Lee Sekwang, Na Yoon Hye, Moon Hyun Im, Tae Woo Suk, Pyun Sung-Bom
Department of Physical Medicine and Rehabilitation, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea.
Department of Biomedical Sciences, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2017 Dec;41(6):1076-1081. doi: 10.5535/arm.2017.41.6.1076. Epub 2017 Dec 28.
Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech due to cerebellar lesions. CM is often reported in children. We describe a rare case of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability, decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging revealed significant volume reduction of medial frontal projection fibers from the corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps major and minor and bilateral cingulum-angular bundles were not visualized. Cerebello-frontal pathway reconstructed from the FMRIB Software Library showed continuity of fibers, with decreased number of fibers on qualitative analysis. These results suggest that cerebello-frontal disconnection may be a neuroanatomical mechanism of CM. Damage of brain network between occipital lobe, cingulate and cerebellum caused by hemorrhage may also have role in the mechanism of CM in our case.
小脑缄默症(CM)是一种罕见的神经系统疾病,其特征是由于小脑病变导致言语缺失。CM在儿童中常有报道。我们描述了一例自发性小脑出血后发生CM的罕见病例。该患者表现为缄默、易怒、自发运动减少和口咽失用症。弥散张量成像显示胼胝体内侧额叶投射纤维体积显著减小。在基于解剖结构的纤维束示踪(TRACULA)分析中,未显示出胼胝体的主要和次要钳状束以及双侧扣带回-角回束。从FMRIB软件库重建的小脑-额叶通路显示纤维连续,定性分析显示纤维数量减少。这些结果表明,小脑-额叶分离可能是CM的神经解剖学机制。在我们的病例中,出血导致的枕叶、扣带回和小脑之间脑网络的损伤也可能在CM的发病机制中起作用。