Outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression.
OBJECTIVE
To determine whether multiple subpial transection in the posterior temporal lobe has an impact on long-term outcome in children who have drug-resistant Landau-Kleffner syndrome (LKS) or other "electrical status epilepticus during sleep" (ESES)-related regression. Given the wide variability in outcomes reported in the literature, a secondary aim was to explore predictors of outcome.


METHODS
The current study includes a surgery group (n = 14) comprising patients who underwent multiple subpial transection of the posterior temporal lobe and a nonsurgery comparison group (n = 21) comprising patients who underwent presurgical investigations for the procedure, but who did not undergo surgery. Outcomes were assessed utilizing clinical note review as well as direct assessment and questionnaires.


RESULTS
The distribution of nonclassical cases was comparable between groups. There were some differences between the surgery and nonsurgery groups at presurgical investigation including laterality of discharges, level of language impairment, and age; therefore, follow-up analyses focused on change over time and predictors of outcome. There were no statistically significant differences between the groups in language, nonverbal ability, adaptive behavior, or quality of life at follow-up. There was no difference in the proportion of patients showing improvement or deterioration in language category over time for either group. Continuing seizures and an earlier age of onset were most predictive of poorer quality of life at long-term follow-up (F2,23 = 26.2, p = <0.001, R(2) = 0.714).


CONCLUSIONS
Both surgery and nonsurgery groups had similar proportions of classic LKS and ESES-related regression. Because no significant differences were found in the changes observed from baseline to follow-up between the two groups, it is argued that there is insufficient evidence to suggest that multiple subpial transection provides additional benefits over and above the mixed recovery often seen in LKS and related regressive epilepsies.
UCL Institute of Child Health, London, United Kingdom.
Michelle
M
Downes
UCL Institute of Child Health, London, United Kingdom.|Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Rebecca
R
Greenaway
UCL Institute of Child Health, London, United Kingdom.|Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Maria
M
Clark
UCL Institute of Child Health, London, United Kingdom.|Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
J
J
Helen Cross
UCL Institute of Child Health, London, United Kingdom.
Nicola
N
Jolleff
UCL Institute of Child Health, London, United Kingdom.|Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
William
W
Harkness
Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Marios
M
Kaliakatsos
Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Stewart
S
Boyd
Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Steve
S
White
UCL Institute of Child Health, London, United Kingdom.
Brian G R
BG
Neville
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis* / Q000601:surgery*; D008297:Male; D010841:Pia Mater / Q000473:pathology / Q000601:surgery; D012893:Sleep Wake Disorders / Q000175:diagnosis* / Q000601:surgery*; D013702:Temporal Lobe / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome
Electrical status epilepticus during sleep; Landau-Kleffner syndrome; Multiple subpial transection; Neurocognitive outcome; Neurosurgery