Sobstyl Michał, Jezierski Paweł, Konopko Magdalena, Stapińska-Syniec Angelika
Department of Neurosurgery, Institute of Psychiatry and Neurology, Sobieskiego 9 Street 02-957, Warsaw, Poland.
1st Department of Neurology, Institute of Psychiatry and Neurology, Sobieskiego 9 Street, 02-957 Warsaw, Poland.
Epilepsy Behav Rep. 2023 Dec 1;25:100637. doi: 10.1016/j.ebr.2023.100637. eCollection 2024.
Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in the tumor suppressor genes TSC1 or TSC2. TSC is characterized by the formation of multiple tumors in various organs. The most common neurological manifestation of the disorder is epilepsy present in 79-90% of cases. At least one-third of TSC patients develop drug-resistant epilepsy (DRE) which remains a great challenge for clinicians. Neuromodulation is an option in cases of multifocal epilepsy, epilepsy originating in eloquent areas, or the inability to identify the ictal onset zone. Deep brain stimulation of the anterior thalamic nucleus (ANT-DBS) may be used in the treatment of multifocal DRE. Here, we present a case of a patient with multifocal DRE caused by TSC, who was treated with ANT-DBS. A follow-up period of eight months showed that the patient's multifocal DRE was successfully treated by ANT-DBS.
结节性硬化症(TSC)是一种常染色体显性疾病,由肿瘤抑制基因TSC1或TSC2的突变引起。TSC的特征是在各个器官中形成多个肿瘤。该疾病最常见的神经学表现是癫痫,在79 - 90%的病例中出现。至少三分之一的TSC患者会发展为药物难治性癫痫(DRE),这对临床医生来说仍然是一个巨大的挑战。神经调节是治疗多灶性癫痫、起源于明确区域的癫痫或无法确定发作起始区的癫痫的一种选择。丘脑前核深部脑刺激(ANT - DBS)可用于治疗多灶性DRE。在此,我们报告一例由TSC引起的多灶性DRE患者,其接受了ANT - DBS治疗。八个月的随访期显示,该患者的多灶性DRE通过ANT - DBS得到了成功治疗。