increased emphasis on early surgical interventions to preserve or rescue neurodevelopmental outcomes in infants with early intractable epilepsy. The enthusiasm for early treatments is often tempered by concerns regarding the morbidity of neurosurgical procedures in very young patients. Here, the authors report outcomes following the surgical management of infants (younger than 1 year).METHODSThe authors performed a retrospective review of patients younger than 1 year of age who underwent surgery for epilepsy at Miami (Nicklaus) Children’s Hospital and Jackson Memorial Hospital between 1994 and 2018. Patient demographics, including the type of interventions, were recorded. Seizure outcomes (at last follow-up and at 1 year postoperatively) as well as complications are reported.RESULTSThirty-eight infants (median age 5.9 months) underwent a spectrum of surgical interventions, including hemispherectomy (n = 17), focal resection (n = 13), and multilobe resections (n = 8), with a mean follow-up duration of 9.1 years. Hemimegalencephaly and cortical dysplasia were the most commonly encountered pathologies. Surgery for catastrophic epilepsy resulted in complete resolution of seizures in 68% (n = 26) of patients, and 76% (n = 29) had a greater than 90% reduction in seizure frequency. Overall mortality and morbidity were 0% and 10%, respectively. The latter included infections (n = 2), infarct (n = 1), and immediate reoperation for seizures (n = 1).CONCLUSIONSSurgical intervention for catastrophic epilepsy in infants remains safe, efficacious, and durable. The authors’ work provides the longest follow-up of such a series on infants to date and compares favorably with previously published series.

Long-term follow-up in patients with catastrophic epilepsies

Objective: to investigate the features of the course and long-term outcome of catastrophic epilepsies. Patients and methods. A total of 130 patients (62 (47.7%) men and 68 (52.3%) women) aged 21 to 78 years were examined. The follow-up was 1 year to 14 years. The inclusion criteria for the investigation were above 18 years of age; severe epilepsy with daily seizures considered as catastrophic epilepsy. The exclusion criteria were a history of nonepileptic seizures of any etiology, as well as noncompliance. The examination included history data collection; seizure diary analysis; clinical and neurological examination; routine EEG and/or EEG-video monitoring; brain magnetic resonance imaging; and laboratory tests. Results and discussion. The onset of epilepsy immediately with catastrophic seizures was observed in 28 (21.5%) patients; this onset was most frequently seen in 18 of the 28 children. At the same time, catastrophic seizures did not appear immediately, but it took some time for them to occur after the onset of active epilepsy in 102 (78.5%) patients. Moreover, the onset of catastrophic seizures could not be associated with any external influences or errors in 58 (56.9%) of these patients taking the drugs. Transition to catastrophic epilepsy could occur both abruptly in 11 (10.8%) patients and gradually in 91 (89.2%). After treatment correction, 16.8% of patients achieved remission at the time of study completion; the frequency of seizures decreased by 50% or more in 27.1% of cases; the effect was absent in 56.1%. Conclusion. Based on the findings, it can be concluded that in catastrophic epilepsy, the probability exists for spontaneous occurrence and remission of destructive seizures, frequently regardless of the therapy. The onset of the disease and that of catastrophic seizures can be spread in time. The efficiency of treatment remains low in patients with catastrophic epilepsies.

Tailored disconnection based on presurgical evidence in catastrophic epilepsy: report of 2 cases

Catastrophic epilepsy in infants, often due to extensive cortical dysplasia, has devastating consequences with respect to brain development. Conventional lobar, multilobar, or hemispheric resection in these infants is challenging, carrying an increased operative risk compared with that in older children. Removing a larger tissue volume versus removing or disconnecting the epileptogenic region does not always guarantee better seizure outcome. The authors describe 2 infants with catastrophic epilepsy who benefited from individually tailored disconnections based on a hypothesized epileptogenic zone following intensive presurgical evaluation. Two infants with catastrophic epilepsy and epileptic spasms underwent leukotomies between 3 and 12 months of age. They were followed up postoperatively for 19–36 months. Both patients had 90%–100% seizure reduction and a significantly improved neurodevelopmental outcome without postoperative complication. Cortical malformation was seen in both patients. Modifications of established surgical disconnection techniques, tailored to each patient's specific epileptogenic zone, optimized seizure and neurodevelopmental outcomes while minimizing the risks associated with more extensive resections.