Cerebellar Mutism Syndrome in Pediatric Neuro-oncology: A Multidisciplinary Perspective and Call for Research Priorities

Malbari, Fatema; Gill, Jason; Daigle, Amy; Rodriguez, Lisa L.; Raghubar, Kimberly P.; Davis, Kimberly C.; Scheurer, Michael E.; Marina, M.; Kralik, Stephen F.; Meoded, Avner; Okcu, M. Fatih; Chintagumpala, Murali; Aldave, Guillermo; Weiner, Howard L.; Kahalley, Lisa S.

doi:10.1016/j.pediatrneurol.2022.04.014

Cited by 8 publications

(9 citation statements)

References 80 publications

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“…As a previous study pointed out, there is no standardized clinical criterion for pCMS, so the rate of pCMS is varied in the literature. 36 In our study, 52 patients were not included in the analysis because the tumors were located at the cerebellar hemisphere, and none of these patients were diagnosed with pCMS. When including them, the rate of pCMS was 26.7%.…”

Section: Discussionmentioning

confidence: 99%

Postoperative MRI features of cerebellar mutism syndrome: a retrospective cohort study

Yang¹,

Zhang

Cai³

et al. 2022

Journal of Neurosurgery: Pediatrics

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OBJECTIVE In this study, the authors aimed to investigate the relationship between postoperative MRI features and cerebellar mutism syndrome. METHODS A retrospective cohort of patients who underwent tumor resection from July 2013 to March 2021 for midline posterior fossa tumors was investigated. All patients were followed up at least once. Clinical data were extracted from medical records and follow-up databases. Two neuroradiologists independently reviewed preoperative and postoperative MRI. Univariable and multivariable analyses were performed to compare the postoperative cerebellar mutism syndrome (pCMS) and non-pCMS groups. Correlation analysis was performed using the Spearman correlation coefficient analysis. RESULTS Of 124 patients, 47 (37.9%) developed pCMS. The median follow-up duration was 45.73 (Q1: 33.4, Q3: 64.0) months. The median duration of mutism was 45 days. The median tumor size was 48.8 (Q1: 42.1, Q3: 56.8) mm. In the univariable analysis, abnormal T2-weighted signal of the left dentate nucleus (DN) (74.5% in the pCMS group vs 36.4% in the non-pCMS group, p < 0.001), right DN (83.0% vs 40.3%, p < 0.001), left superior cerebellar peduncle (SCP) (74.5% vs 27.3%, p < 0.001), right SCP (63.8% vs 23.4%, p < 0.001), left middle cerebellar peduncle (MCP) (51.1% vs 26.0%, p = 0.008), and right MCP (61.7% vs 26.0%, p < 0.001); male sex (83.0% vs 45.5%, p < 0.001); vermis 3 impairment (49.4% vs 19.1%, p = 0.002); solid tumor (91.5% vs 72.7%, p = 0.022); and hydrocephalus (72.3% vs 45.5%, p = 0.006) were more frequent in the pCMS group than in the non-pCMS group. Multivariable logistic analysis showed that male sex (adjusted OR 4.08, p = 0.010) and the cerebro-cerebellar circuit score of T2-weighted images (adjusted OR 2.15, p < 0.001) were independent risk factors for pCMS. The cerebro-cerebellar circuit score positively correlated with the duration of mutism. In Cox regression analysis, the cerebro-cerebellar integrated circuit injury score of T2 (adjusted HR 0.790, 95% CI 0.637–0.980; p = 0.032) and injury of vermis 3 (adjusted HR 3.005, 95% CI 1.197–7.547; p = 0.019) were independently associated with the duration of mutism. CONCLUSIONS Male sex and cerebro-cerebellar circuit damage are independent risk factors for pCMS. The cerebro-cerebellar circuit score indicates the duration of mutism.

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Section: Discussionmentioning

confidence: 99%

Postoperative MRI features of cerebellar mutism syndrome: a retrospective cohort study

Yang¹,

Zhang

Cai³

et al. 2022

Journal of Neurosurgery: Pediatrics

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“…The term cerebellar mutism syndrome is universally used in order to describe the loss, transient or permanent, of speech that is intimately related to any kind of cerebellar insult. Initially, CMS was reported in 1958, referring to a child who was mute after resection of a tumor involving the posterior fossa posterior fossa• this entity was termed akinetic mutism [1,2]. The first relevant cases of postoperative mutism affecting pediatric population, which was a subsequent of surgery involving the structures of the posterior fossa, were recorded in the 1970s [3,4].…”

Section: Of 14mentioning

confidence: 99%

“…Based on the complexity of this entity, as well as on the fact that it incorporates multiple individual signs and symptoms, the term cerebellar mutism is frequently encountered in the current literature cerebellar mutism syndrome or posterior fossa syndrome. According to a recently published paper [1], this syndrome is characterized by a constellation of symptoms including mutism/reduced speech, emotional lability, cerebellar syndrome, brainstem dysfunction, hypotonia, and oropharyngeal dysfunction/dysphagia [9][10][11][12][13].…”

Section: Of 14mentioning

confidence: 99%

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

PANAGOPOULOS¹,

STRANJALIS²,

Gavra³

et al. 2022

Preprint

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Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, is encountered in a subset of children who have undergone an operative procedure of the posterior cranial fossa, mainly involving the vermis, and the most frequent underlying pathology is proved to be medulloblastoma. The most common characteristics of this syndrome include an often transient, although protracted, language impairment, emotional lability, along with cerebellar, and brainstem dysfunction. Nevertheless, a significant number of patients experience persistent neurological deficits and lasting neurocognitive impairment. A lot of research and clinical studies have been performed in order to better delineate this syndrome. The main obstacles in our way to highlight all aspects of this syndrome were related with an inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty surrounding risk factors and etiology. Currently, there is a combination of diagnostic criteria that are regarded as prerequisites in order to establish the diagnosis of CMS. These include language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement. Several risk factors are recognized as implicated in the pathogenesis of this syndrome, including midline tumor location, diagnosis of medulloblastoma, younger age at diagnosis, and preoperatively established language impairment. A proposed etiology of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Specific treatment for CMS is lacking, and it continues to be directed at symptom management. Our aim is to present a comprehensive narrative review of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. Moreover, we attempt to recognize the most widely recognized priorities of the research community in order to expand our knowledge in the era of diagnostics, prevention, and therapeutic options for patients suffering from CM, or who are at risk for development of this syndrome.

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“…The historically first report of this term (CMS) is encountered in 1958, referring to a pediatric patient who was unable to speak after resection of a space-occupying lesion that was involving the posterior fossa. This entity was termed akinetic mutism [1,2]. The first relevant cases of postoperative mutism affecting the pediatric population, which were an operation involving the structures of the posterior fossa, were recorded in the 1970s [3,4].…”

Section: Introductionmentioning

confidence: 99%

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

et al. 2022

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Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, is an entity that entails a constellation of signs and symptoms which are recorded in a limited number of pediatric patients who have been operated on mainly for tumors involving the posterior cranial fossa, and more precisely, the region of the vermis. Medulloblastoma seems to constitute the most commonly recognized pathological substrate, associated with this entity. The most prevalent constituents of this syndrome are noted to be a, often transient, although protracted, language impairment, emotional lability, along with cerebellar and brainstem dysfunction. Apart from that, a definite proportion of involved individuals are affected by irreversible neurological defects and long-lasting neurocognitive impairment. A bulk of literature and evidence based on clinical trials exist, which reflect the continuous effort of the scientific community to highlight all perspectives of this complex phenomenon. There are several circumstances that intervene in our effort to delineate the divergent parameters that constitute the spectrum of this syndrome. In summary, this is implicated by the fact that inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty regarding risk factors and etiology are all constituents of a non-well-investigated syndrome. Currently, a preliminary consensus exists about the identification of a group of diagnostic prerequisites that are managed as sine qua non, in our aim to document the diagnosis of CMS. These include language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement. It is common concept that midline tumor location, diagnosis of medulloblastoma, younger age at diagnosis, and preoperatively established language impairment should be accepted as the most determinant predisposing conditions for the establishment of this syndrome. A well-recognized pathophysiological explanation of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Despite the relative advancement that is recorded regarding the diagnostic section of this disease, no corresponding encouraging results are reported, regarding the available treatment options. On the contrary, it is mainly targeted toward the symptomatic relief of the affected individuals. The basic tenet of our review is centered on the presentation of a report that is dedicated to the definition of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. Apart from that, an effort is made that attempts to elucidate the paramount priorities of the scientific forum, which are directed toward the expansion our knowledge in the era of diagnostics, prevention, and therapeutic options for patients suffering from CM, or who are at risk for development of this syndrome.

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Cerebellar Mutism Syndrome in Pediatric Neuro-oncology: A Multidisciplinary Perspective and Call for Research Priorities

Cited by 8 publications

References 80 publications

Postoperative MRI features of cerebellar mutism syndrome: a retrospective cohort study

Postoperative MRI features of cerebellar mutism syndrome: a retrospective cohort study

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

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