IRCCS NEUROMED, Via Atinense, 18, 86077 Pozzilli, IS, Italy.
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Clin Neurophysiol. 2022 Feb;134:73-80. doi: 10.1016/j.clinph.2021.11.075. Epub 2021 Dec 21.
Oromandibular dystonia (OMD) is a rare form of focal idiopathic dystonia. OMD was clinically identified at the beginning of the 20th century, and the main clinical features have been progressively described over the years. However, OMD has several peculiarities that still remain unexplained, including the high rate of oral trauma, which is often related to the onset of motor symptoms. The purpose of this paper was to formulate a hypothesis regarding the pathophysiology of OMD, starting from the neuroanatomical basis of the masticatory and facial systems and highlighting the features that differentiate this condition from other forms of focal idiopathic dystonia. We provide a brief review of the clinical and etiological features of OMD as well as neurophysiological and neuroimaging findings obtained from studies in patients with OMD. We discuss possible pathophysiological mechanisms underlying OMD and suggest that abnormalities in sensory input processing may play a prominent role in OMD pathophysiology, possibly triggering a cascade of events that results in sensorimotor cortex network dysfunction. Finally, we identify open questions that future studies should address, including the effect of abnormal sensory input processing and oral trauma on the peculiar neurophysiological abnormalities observed in OMD.
口颌肌张力障碍(OMD)是一种罕见的局灶性特发性肌张力障碍。OMD 于 20 世纪初被临床识别,多年来其主要临床特征已逐步被描述。然而,OMD 具有一些仍未得到解释的特殊之处,包括高口腔创伤率,其常与运动症状的发生有关。本文旨在从咀嚼和面部系统的神经解剖学基础出发,针对 OMD 的病理生理学提出一个假说,并突出该病症与其他形式的局灶性特发性肌张力障碍的区别。我们简要回顾了 OMD 的临床和病因特征以及从 OMD 患者的神经生理学和神经影像学研究中获得的结果。我们讨论了 OMD 可能的病理生理学机制,并提出感觉输入处理异常可能在 OMD 病理生理学中起重要作用,可能引发一系列导致感觉运动皮层网络功能障碍的事件。最后,我们确定了未来研究应解决的开放性问题,包括异常感觉输入处理和口腔创伤对口颌肌张力障碍中观察到的特殊神经生理学异常的影响。