post-operativE Pediatric Cerebellar Mutism Syndrome

In 2015 the Board of the Posterior Fossa Society initiated and international consensus process in order to create formal definition of the clinical entities commonly known as cerebellar mutism (CM) and posterior fossa syndrome (PFS) [1, 2], as seen in children after posterior fossa tumor surgery. This resulted in a new definition of Post-operative Pediatric Cerebellar Mutism Syndrome:

"Post-operative pediatric CMS is characterized by delayed onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children. Additional common features include hypotonia and oropharyngeal dysfunction/dysphagia. It may frequently be accompanied by the cerebellar motor syndrome, cerebellar cognitive affective syndrome  and brain stem dysfunction including long tract signs and cranial neuropathies. The mutism is always transient, but recovery from CMS may be prolonged. Speech and language may not return to normal, and other deficits of cognitive, affective and motor function often persist." [3]

See definitions for further specification of individual phrases in the new CMS definition, and description of older terms that have often been used in relation to this condition.

Cerebellar cognitive affective syndrome

The Cerebellar Cognitive Affective Syndrome (CCAS) refers to a persistent pattern of executive, visual-spatial, affective and language-related symptoms most commonly described in adults with a wide range of lesions and diseases of the cerebellum [4]. It can also be observed in children [5].

Post-operative Pedriatic CMS versus CCAS

Per definition, those affected by post-operative pediatric CMS always become mute or have severely reduced speech output, and are frequently affected by motor deficits that can be either cerebellar in origin (e.g. hypotonia, ataxia) or brainstem-related (e.g. hemiparesis, cranial nerve palsies). In contrast, those affected by the CCAS do not become mute or have motor problems, and the anatomical substrate of their symptoms is confined to the cerebellum. In reality, however, many have cerebellar motor symptoms too, since the CCAS is often observed in people with a variety of neurological leasions that affect motor function. Both syndromes can be said to represent the same pattern of cerbellar dysfunction, occurring either acutely after surgery and designated post-operative pediatric CMS, or persistently over time and termed the CCAS [6]. The main differences have to do with surgery, mutism and brainstem involvement.

For information on definitions, incidence, symptoms, anatomy, pathophysiology, imaging findings, risk factors, prognosis, treatment and prevention, see individual pages under the tabs above.

Posterior Fossa Society

The Posterior Fossa Society was founded by Thora Gudrunardottir together with Elizabeth Wells and Karin Walsh in 2014. Thora was the society's first president from 2014-2016. The Posterior Fossa Society is an international group of researchers and health care professionals (doctors, nurses, psychologists, speech pathologists, linguists, neuroscientists etc.) who are dedicated to research into the causes, features, treatment and prevention of the post-operative pediatric CMS and CCAS in children and adults. It reaches its goals by systematically gathering and exchanging information both online and during international meetings and conferences

Further Information

The Posterior Fossa Society has gotten it’s own website. Check out

www.posteriorfossasociety.org

2021 conference

The Posterior Fossa Society will be hosting an open state-of-the-art conference on pediatric post-operative CMS and the CCAS in Liverpool, 11-13 June 2021. See here for further details. For marketing purposes the terms Cerebellar Mutism and Posterior Fossa Syndrome are used in some of the advertising material. Make no mistake - this is referring to the very same pediatric post-operative CMS that is featured on this webpage. I will be giving a talk at the conference - looking forward to seeing you there!

Disclaimer: This website was created by Thora Gudrunardottir, MD, and the information published here represents the gathered results of her own research and review of the litterature only. It does not reflect the views of any particular hospital, institute, professional society or organization. It cannot be guaranteed that all the information is 100% up to date all the time, although every effort is made to keep it that way.

1. Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K (2011) Cerebellar mutism : Review of the literature. Childs Nerv Syst 27(3):355-63

2. Gudrunardottir T, De Smet H, Bartha-Döring L, Van Dun K, Verhoeven J, Paquier P, Mariën P. Posterior Fossa Syndrome and Cerebellar Mutism. Chapter in: Mariën P, Monti M (eds). The Linguistic Cerebellum, 1st edn. Academic Press (Elsevier), Oxford, UK,  pp 257-281

3. Gudrunardottir T, Morgan AT, Lux AL, Walker DA, Walsh KS, Wells EM, Wisoff JH, Juhler M, Schmahmann JD, Keating RF, Catsman-Berrevoets C, for the Iceland Delphi Group (2016) Consensus Paper on Post-operative Pediatric Cerebellar Mutism Syndrome: The Iceland Delphi Results. Childs Nerv Syst May 3. [Epub]

4. Schmahmann, J. D. & Sherman, J. C. (1998). The cerebellar cognitive affective syndrome. Brain, 121 ( Pt 4), 561-579

5. Catsman-Berrevoets, C. E. & Aarsen, F. K. (2010). The spectrum of neurobehavioural deficits in the Posterior Fossa Syndrome in children after cerebellar tumour surgery. Cortex, 46, 933-946

6. Turkel, S. B., Shu, C. L., Nelson, M. D., Hyder, D., Gilles, F. H., Woodall, L. et al. (2004). Case series: acute mood symptoms associated with posterior fossa lesions in children. J.Neuropsychiatry Clin.Neurosci., 16, 443-445