Paquier Philippe F, Walsh Karin S, Docking Kimberley M, Hartley Helen, Kumar Ram, Catsman-Berrevoets Coriene E
Department of Neuropsychology, University Hospital Erasme, Université Libre de Bruxelles (ULB), Brussels, Belgium.
Clinical and Experimental Neurolinguistics, Center for Linguistics (CLIN), Vrije Universiteit Brussel (VUB), Brussels, Belgium.
Childs Nerv Syst. 2020 Jun;36(6):1215-1222. doi: 10.1007/s00381-019-04229-6. Epub 2019 Jun 20.
Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase.
The impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular.
小脑肿瘤是儿童最常见的脑肿瘤。现代治疗方法和积极的手术提高了总体生存率。因此，越来越多的幸存者面临着出现不良和长期神经功能缺损的高风险，包括认知、行为、言语和语言方面的缺损。术后小脑缄默综合征（pCMS）是儿童小脑肿瘤手术中一种广为人知且经常发生的并发症。在急性期，患有pCMS的儿童可能会出现小脑运动功能恶化以及锥体束和颅神经病变。最使人衰弱的是缄默或言语严重减少以及可能出现的一系列神经行为症状。从长期来看，从pCMS中恢复的儿童比起在小脑肿瘤手术后未发生pCMS的儿童，仍然有更多的运动、行为和认知问题。这些长期后遗症的严重程度似乎与缄默期的长短有关。
pCMS对患者及其家庭的影响再怎么强调都不为过。本论文旨在讨论目前关于pCMS患儿的自然病程、恢复情况和康复的知识。我们提出了未来制定康复计划的优先事项，以提高小脑肿瘤手术后尤其是发生pCMS后的儿童的长期生活质量和参与度。
