Poliachik Sandra L, Friedman Seth D, Poliakov Andrew V, Budech Christopher B, Ishak Gisele E, Shaw Dennis W W, Gospe Sidney M
Department of Radiology, Seattle Children's Hospital, Seattle Washington.
Department of Radiology, Seattle Children's Hospital, Seattle Washington; Department of Radiology, University of Washington, Seattle Washington.
Pediatr Neurol. 2016 Jan;54:43-8. doi: 10.1016/j.pediatrneurol.2015.09.012. Epub 2015 Sep 25.
In this observational study, white matter structure, functional magnetic resonance imaging (fMRI) task-based responses, and functional connectivity were assessed in four subjects with high functioning pyridoxine-dependent epilepsy and age-matched control subjects.
Four male subjects with pyridoxine-dependent epilepsy (mean age 31 years 8 months, standard deviation 12 years 3 months) and age-matched control subjects (32 years 4 months, standard deviation 13 years) were recruited to participate in the study. Diffusion tensor data were collected and postprocessed in Functional Magnetic Resonance Imaging of the Brain Software Library to quantify corpus callosum tracts as a means to assess white matter structure. Task-based fMRI data were collected and Functional Magnetic Resonance Imaging of the Brain Software Library used to assess task response. The fMRI resting-state data were analyzed with the functional connectivity toolbox Conn to determine functional connectivity.
Subjects with high functioning pyridoxine-dependent epilepsy retained structural white matter connectivity compared with control subjects, despite morphologic differences in the posterior corpus callosum. fMRI task-based results did not differ between subjects with pyridoxine-dependent epilepsy and control subjects; functional connectivity as measured with resting-state fMRI was lower in subjects with pyridoxine-dependent epilepsy for several systems (memory, somatosensory, auditory).
Although corpus callosum morphology is diminished in the posterior portions, structural connectivity was retained in subjects with pyridoxine-dependent epilepsy, while functional connectivity was diminished for memory, somatosensory, and auditory systems.
在这项观察性研究中，对4名高功能吡哆醇依赖性癫痫患者及年龄匹配的对照受试者的白质结构、基于功能磁共振成像（fMRI）任务的反应以及功能连接性进行了评估。
招募了4名吡哆醇依赖性癫痫男性受试者（平均年龄31岁8个月，标准差12岁3个月）及年龄匹配的对照受试者（32岁4个月，标准差13岁）参与研究。在脑功能磁共振成像软件库中收集并后处理扩散张量数据，以量化胼胝体束，作为评估白质结构的一种手段。收集基于任务的fMRI数据，并使用脑功能磁共振成像软件库评估任务反应。使用功能连接工具箱Conn分析fMRI静息态数据，以确定功能连接性。
与对照受试者相比，高功能吡哆醇依赖性癫痫患者保留了白质结构连接性，尽管胼胝体后部存在形态学差异。基于fMRI任务的结果在吡哆醇依赖性癫痫患者和对照受试者之间没有差异；在几个系统（记忆、体感、听觉）中，吡哆醇依赖性癫痫患者静息态fMRI测量的功能连接性较低。
尽管胼胝体后部形态减小，但吡哆醇依赖性癫痫患者保留了结构连接性，而记忆、体感和听觉系统的功能连接性降低。
