Pilot Study of Neurodevelopmental Impact of Early Epilepsy Surgery in Tuberous Sclerosis Complex.,Pediatric Neurology

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Pilot Study of Neurodevelopmental Impact of Early Epilepsy Surgery in Tuberous Sclerosis Complex.
Pediatric Neurology ( IF 3.8 ) Pub Date : 2020-04-14 , DOI: 10.1016/j.pediatrneurol.2020.04.002
Leslie E Grayson ₁ , Jurriaan M Peters ₂ , Tarrant McPherson ₃ , Darcy A Krueger ₄ , Mustafa Sahin ₅ , Joyce Y Wu ₆ , Hope A Northrup ₇ , Brenda Porter ₈ , Gary R Cutter ₃ , Sarah E O'Kelley ₉ , Jessica Krefting ₁ , Scellig S Stone ₁₀ , Joseph R Madsen ₁₀ , Aria Fallah ₁₁ , Jeffrey P Blount ₁₂ , Howard L Weiner ₁₃ , E Martina Bebin ₁ ,

Affiliation

Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama.
Localization Laboratory, Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Department of Biostatistics, University of Alabama at Birmingham, Birmingham, Alabama.
Department of Neurology, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Department of Neurology and the F.M. Kirby Neurobiology Center, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Division of Pediatric Neurology, UCLA Mattel Children's Hospital, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California.
Department of Pediatrics, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, Texas.
Department of Neurology, The Stanford University Medical Center, Stanford, California.
Department of Psychology, University of Alabama at Birmingham, Birmingham, Alabama.
Division of Pediatric Neurosurgery, Department of Neurosurgery, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Department of Neurosurgery, Division of Pediatric Neurosurgery, University of California Los Angeles Mattel Children's Hospital, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California.
Department of Neurosurgery, Division of Pediatric Neurosurgery, Children's Hospital of Alabama and University of Alabama at Birmingham, Birmingham, Alabama.
Division of Pediatric Neurosurgery, Department of Surgery, Texas Children's Hospital and Department of Neurosurgery, Baylor College of Medicine, Houston, Texas.

Background

To determine if early epilepsy surgery mitigates detrimental effects of refractory epilepsy on development, we investigated surgical and neurodevelopmental outcomes in children with tuberous sclerosis complex who underwent surgery before age two years.

Methods

Prospective multicenter observational study of 160 children with tuberous sclerosis complex. Surgical outcome was determined for the seizure type targeted by surgery. We obtained Vineland Adaptive Behavior Scales, Second Edition (Vineland-II); Mullen Scales of Early Learning; and Preschool Language Scales, Fifth Edition, at age three, six, nine, 12, 18, 24, and 36 months. Surgical cases were compared with children without seizures, with controlled seizures, and with medically refractory seizures.

Results

Nineteen children underwent surgery (median age 17 months, range 3.7 to 21.3), and mean follow-up was 22.8 months (range 12 to 48). Surgical outcomes were favorable in 12 (63%, Engel I-II) and poor in seven (37%, Engel III-IV). Nine (47%) had new or ongoing seizures distinct from those surgically targeted. All children with seizures demonstrated longitudinal decline or attenuated gains in neurodevelopment, the surgical group scoring the lowest. Favorable surgical outcome was associated with increased Mullen Scales of Early Learning receptive and expressive language subscores compared with the medically refractory seizure group. A nonsignificant but consistent pattern of improvement with surgery was seen in all tested domains.

Conclusions

These pilot data show neurodevelopmental gains in some domains following epilepsy surgery. A properly powered, prospective multicenter observational study of early epilepsy surgery is needed, using both surgical and developmental outcome metrics.

中文翻译：

早期癫痫手术对结节性硬化症患者神经发育影响的初步研究。

背景

为了确定早期癫痫手术是否减轻难治性癫痫对发育的不利影响，我们调查了在两岁前接受手术的结节性硬化症儿童的手术和神经发育结果。

方法

160 例结节性硬化症患儿的前瞻性多中心观察研究。针对手术靶向的癫痫发作类型确定手术结果。我们获得了 Vineland Adaptive Behavior Scales, Second Edition (Vineland-II)；马伦早期学习量表；学龄前语言量表，第五版，3、6、9、12、18、24 和 36 个月。将手术病例与无癫痫发作、控制性癫痫发作和药物难治性癫痫发作的儿童进行比较。

结果

19 名儿童接受了手术（中位年龄 17 个月，范围 3.7 至 21.3），平均随访时间为 22.8 个月（范围 12 至 48）。12 人（63%，Engel I-II）手术结果良好，7 人（37%，Engel III-IV）手术结果不佳。9 人 (47%) 有新的或持续的癫痫发作，与手术靶向的癫痫发作不同。所有癫痫儿童都表现出神经发育的纵向下降或减弱，手术组得分最低。与医学难治性癫痫发作组相比，良好的手术结果与马伦早期学习量表的接受和表达语言子分数增加有关。在所有测试领域都看到了一种不显着但一致的手术改善模式。