Highlights
- •Cognitive and behavioral testing in patients with Lennox–Gastaut syndrome (LGS) can be challenging.
- •As part of the ESTEL trial (thalamic DBS in LGS), we performed cognitive and behavioral measurements in 19 patients with LGS pre- and post-DBS treatment.
- •After 3-months of stimulation, caregiver-reported epilepsy severity (GASE) and disability (GAD) improved.
- •We did not find CM-DBS caused a decline in cognition, adaptive skills, epilepsy disability, quality of life or depression scores.
- •Raw scores appeared to better reflect caregiver reported changes in performance than standardized scores, due to their greater dynamic range.
Abstract
Purpose
We previously reported seizure and EEG outcomes of the ESTEL study (Electrical Stimulation
of Thalamus for Epilepsy of Lennox–Gastaut phenotype). To assess potential cognitive
and behavioral changes during chronic, duty-cycle stimulation of bilateral thalamic
centromedian nucleus, we compared standardized cognitive and behavioral measurements,
as well as caregiver assessments of disability/severity, before implantation and after
3-months stimulation.
Methods
Twenty patients with LGS (17–37 years;13 females) were studied; one participant was
not randomized due to DBS device removal, with outcomes of 19 remaining participants
reported here. Cognitive and behavioral measurements were performed at baseline (i.e.,
before DBS implantation), at the end of the blinded stimulation phase, and at study
exit. Instruments measured cognition (NIH toolbox cognitive battery, NIHTB-CB), adaptive
skills (ABAS-3), epilepsy severity (GASE) and disability (GAD), quality of life (QOLIE-31),
and depression (PHQ-9). Changes in scores after 3-months of stimulation relative to
baseline were explored using Wilcoxon matched-pairs signed rank tests.
Results
After 3-months of stimulation, caregiver-reported epilepsy severity (GASE) and disability
(GAD) improved (p<0.05). No other instrument showed a significant change from baseline. Measurements
that required direct participant involvement, rather than caregivers, was completed
by only a subset of higher-functioning individuals (NIHTB-CB, n = 13; QOLIE-31, n = 3; and PHQ-9, n = 6). In addition to cognitive impairments, behavioral and physical limitations were
common obstacles to instrument completion. Standardized scores were hindered by ‘floor
effects’; however, raw scores better reflected clinical impressions of participants’
functioning and were more sensitive to caregiver-reported changes following treatment.
Conclusion
DBS treatment is associated with reduced epilepsy severity and disability in young
adults with LGS. Performing cognitive and behavioral outcome measurement in patients
with cognitive impairment is challenging but possible and requires careful selection
of instruments and modifications of score interpretation to avoid floor effects.
Keywords
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Article info
Publication history
Published online: July 29, 2022
Accepted:
July 24,
2022
Received in revised form:
June 25,
2022
Received:
April 30,
2022
Identification
Copyright
Crown Copyright © 2022 Published by Elsevier Ltd on behalf of British Epilepsy Association. All rights reserved.
