Abstract
Background
Drug-resistant juvenile myoclonic epilepsy (DR-JME) remains a significant challenge in neurology. Traditional management strategies often fail to achieve satisfactory control, necessitating innovative treatments.
Objective
This case report aims to evaluate the efficacy and safety of deep brain stimulation (DBS) targeting the subthalamic nucleus (STN-DBS) in a patient with DR-JME.
Methods
We describe the treatment of a patient with DR-JME using STN-DBS. The patient underwent implantation and received high-frequency stimulation (HFS) at the STN.
Results
One year post-implantation, the patient demonstrated a substantial reduction in motor seizure frequency by 87.5%, with improvements in quality of life and seizure severity by 52.0% and 46.7%, respectively. No adverse events were reported during the follow-up period.
Conclusions
This case represents the first report of favorable outcomes with STN-DBS in a patient with DR-JME, suggesting that long-term HFS of the STN may be a promising treatment option for patients suffering from this condition.
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Data availability
The original contributions provided in the study are all included in the article and can be further queried to the corresponding author if specifically requested.
References
Juul-Jensen P, Foldspang A (1983) Natural history of epileptic seizures. Epilepsia 24:297–312
Syvertsen M, Hellum MK, Hansen G et al (2017) Prevalence of juvenile myoclonic epilepsy in people <30 years of age-A population-based study in Norway. Epilepsia 58:105–112
Janz D (1985) Epilepsy with impulsive petit mal (juvenile myoclonic epilepsy). Acta Neurol Scand 72:449–459
Scheffer IE, Berkovic S, Capovilla G et al (2017) ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia 58:512–521
Calleja S, Salas-Puig J, Ribacoba R, Lahoz CH (2001) Evolution of juvenile myoclonic epilepsy treated from the outset with sodium valproate. Seizure 10:424–427
Ascoli M, Mastroianni G, Gasparini S et al (2021) Diagnostic and therapeutic approach to drug-resistant juvenile myoclonic epilepsy. Expert Rev Neurother 21:1265–1273
Fisher R, Salanova V, Witt T et al (2010) Electrical stimulation of the anterior nucleus of thalamus for treatment of refractory epilepsy. Epilepsia 51:899–908
Dalic LJ, Warren AEL, Bulluss KJ et al (2022) DBS of thalamic centromedian nucleus for Lennox-Gastaut syndrome (ESTEL Trial). Ann Neurol 91:253–267
Vitek JL, Jain R, Chen L et al (2020) Subthalamic nucleus deep brain stimulation with a multiple independent constant current-controlled device in Parkinson’s disease (INTREPID): a multicentre, double-blind, randomised, sham-controlled study. Lancet Neurol 19:491–501
Prabhu S, Chabardes S, Sherdil A et al (2015) Effect of subthalamic nucleus stimulation on penicillin induced focal motor seizures in primate. Brain Stimul 8:177–184
Ryan LJ, Sanders DJ (1994) Subthalamic nucleus and globus pallidus lesions alter activity in nigrothalamic neurons in rats. Brain Res Bull 34:19–26
Vercueil L, Benazzouz A, Deransart C et al (1998) High-frequency stimulation of the subthalamic nucleus suppresses absence seizures in the rat: comparison with neurotoxic lesions. Epilepsy Res 31:39–46
Wille C, Steinhoff BJ, Altenmüller DM et al (2011) Chronic high-frequency deep-brain stimulation in progressive myoclonic epilepsy in adulthood–report of five cases. Epilepsia 52:489–496
Horn A, Li N, Dembek TA et al (2019) Lead-DBS v2: Towards a comprehensive pipeline for deep brain stimulation imaging. Neuroimage 184:293–316
Ewert S, Plettig P, Li N et al (2018) Toward defining deep brain stimulation targets in MNI space: a subcortical atlas based on multimodal MRI, histology and structural connectivity. Neuroimage 170:271–282
Camfield CS, Striano P, Camfield PR (2013) Epidemiology of juvenile myoclonic epilepsy. Epilepsy Behav 28(Suppl 1):S15-17
Gloor P, Fariello RG (1988) Generalized epilepsy: some of its cellular mechanisms differ from those of focal epilepsy. Trends Neurosci 11:63–68
Kazis D, Petridis F, Chatzikonstantinou S et al (2021) Gray matter changes in juvenile myoclonic epilepsy. A voxel-wise meta-analysis. Medicina (Kaunas) 57:1136
Geithner J, Schneider F, Wang Z et al (2012) Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25–63 years of follow-up. Epilepsia 53:1379–1386
Loscher W, Potschka H, Sisodiya SM, Vezzani A (2020) Drug resistance in epilepsy: clinical impact, potential mechanisms, and new innovative treatment options. Pharmacol Rev 72:606–638
Kostov H, Larsson PG, Røste GK (2007) Is vagus nerve stimulation a treatment option for patients with drug-resistant idiopathic generalized epilepsy? Acta Neurol Scand Suppl 187:55–58
Loddenkemper T, Pan A, Neme S et al (2001) Deep brain stimulation in epilepsy. J Clin Neurophysiol 18:514–532
Yan H, Ren L, Yu T (2022) Deep brain stimulation of the subthalamic nucleus for epilepsy. Acta Neurol Scand 146:798–804
Thuberg D, Buentjen L, Holtkamp M et al (2021) Deep brain stimulation for refractory focal epilepsy: unraveling the insertional effect up to five months without stimulation. Neuromodulation 24:373–379
Benabid AL, Koudsie A, Benazzouz A et al (2001) Deep brain stimulation of the corpus luysi (subthalamic nucleus) and other targets in Parkinson’s disease. Extension to new indications such as dystonia and epilepsy. J Neurol 248(Suppl 3):37–47
Gale K (1992) Subcortical structures and pathways involved in convulsive seizure generation. J Clin Neurophysiol 9:264–277
Devergnas A, Piallat B, Prabhu S et al (2012) The subcortical hidden side of focal motor seizures: evidence from micro-recordings and local field potentials. Brain 135:2263–2276
Ren L, Yu T, Wang D et al (2020) Subthalamic nucleus stimulation modulates motor epileptic activity in humans. Ann Neurol 88:283–296
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The study was approved by the Ethics Committee of The First Affiliated Hospital of Anhui Medical University (Ethics Committee Approval code: 2019H022). All procedures performed in studies involving human participants were conducted in accordance with the ethical standards of the institutional and/or national research committee and with the 1975 Helsinki declaration and its later amendments or comparable ethical standards.
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Shan, M., Mao, H., Hu, T. et al. Deep brain stimulation of the subthalamic nucleus for a patient with drug resistant juvenile myoclonic epilepsy: 1 year follow-up. Neurol Sci (2024). https://doi.org/10.1007/s10072-024-07553-1
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DOI: https://doi.org/10.1007/s10072-024-07553-1