Early hemispherectomy in catastrophic epilepsy

Abstract

The authors report their experience about a neuro-cognitive and epileptic long-term follow-up of children with catastrophic epilepsy treated with hemispherectomy in the first 5 years of life. Nineteen children with resistant epilepsy that significantly interfered with their neuro-cognitive development underwent hemispherectomy within 5 years of life (mean: 2 years, 3 months; range: 5 months to 5 years). All patients were assessed before surgery and after, at least at the end of the follow-up (mean: 6 years and 6 months; range: 2-11 years and 2 months) with a full clinical examination including motor ability and functional status evaluation as well as behaviour observation, neuroimaging and an ictal/interictal prolonged scalp video-EEG. A seizure-free outcome was obtained in 73.7% of patients. Gross motility generally improved and cognitive competence did not worsen, with an evident progress in two cases. Consistently with previous reports, evolution was worse in cortical dysplasia than in progressive or acquired vascular cerebropathies. The excellent epileptic outcome and the lack of developmental deterioration in comparison with other more aged series seem to suggest a possible better evolution in earlier surgery treatment. To confirm this suggestion, however, further experience with larger series is needed.

Fifty Consecutive Hemispherectomies

“…This reassuring finding is consistent with those of prior studies 24, 27, 55–58 . Despite the overall group-level stability, some individuals demonstrated significant changes in FSIQ.…”

Surgical Treatments for Epilepsies in Children Aged 1–36 Months

“…Five studies were conducted in the United States. The remaining 11 non-US studies were conducted in Germany [19][20][21] (n = 3), Japan 22,23 (n = 2), Canada 24,25 (n = 2), Italy 26,27 (n = 2), Sweden 18 (n = 1), or included data from multiple countries (n = 1). 28 All US studies were single-center studies from the University of California at Los Angeles, 29,30 University of Colorado, 31 Cleveland Clinic, 32 Boston Children's Hospital, 33 and Miami Children's hospital.…”

“…Eight retrospective prestudies/poststudies (in 9 articles) included 188 infants 18,20,23,26,[28][29][30]32,33 and reported on seizure freedom from 6 months to mean 4.3 years after surgery (Table 1). One study 23 did not report the follow-up interval for the subgroup of included patients.…”

Surgical Treatment of Pediatric Epileptic Encephalopathies

“…Villarejo-Ortega et al showed that although patients with perinatal vascular lesions had less cognitive improvement after-hemispherectomy than late onset acquired epilepsy such as Rasmussen's encephalopathy, there was an inverse linear correlation between the degree of cognitive improvement and the age at surgery [ 104 ]. Lettori et al also suggested that young age at surgery affords better cognitive improvement [ 105 ]. The percentage of patients enjoying developmental improvement varies from 57% to 72% [ 87 , 106 – 108 ].…”

“…Although developmental improvement can be an attainable goal, the majority of encephalopathic children undergoing epilepsy surgery experience intellectual stability in contrast to their presurgical decline [ 105 , 110 – 113 ]. Devlin et al published their hemispherectomy experience on 33 children that showed lack of cognitive decline generally and improvement in 4 patients across all categories of pathology, developmental, acquired, or progressive [ 110 ].…”