Posterior fossa syndrome—a narrative review

Posterior fossa syndrome (PFS), or cerebellar mutism syndrome (CMS), is a collection of neurological symptoms that occur following surgical resection of a posterior fossa tumour, and is characterised by either a reduction or an absence of speech. Some authors suggest that CM is only one symptom of the CMS complex that also includes ataxia, hypotonia and irritability as well as cranial nerve deficits, neurobehavioral changes and urinary retention or incontinence. It is seen almost exclusively in children. In 1985 Rekate et al published the first work describing CM as a clinical entity, occurring as a consequence of bilateral cerebellar injury. Other associated symptoms include visual impairment, altered mood, impaired swallowing and significant gross and fine motor deficits. The effects of this can have a devastating impact on both the patient and their carers, posing a significant clinical challenge to neurorehabilitation services. The reported incidence was between 8% and 31% of children undergoing surgery for posterior fossa tumour. The underlying pathologies include vasospasm, oedema, and axonal/neuronal injury. Neuroimaging has contributed to a better understanding of the anatomical location of postoperative injury. There have been a number of suggestions for treatment interventions for PFS. However, apart from some individual reports, there have been no clinical trials indicating possible benefit. Occupational therapy, speech and language therapy, as well as neurocognitive support, contribute to the recovery of these patients.