Posterior fossa syndrome—a narrative review

Abstract

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 . 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.

Abnormal Cerebellar Development in Autism Spectrum Disorders

“…Some children who have tumor resection surgery for medulloblastomas, a type of cerebellar tumor, also exhibit symptoms of CCAS [27] and some experience posterior fossa syndrome (PFS). PFS is characterized by a similar but often more severe constellation of symptoms including mutism, emotional lability, ataxia, hypotonia, and behavioral disturbances [28,29]. While the acute, severe presentation of PFS often resolves with time, children who experience PFS often suffer long-term neurocognitive impairment [30][31][32].…”

Preoperative and postoperative high angular resolution diffusion imaging tractography of cerebellar pathways in posterior fossa tumors

“…Posterior fossa syndrome (PFS) is a collection of neurological symptoms seen in 8%–32% cases after the tumor resection of the posterior fossa (Wahab et al, 2016; Yecies et al, 2019). PFS is also called cerebellar mutism, cerebellar speech syndrome, cerebellar mutism and subsequent dysarthria, and cerebellar cognitive affective syndrome (Yecies et al, 2019).…”

The outcome of using zolpidem for treatment of postoperative posterior fossa syndrome in children with brain tumor

“…[2,3] Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome, is a common and often a transient complication after surgical resection of posterior fossa tumors in children with an incidence rate ranging from 2%–50%. [4,5]…”

“…[2,3] Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome, is a common and often a transient complication after surgical resection of posterior fossa tumors in children with an incidence rate ranging from 2%-50%. [4,5] The signs and symptoms are diverse and have different clinical presentation, with the hallmark characteristics of PFS being mutism which has a great psychosocial impact on patients and their families and may lead to long-term cognitive morbidity. [6,7] The average time to develop the PFS signs and symptoms ranged from 24 to 107 hours after surgery and can resolve within weeks to months of symptom onset while some patients may continue to have persistent symptoms.…”