Department of Neurology, Faculty of Medicine and Dentistry, University Hospital, Palacky University, I. P. Pavlova 6, 775 20, Olomouc, Czech Republic.
Department of Neurology and Psychiatry, The Neuroscience Institute, University of Santo Tomás Hospital, Manila, Philippines.
J Neural Transm (Vienna). 2021 Apr;128(4):509-519. doi: 10.1007/s00702-021-02310-6. Epub 2021 Feb 16.
The complex phenomenological understanding of dystonia has transcended from the clinics to genetics, imaging and neurophysiology. One way in which electrophysiology will impact into the clinics are cases wherein a dystonic clinical presentation may not be typical or a "forme fruste" of the disorder. Indeed, the physiological imprints of dystonia are present regardless of its clinical manifestation. Underpinnings in the understanding of dystonia span from the peripheral, segmental and suprasegmental levels to the cortex, and various electrophysiological tests have been applied in the course of time to elucidate the origin of dystonia pathophysiology. While loss of inhibition remains to be the key finding in this regard, intricacies and variabilities exist, thus leading to a notion that perhaps dystonia should best be gleaned as network disorder. Interestingly, the complex process has now spanned towards the understanding in terms of networks related to the cerebellar circuitry and the neuroplasticity. What is evolving towards a better and cohesive view will be neurophysiology attributes combined with structural dynamic imaging. Such a sound approach will significantly lead to better therapeutic modalities in the future.
扭转痉挛的复杂现象学理解已经超越了临床范畴,深入到遗传学、影像学和神经生理学领域。电生理学将对临床产生影响的一种情况是,扭转痉挛的临床表现可能不典型,或者是该疾病的“顿挫型”。事实上,无论其临床表现如何,扭转痉挛的生理印记都存在。对扭转痉挛的理解基础从外周、节段和超节段水平到皮层都有涉及,并且随着时间的推移,已经应用了各种电生理学测试来阐明扭转痉挛病理生理学的起源。虽然抑制丧失仍然是这方面的关键发现,但存在复杂性和可变性,因此导致一种观点,即扭转痉挛最好被视为网络障碍。有趣的是,这个复杂的过程现在已经扩展到理解与小脑回路和神经可塑性相关的网络。朝着更好的、有凝聚力的观点发展的将是结合结构动态成像的神经生理学特征。这种合理的方法将显著导致未来更好的治疗方式。