Department of Neurosurgery, University Hospital Carl Gustav Carus, Technical University of Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
Neurosurg Rev. 2010 Oct;33(4):401-8. doi: 10.1007/s10143-010-0278-1. Epub 2010 Jul 29.
Cerebellar mutism (CM) is a rare and severe form of speech and language impairment, mostly diagnosed in children and adolescents and rarely reported in adults. We here review the literature and summarize all anatomical structures related to the pathogenesis of this rare syndrome. We also report two illustrative cases of CM following surgical treatment of Lhermitte-Duclos disease (LDD; dysplastic gangliocytoma) in two adult patients. LDD is a rare benign cerebellar tumor. Surgical excision appears to be the only effective treatment. However, surgery is hampered by the difficulty to distinguish between tumor and healthy cerebellar tissue, which may result in extensive resection and cause neurological deficits such as CM. A review of the literature and our two cases suggest that lesions or functional impairment of paravermian structures including dentate nuclei, vermis, lateral hemispheres, and cerebellocortical pathways contribute to the development of CM. However, there is no single anatomical structure identified to be associated with CM. It is unknown whether some diseases such as LDD carry a higher risk of postoperative CM than others. As illustrated by our two cases, although there are no special means, optimal preoperative diagnosis might contribute to the prevention of this syndrome. Despite the severity, CM carries a favorable prognosis and generally resolves within a few months.To conclude, we review the clinical signs and particularly the pathophysiological observations and anatomical structures affected in the development of postoperative CM and contribute two cases illustrating the pathogenesis, prognosis, and possible prevention of this syndrome, to focus that CM might also occur in adults even in association with rare tumors.
小脑缄默症（CM）是一种罕见且严重的言语和语言障碍形式，主要在儿童和青少年中诊断，在成年人中很少见。我们在此回顾文献并总结与这种罕见综合征发病机制相关的所有解剖结构。我们还报告了两例成人 Lhermitte-Duclos 病（LDD；发育性神经节细胞瘤）手术后出现 CM 的病例。LDD 是一种罕见的良性小脑肿瘤。手术切除似乎是唯一有效的治疗方法。然而，由于难以区分肿瘤和健康的小脑组织，手术受到阻碍，这可能导致广泛切除并导致神经功能缺损，如 CM。文献复习和我们的两例病例表明，矢状旁结构（包括齿状核、蚓部、外侧半球和小脑皮质通路）的病变或功能障碍可能导致 CM 的发生。然而，没有一个单一的解剖结构与 CM 相关。目前尚不清楚某些疾病（如 LDD）是否比其他疾病术后发生 CM 的风险更高。正如我们的两例病例所示，尽管没有特殊手段，但最佳的术前诊断可能有助于预防这种综合征。尽管严重，CM 具有良好的预后，通常在几个月内缓解。总之，我们回顾了术后 CM 发展过程中的临床体征，特别是病理生理学观察和受影响的解剖结构，并报告了两例阐明该综合征发病机制、预后和可能预防的病例，以强调 CM 也可能发生在成年人中，甚至与罕见肿瘤相关。
