■脊髓小脑共济失调(SCA)表示常染色体显性小脑共济失调的扩展列表。尽管震颤是SCA临床频谱的重要方面,其患病率,现象学,和病理生理学是未知的。
■这篇评论旨在描述在不同SCA中看到的各种类型的震颤,讨论了震颤的病理生理学,以及可能的治疗方式。
■作者使用包括震颤和各种SCA在内的搜索词在PubMed上进行了文献检索。在排除重复出版物后,相关文章被纳入审查。
■虽然动作(姿势和意图)震颤最常与SCA相关,休息和其他罕见的地震也有记录。震颤的患病率和类型在不同的SCA之间有所不同。SCA12,在某些种族人群中很常见,呈现出一种独特的情况,震颤通常是主要表现。SCAs的临床表现可能与特发性震颤或帕金森病相混淆。SCA中震颤的病理生理学主要涉及小脑及其网络,尤其是小脑-丘脑-皮层回路.此外,与基底神经节的连接,和纹状体多巴胺能功能障碍可能有一定作用。震颤的医学管理通常由现象学和相关的临床特征指导。深部脑刺激手术可能有助于治疗难治性震颤。
■震颤是SCA的元素成分,不同的现象学,并强调小脑在震颤中的作用。进一步的研究将有助于描绘临床,病理生理学,和SCA中震颤的治疗方面。
UNASSIGNED: Spinocerebellar ataxia (SCA) denotes an expanding list of autosomal dominant cerebellar ataxias. Although tremor is an important aspect of the clinical spectrum of the SCAs, its prevalence, phenomenology, and pathophysiology are unknown.
UNASSIGNED: This
review aims to describe the various types of tremors seen in the different SCAs, with a discussion on the pathophysiology of the tremors, and the possible treatment modalities.
UNASSIGNED: The authors conducted a literature search on PubMed using search terms including tremor and the various SCAs. Relevant articles were included in the
review after excluding duplicate publications.
UNASSIGNED: While action (postural and intention) tremors are most frequently associated with SCA, rest and other rare tremors have also been documented. The prevalence and types of tremors vary among the different SCAs. SCA12, common in certain ethnic populations, presents a unique situation, where the tremor is typically the principal manifestation. Clinical manifestations of SCAs may be confused with essential tremor or Parkinson\'s disease. The pathophysiology of tremors in SCAs predominantly involves the cerebellum and its networks, especially the cerebello-thalamo-cortical circuit. Additionally, connections with the basal ganglia, and striatal dopaminergic dysfunction may have a role. Medical management of tremor is usually guided by the phenomenology and associated clinical features. Deep brain stimulation surgery may be helpful in treatment-resistant tremors.
UNASSIGNED: Tremor is an elemental component of SCAs, with diverse phenomenology, and emphasizes the role of the cerebellum in tremor. Further studies will be useful to delineate the clinical, pathophysiological, and therapeutic aspects of tremor in SCAs.