Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy

Abstract

A boy with a right congenital hemiparesis due to a left pre-natal middle cerebral artery infarct developed focal epilepsy at 33 months and then an insidious and subsequently more rapid, massive cognitive and behavioural regression with a frontal syndrome between the ages of 4 and 5 years with continuous spike-waves during sleep (CSWS) on the EEG. Both the epilepsy and the CSWS were immediately suppressed by hemispherotomy at the age of 5 years and 4 months. A behavioural-cognitive follow-up prior to hemispherotomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the "healthy" frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. This outcome is probably explained by another facet of the epilepsy, namely the structural effects of prolonged epileptic discharges in rapidly developing cerebral networks which are, at the same time undergoing the reorganization imposed by a unilateral early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and post-operatively, may miss particular epileptic cognitive dysfunctions as they are likely to be different from case to case. Such detailed and rarely available complementary clinical and EEG data obtained in a single case at different time periods in relation to the epilepsy, including per-operative electrophysiological findings, may help to understand the different cognitive deficits and recovery profiles and the limits of full cognitive recovery

The effect of surgery in encephalopathy with electrical status epilepticus during sleep

“…Two of three of our focal resection or hemispherotomy patients became seizure‐free, which is similar to the experience of Kallay et al. (2008) and Loddenkemper et al.…”

“…Two of three of our focal resection or hemispherotomy patients became seizure-free, which is similar to the experience of Kallay et al (2008) and Loddenkemper et al (2009) after hemispherotomy, hemispherectomy, or focal resection in ESES patients. Other series of epilepsy surgery in ESES have not been reported.…”

“…One patient with IQ below 30 lost ambulation at the beginning of SES and showed developmental progression by learning to walk again after surgery. Catch‐up development, that is, significantly improved cognitive level after surgery seems to be possible, although rare, in ESES, as shown by three of our patients and previously also by Kallay et al. (2008) and Loddenkemper et al.…”

“…Focal resections resulted in either focal continuous spiking or regional SES on the side of the operation with diminished source strength. Termination of SES is a predicted result when the epileptogenic area is completely resected (Kallay et al., 2008; Loddenkemper et al., 2009). Interhemispheric propagation may continue after anterior or total callosotomy, as seen in most of our patients (Gates et al., 1984; Spencer et al., 1988; Quattrini et al., 1997).…”

“…Termination of SES, seizure freedom, and developmental benefits has been reported after resective surgery or hemispherotomy in ESES (Kallay et al, 2008;Loddenkemper et al, 2009).…”