Chibawanye I Ene, Silva Elizabeth, Noll Kyle, Bradshaw Mariana, Connelly Katherine, Liu Ho-Ling, Tummala Ravi, Ferson David, Lang Frederick F, Kumar Vinodh A
Department of Neurosurgery, MD Anderson Cancer Center, Houston, Texas.
Department of Anesthesiology and Perioperative Medicine, MD Anderson Cancer Center, Houston, Texas.
J Neurosurg Case Lessons. 2025 May 5;9(18). doi: 10.3171/CASE2597.
Signed language integrates complex sensorimotor processes involving visual and motor networks. The contribution of language regions such as Broca's and Wernicke's areas during signing is not well established. Here, the authors report the case of a 45-year-old right-handed male with congenital deafness, who communicates using American Sign Language (ASL) and Signed English, presenting with focal seizures. Imaging showed a diffuse infiltrative glioma in the right cerebral hemisphere.
Remarkably, preoperative functional MRI language activations were bihemispheric and overlapped for both traditional language paradigms and sign paradigms, suggesting that the same cortical regions of the brain are used for language processing in this patient. This case is unique because it is the first reported awake craniotomy performed in a deaf patient with a right hemispheric glioma. The authors also found that direct cortical stimulation of the right premotor cortex (Exner's area) resulted in interruption of signing, which has not been previously reported in the literature in a deaf patient.
Prior to surgery for brain lesions in deaf patients, careful preoperative assessment and intraoperative testing (including the presence of an experienced ASL sign interpreter) is required to understand the distribution of language processing within the brain and to minimize postoperative morbidity. https://thejns.org/doi/10.3171/CASE2597.
手语整合了涉及视觉和运动网络的复杂感觉运动过程。在使用手语时,诸如布洛卡区和韦尼克区等语言区域的作用尚未明确。在此,作者报告了一名45岁的右利手男性先天性耳聋患者的病例,该患者使用美国手语(ASL)和手语英语进行交流,出现局灶性癫痫发作。影像学检查显示右侧大脑半球有弥漫性浸润性胶质瘤。
值得注意的是,术前功能磁共振成像语言激活在双侧大脑半球,并且在传统语言范式和手语范式中均有重叠,这表明该患者大脑中相同的皮质区域用于语言处理。该病例独特之处在于,这是首例报道的对患有右侧半球胶质瘤的耳聋患者进行的清醒开颅手术。作者还发现,直接皮质刺激右侧运动前皮质(埃克斯纳区)会导致手语中断,这在耳聋患者的文献中此前尚未有报道。
在为耳聋患者进行脑部病变手术前,需要进行仔细的术前评估和术中测试(包括要有经验丰富的美国手语翻译在场),以了解大脑内语言处理的分布情况,并将术后发病率降至最低。https://thejns.org/doi/10.3171/CASE2597 。