Purpose: Cerebellar mutism syndrome (CMS) represents a major complication affecting many children that undergo surgery for posterior fossa lesions. Etiology and pathophysiology are still not fully understood. CMS deeply influences quality of life and recovery of these patients. An effective treatment has not been defined yet. This case-based review aims at analyzing the available evidence and knowledge to better delineate this phenomenon and to determine whether CMS can be successfully treated with pharmacological therapy.
Methods: Systematic research and retrieval of databases were conducted analyzing all papers where medical treatment of CMS was reported. A summary of the latest understanding and reports regarding definition, clinical manifestations, pathophysiology, management, and outcome of CMS has been conducted.
Results: Consensus on definition of this syndrome is lacking. CMS is the term accepted by the Posterior Fossa Society in 2016. Pathophysiology is still poorly understood but the most likely mechanism is injury along proximal components of the efferent cerebellar pathway. Nine papers describing positive effects of pharmacological therapy for CMS have been identified. Fluoxetine, zolpidem, bromocriptine, and midazolam are the drugs that seem to alleviate symptoms of CMS and improve recovery. To date, cognitive rehabilitation and physiotherapy are the only treatment options available.
Conclusion: CMS has deep impact on affected children and their families. Despite attempts to identify preventive measures and treatment, cases still occur on a regular basis. Pharmacological treatments have been proposed to help reduce the symptoms of CMS with some promising results, but reports are limited; therefore, further studies are needed.
Keywords: Cerebellar dysfunction; Mutism; Pharmacological therapy; Posterior fossa surgery.
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.