Dataset Information

Characterizing Posterior Fossa Syndrome: A Survey of Experts.

ABSTRACT:

Background

Despite recent efforts, disagreement remains among frontline clinicians regarding the operational definition of a syndrome commonly referred to as posterior fossa syndrome or cerebellar mutism syndrome.

Methods

We surveyed experts in the clinical care of children with posterior fossa tumors to identify trends and discrepancies in diagnosing posterior fossa syndrome.

Results

All surveyed professionals conceptualized posterior fossa syndrome as a spectrum diagnosis. The majority agreed that mutism is the most important symptom for diagnosis. However, results highlighted ongoing discrepancies related to important features of posterior fossa syndrome.

Conclusions

Greater posterior fossa syndrome conceptual alignment among providers is needed to formulate specific diagnostic criteria that would further research and clinical care. The authors propose preliminary diagnostic criteria for posterior fossa syndrome that require refinement through careful clinical characterization and targeted empirical investigation.

SUBMITTER: Wickenhauser ME

PROVIDER: S-EPMC7010537 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

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Publications

Characterizing Posterior Fossa Syndrome: A Survey of Experts.

Wickenhauser Molly E ME Khan Raja B RB Raches Darcy D Ashford Jason M JM Robinson Giles W GW Russell Kathryn M KM Conklin Heather M HM

Pediatric neurology 20191129

<h4>Background</h4>Despite recent efforts, disagreement remains among frontline clinicians regarding the operational definition of a syndrome commonly referred to as posterior fossa syndrome or cerebellar mutism syndrome.<h4>Methods</h4>We surveyed experts in the clinical care of children with posterior fossa tumors to identify trends and discrepancies in diagnosing posterior fossa syndrome.<h4>Results</h4>All surveyed professionals conceptualized posterior fossa syndrome as a spectrum diagnosis ...[more]

PMID: 31911026

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Project description:Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined.This observational, prospective, multicentre study will include 500 children with posterior fossa tumours. It opened late 2014 with participation from 20 Nordic and Baltic centres. From 2016, five British centres and four Dutch centres will join with a total annual accrual of 130 patients. Three other major European centres are invited to join from 2016/17. Follow-up will run for 12 months after inclusion of the last patient. All patients are treated according to local practice. Clinical data are collected through standardized online registration at pre-determined time points pre- and postoperatively. Neurological status and speech functions are examined pre-operatively and postoperatively at 1-4 weeks, 2 and 12 months. Pre- and postoperative speech samples are recorded and analysed. Imaging will be reviewed centrally. Pathology is classified according to the 2007 WHO system. Germline DNA will be collected from all patients for associations between CMS characteristics and host genome variants including pathway profiles.Through prospective and detailed collection of information on 1) differences in incidence and clinical course of CMS for different patient and tumour characteristics, 2) standardized surgical data and their association with CMS, 3) diversities and results of other therapeutic interventions, and 4) the role of host genome variants, we aim to achieve a better understanding of risk factors for and the clinical course of CMS - with the ultimate goal of defining strategies for prevention and treatment of this severely disabling condition.Clinicaltrials.gov : NCT02300766 , date of registration: November 21, 2014.

Dataset Information

Characterizing Posterior Fossa Syndrome: A Survey of Experts.

Background

Methods

Results

Conclusions

Publications

Characterizing Posterior Fossa Syndrome: A Survey of Experts.

Similar Datasets

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