Neurology Unit, Stroke Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Via Amendola 2, 42122, Reggio Emilia, Italy.
Neuroradiology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Via Amendola 2, 42122, Reggio Emilia, Italy.
Neurol Sci. 2024 Sep;45(9):4161-4171. doi: 10.1007/s10072-024-07571-z. Epub 2024 May 9.
Cerebellar mutism syndrome (CMS) is a frequent complication of surgical intervention on posterior fossa in children. It has been only occasionally reported in adults and its features have not been fully characterized. In children and in young adults, medulloblastoma is the main reason for neurosurgery. A single case of postsurgical CMS is presented in an adult patient with a cerebellar hemorrhage and a systematic review of the published individual cases of CMS in adults was done. Literature review of individual cases found 30 patients, 18/30 (60%) males, from 20 to 71 years at diagnosis. All but one case was post-surgical, but in one of the post-surgical cases iatrogenic basilar artery occlusion was proposed as cause for CMS. The causes were: primary tumors of the posterior fossa in 16/22 (72.7%) metastasis in 3/30 (10%), ischemia in 3/30 (10%) cerebellar hemorrhage in 3/30 (10%), and benign lesions in 2/30 (6.7%) patients. 8/30 patients (26.7%) were reported as having persistent or incomplete resolution of CMS within 12 months. CMS is a rare occurrence in adults and spontaneous cerebellar hemorrhage has been reported in 3/30 (10%) adult patients. The generally accepted hypothesis is that CMS results from bilateral damage to the dentate nucleus or the dentate-rubro-thalamic tract, leading to cerebro-cerebellar diaschisis. Several causes might contribute in adults. The prognosis of CMS is slightly worse in adults than in children, but two thirds of cases show a complete resolution within 6 months.
小脑缄默症(CMS)是儿童后颅窝手术干预的常见并发症。它在成人中仅偶尔被报道,其特征尚未完全描述。在儿童和年轻成人中,髓母细胞瘤是神经外科的主要原因。本文报告了一例成人小脑出血术后 CMS,并对已发表的成人 CMS 个案进行了系统回顾。对个案文献的回顾发现了 30 例患者,18/30(60%)为男性,诊断时年龄为 20 至 71 岁。除了一例外,所有患者均为术后,而在一例术后病例中,医源性基底动脉闭塞被认为是 CMS 的原因。病因包括:后颅窝原发性肿瘤 16/22(72.7%)、转移瘤 3/30(10%)、缺血 3/30(10%)、小脑出血 3/30(10%)和良性病变 2/30(6.7%)。8/30 例(26.7%)患者在 12 个月内 CMS 持续或不完全缓解。CMS 在成人中罕见发生,3/30(10%)成人患者报告自发性小脑出血。普遍接受的假说认为 CMS 是由于齿状核或齿状红核丘脑束双侧损伤引起的,导致脑-小脑失联络。成人可能有多种原因。CMS 在成人中的预后略差于儿童,但三分之二的病例在 6 个月内完全缓解。