Fosi Tangunu, Chu Carlton, Chong Wui Khean, Clark Chris, Scott Rod C, Boyd Stewart, De Haan Michelle, Neville Brian
Young Epilepsy, Surrey, United Kingdom; Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom; Neurosciences Unit, University College London Institute of Child Health, London, United Kingdom.
Epilepsia. 2015 Apr;56(4):608-16. doi: 10.1111/epi.12907. Epub 2015 Mar 2.
To explore the structure-function relation of the temporal lobe in newly diagnosed West syndrome of unknown cause (uWS).
Quantitative magnetic resonance imaging (three-dimensional [3D] structural MRI and diffusion tensor imaging [DTI]) was analyzed using voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS) in 22 patients and healthy age-matched controls. The electrophysiologic responsiveness of the temporal lobe was measured using the N100 auditory event-related potential (aERP) to a repeated 1,000 Hz tone. Neurocognitive function was assessed using the Bayley Scales of Infant Development, Second Edition (BSID-II). Tests followed first-line treatment with vigabatrin (17 patients) or high-dose oral prednisolone (5 patients).
Total temporal lobe volume was similar in patients and controls. Patients had a smaller temporal stem (TS) (p < 0.0001) and planum temporale (PT) (p = 0.029) bilaterally. TS width asymmetry with a larger right-sided width in controls was absent in patients (p = 0.033). PT asymmetry was present in both groups, being larger on the right (p = 0.048). VBM gray matter volume was increased at the left temporal lobe (superior and middle temporal gyri, the peri-rhinal cortex, and medial temporal lobe) (p < 0.005, family wise error-corrected). VBM gray matter volume correlated with the duration of infantile spasms (Pearson's r = -0.630, p = 0.009). DTI metrics did not differ between patients and controls on TBSS. Mean BSID-II scores were lower (p < 0.001) and auditory N100 ERP attenuated less in patients than in controls (p = 0.002).
The functional networking and white matter development of the temporal lobe are impaired following infantile spasms. Treatment may promote structural plasticity within the temporal lobe following infantile spasms, manifest as increased gray matter volume on VBM. It remains to be investigated further whether this predicts patients' long-term cognitive difficulties.
探讨新诊断的病因不明的韦斯特综合征（uWS）中颞叶的结构-功能关系。
对22例患者和年龄匹配的健康对照者进行定量磁共振成像（三维[3D]结构MRI和扩散张量成像[DTI]），采用基于体素的形态计量学（VBM）和基于纤维束的空间统计学（TBSS）进行分析。使用N100听觉事件相关电位（aERP）对重复的1000 Hz音调测量颞叶的电生理反应性。使用贝利婴儿发育量表第二版（BSID-II）评估神经认知功能。在使用vigabatrin（17例患者）或高剂量口服泼尼松龙（5例患者）进行一线治疗后进行测试。
患者和对照者的颞叶总体积相似。患者双侧颞叶干（TS）（p < 0.0001）和颞平面（PT）（p = 0.029）较小。患者不存在对照组中右侧宽度较大的TS宽度不对称（p = 0.033）。两组均存在PT不对称，右侧更大（p = 0.048）。VBM灰质体积在左侧颞叶（颞上回和颞中回、鼻周皮质和内侧颞叶）增加（p < 0.005，家族性错误校正）。VBM灰质体积与婴儿痉挛持续时间相关（Pearson相关系数r = -0.630，p = 0.009）。在TBSS上，患者和对照者之间的DTI指标没有差异。患者的平均BSID-II评分较低（p < 0.001），听觉N100 ERP衰减程度低于对照者（p = 0.002）。
婴儿痉挛后颞叶的功能网络和白质发育受损。治疗可能促进婴儿痉挛后颞叶内的结构可塑性，表现为VBM上灰质体积增加。这是否预示患者的长期认知困难仍有待进一步研究。
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