Timmers Inge, van den Hurk Job, Hofman Paul Am, Zimmermann Luc Ji, Uludağ Kâmil, Jansma Bernadette M, Rubio-Gozalbo M Estela
Department of Pediatrics, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Cognitive Neuroscience, Maastricht University, Maastricht, The Netherlands.
Department of Cognitive Neuroscience, Maastricht University, Maastricht, The Netherlands; Maastricht Brain Imaging Center (M-BIC), Maastricht, The Netherlands; Laboratory of Biological Psychology, University of Leuven, Leuven, Belgium.
Brain Res. 2015 Aug 7;1616:166-76. doi: 10.1016/j.brainres.2015.05.007. Epub 2015 May 13.
Patients with the inherited metabolic disorder classic galactosemia have language production impairments in several planning stages. Here, we assessed potential deviations in recruitment and connectivity across brain areas responsible for language production that may explain these deficits. We used functional magnetic resonance imaging (fMRI) to study neural activity and connectivity while participants carried out a language production task. This study included 13 adolescent patients and 13 age- and gender-matched healthy controls. Participants passively watched or actively described an animated visual scene using two conditions, varying in syntactic complexity (single words versus a sentence). Results showed that patients recruited additional and more extensive brain regions during sentence production. Both groups showed modulations with syntactic complexity in left inferior frontal gyrus (IFG), a region associated with syntactic planning, and in right insula. In addition, patients showed a modulation with syntax in left superior temporal gyrus (STG), whereas the controls did not. Further, patients showed increased activity in right STG and right supplementary motor area (SMA). The functional connectivity data showed similar patterns, with more extensive connectivity with frontal and motor regions, and restricted and weaker connectivity with superior temporal regions. Patients also showed higher baseline cerebral blood flow (CBF) in right IFG and trends towards higher CBF in bilateral STG, SMA and the insula. Taken together, the data demonstrate that language abnormalities in classic galactosemia are associated with specific changes within the language network. These changes point towards impairments related to both syntactic planning and speech motor planning in these patients.
患有遗传性代谢紊乱经典半乳糖血症的患者在几个语言生成阶段存在语言障碍。在此，我们评估了负责语言生成的脑区在神经募集和连接方面的潜在偏差，这些偏差可能解释了这些缺陷。我们使用功能磁共振成像（fMRI）来研究参与者执行语言生成任务时的神经活动和连接情况。这项研究包括13名青少年患者和13名年龄及性别匹配的健康对照者。参与者在两种句法复杂度不同的条件下（单个单词与句子）被动观看或主动描述一个动画视觉场景。结果显示，患者在句子生成过程中募集了更多且更广泛的脑区。两组在与句法规划相关的左额下回（IFG）以及右岛叶中均表现出句法复杂度的调节。此外，患者在左上颞回（STG）中表现出句法调节，而对照组则没有。此外，患者在右STG和右辅助运动区（SMA）中活动增加。功能连接数据显示出类似的模式，与额叶和运动区域的连接更广泛，与上颞叶区域的连接受限且较弱。患者在右IFG中的基线脑血流量（CBF）也更高，并且在双侧STG、SMA和岛叶中有CBF升高的趋势。综上所述，数据表明经典半乳糖血症中的语言异常与语言网络内的特定变化有关。这些变化表明这些患者在句法规划和言语运动规划方面均存在损伤。
