Incidence, Risks, and Sequelae of Posterior Fossa Syndrome in Pediatric Medulloblastoma

Korah, M.P.; Esiashvili, Natia; Mazewski, Claire; Hudgins, Roger J.; Tighiouart, Mourad; Janss, Anna J.; Schwaibold, Frederick P.; Crocker, Ian; Curran, Walter J.; Marcus, Robert B.

doi:10.1016/j.ijrobp.2009.04.058

Cited by 106 publications

(77 citation statements)

References 37 publications

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“…The incidence of CMS has been reported to range from 8% 46 to 39%, 9,15,40,52,55,62,82,108 although there are reviews reporting a lower rate of 1.6%. 17 In the largest prospective study so far of 450 children who underwent surgery for medulloblastomas, the incidence of CMS was found to be 24%.…”

Section: Incidencementioning

confidence: 99%

“…More extensive tumor resection along with better overall outcome in children who undergo posterior fossa tumor resections can increase the rate of CMS. 76 Korah et al 52 noted that in 2 different periods of time (1990-2000 and 2001-2007) the percentage of gross-total resection or near-total resection among children who underwent medulloblastoma resection was 83% and 93%, respectively, while the incidence of PFS was 17% and 39%, respectively. In addition, all patients with PFS underwent a gross-total resection and there was no radiological evidence of residual tumor.…”

Section: Incidencementioning

confidence: 99%

“…Brainstem involvement seems to be the most important, including preoperative tumor invasion or postoperative edema. 26,52,108 Moreover, McMillan et al 61 supported that brainstem compression could be used as a predictor of postoperative CM. Some authors believe that midline location predisposes to the occurrence of CM, 9,36,53,55 while others have found no significant difference.…”

Section: Risk Factorsmentioning

confidence: 99%

“…It usually appears as a consequence of posterior fossa surgery in children with cerebellar or fourth ventricle tumors. 52,82,108 Other causes that have been reported are infections, 15,19,24,26,72 posterior fossa hematomas (surgically removed 31,89 or not 60 ), arteriovenous malformations, 33,51,82 a brainstem tumor, 82 a pineal gland tumor, 23 and traumatic cerebellar injury. 15,82 Although this clinical entity is mainly seen in children, there are also reports of CM in adults.…”

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confidence: 99%

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Cerebellar mutism

Pitsika¹,

Tsitouras²

2013

PED

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Mutism of cerebellar origin is a well-described clinical entity that complicates operations for posterior fossa tumors, especially in children. This review focuses on the current understanding of principal pathophysiological aspects and risk factors, epidemiology, clinical characteristics, treatment strategies, and outcome considerations. The PubMed database was searched using the term cerebellar mutism and relevant definitions to identify publications in the English-language literature. Pertinent publications were selected from the reference lists of the previously identified articles. Over the last few years an increasing number of prospective studies and reviews have provided valuable information regarding the cerebellar mutism syndrome. Importantly, the clarification of principal terminology that surrounds the wide clinical spectrum of the syndrome results in more focused research and more effective identification of this entity. In children who undergo surgery for medulloblastoma the incidence of cerebellar mutism syndrome was reported to be 24%, and significant risk factors so far are brainstem involvement and midline location of the tumor. The dentate-thalamo-cortical tracts and lesions that affect their integrity are considered significant pathophysiological issues, especially the tract that originates in the right cerebellar hemisphere. Moderate and severe forms of the cerebellar mutism syndrome are the most frequent types during the initial presentation, and the overall neurocognitive outcome is not as favorable as thought in the earlier publications. Advanced neuroimaging techniques could contribute to identification of high-risk patients preoperatively and allow for more effective surgical planning that should focus on maximal tumor resection with minimal risk to important neural structures. Properly designed multicenter trials are needed to provide stronger evidence regarding effective prevention of cerebellar mutism and the best therapeutic approaches for such patients with a combination of pharmacological agents and multidisciplinary speech and behavior augmentation.

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Section: Incidencementioning

confidence: 99%

Section: Incidencementioning

confidence: 99%

Section: Risk Factorsmentioning

confidence: 99%

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confidence: 99%

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Cerebellar mutism

Pitsika¹,

Tsitouras²

2013

PED

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“…The incidence of acute presentation of mutism, dysarthria and dysphagia post-surgery as relatively high, affecting around one in three cases [51,52]. Brain stem invasion, midline tumor location, younger age, absence of radiographic residual tumor [53], histopathologic diagnosis and socioeconomic level of the patient's family [52] are all important risk factors for the development of PFS. The most common feature is mutism, but oropharyngeal dysprexia, emotional lability and neuropsychiatric symptoms occur.…”

Section: Cerebellar Mutismmentioning

confidence: 99%

Autism and Cerebellar Mutism: A Review of the Neuroanatomical Findings

Kaya¹

2012

Autism

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Autistic Spectrum Disorder (ASD) is behaviorally defined syndrome characterized by atypical social interaction, disordered verbal and non-verbal communication, restricted areas of interest and limited imaginative play. The prevalence of ASD is considered to be approximately 4 to 5 per 1000, but numbers differ according to criteria used for diagnosis [1].Individuals with ASD have three core features; 1. Impairments of reciprocal social interactions, 2. Abnormal development and use of language and 3. Repetitive and ritualized behaviors and a narrow range of interests. In addition to the core features of autism, there are common co-morbid neurological disorders. The prevalence of mental retardation in the autism spectrum is approximately 30%. Epilepsy prevalence associated with autism varies from 5% to 44%. Anxiety and mood disorders are also very common in autism.There is heterogeneity in the onset of autism. Although some children have signs of development delays between 0-18 months of life, up to 40% of children with ASD initially demonstrate the near-normal development until 18-24 months.As Brunelle et al. says: All the results are converging toward the description of anatomical and functional anomalies in the regions of the so-called "social brain" [2]. Several brain regions such as frontal lobe, the superior temporal cortex, the parietal cortex, and amygdala have been implicated in social behavior. But the recent developments make us think more about the role of cerebellum in ASD. This review presents an overview of the neuroanatomical abnormalities that occur within ASD. We discuss the findings that have advanced our understanding of cerebellar organization in ASD.

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Posterior fossa syndrome: Review of the behavioral and emotional aspects in pediatric cancer patients

Lanier

Abrams

2016

Cancer

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Medulloblastoma, the most common malignant brain tumor of childhood, occurs in the posterior fossa, the part of the intracranial cavity that contains the brainstem and the cerebellum. The cerebellum is involved in many complex aspects of human behavior and function, and when it is disrupted or insulted, this can lead to significant sequelae in children with posterior fossa tumors. A constellation of impairing and distressing symptoms, including mutism, ataxia/hypotonia, and emotional lability, develops in approximately 25% of children after the surgical resection of posterior fossa tumors. These symptoms may impede treatment and frequently require intervention in order for children to be able to participate in their care. The eventual recovery of speech occurs for most, but with slowly improving dysarthria over many months. Behavioral changes and emotional lability also occur. This phenomenon has been classified differently by different investigators over the past 35 years. For the purposes of this article, the term posterior fossa syndrome is used to refer to the neuropsychiatric and behavioral features that compose this condition. The current review summarizes the development of the clinical understanding of this phenomenon with a focus on near- and long-term psychosocial and psychiatric implications. Also, clinical examples of the presentation, management, and lasting implications of this syndrome are provided. This review is intended to be a resource for clinicians who treat affected children. Cancer 2017;123:551-559. © 2016 American Cancer Society.

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Incidence, Risks, and Sequelae of Posterior Fossa Syndrome in Pediatric Medulloblastoma

Cited by 106 publications

References 37 publications

Cerebellar mutism

Cerebellar mutism

Autism and Cerebellar Mutism: A Review of the Neuroanatomical Findings

Posterior fossa syndrome: Review of the behavioral and emotional aspects in pediatric cancer patients

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