This study reviews the fundamental roles of pre–supplementary motor area (SMA) and SMA-proper responsible for speech-motor functions and auditory perception in succinic semialdehyde dehydrogenase (SSADH) deficiency. We comprehensively searched the databases of PubMed, Google Scholar, and the electronic journals Springer, PreQuest, and Science Direct associated with keywords SSADHD, SMA, auditory perception, speech, and motor with AND operator. Transcranial magnetic stimulation emerged for assessing excitability/inhibitory M1 functions, but its role in pre-SMA and SMA proper dysfunction remains unknown. There was a lack of data on resting-state and task-based functional magnetic resonance imaging (MRI), with a focus on passive and active tasks for both speech and music, in terms of analysis of SMA-related cortex and its connections. Children with SSADH deficiency likely experience a dysfunction in connectivity between SMA portions with cortical and subcortical areas contributing to disabilities in speech-motor functions and auditory perception. Early diagnosis of auditory-motor disabilities in children with SSADH deficiency by neuroimaging techniques invites opportunities for utilizing sensory-motor integration as future interventional strategies.

本研究回顾了在琥珀酸半醛脱氢酶 (SSADH) 缺乏症中负责言语运动功能和听觉感知的预补充运动区 (SMA) 和 SMA-proper 的基本作用。我们全面检索了 PubMed、Google Scholar 和电子期刊 Springer、PreQuest 和 Science Direct 的数据库中与关键字SSADHD、SMA、听觉感知、言语和运动相关的数据库使用 AND 运算符。经颅磁刺激出现用于评估兴奋性/抑制性 M1 功能，但其在前 SMA 和 SMA 适当功能障碍中的作用仍然未知。在分析 SMA 相关皮层及其连接方面，缺乏关于静息状态和基于任务的功能性磁共振成像 (MRI) 的数据，重点是语音和音乐的被动和主动任务。患有 SSADH 缺陷的儿童可能会经历 SMA 部分与皮质和皮质下区域之间的连接功能障碍，从而导致言语运动功能和听觉感知障碍。通过神经影像学技术对 SSADH 缺乏症儿童的听觉运动障碍进行早期诊断为利用感觉运动整合作为未来干预策略提供了机会。