Adaptive, behavioral, and emotional outcomes following postoperative pediatric cerebellar mutism syndrome in survivors treated for medulloblastoma

Kimberly P. Raghubar Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Andrew M. Heitzer Psychology Department, St. Jude Children’s Research Hospital, Memphis, Tennessee;

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Fatema Malbari Section of Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Jason Gill Section of Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Roy V. Sillitoe Section of Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Livia Merrill Department of Psychology, University of Houston, Texas;

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Johanna Escalante Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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M. Fatih Okcu Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Guillermo Aldave Division of Pediatric Neurosurgery, Department of Neurosurgery, Baylor College of Medicine, Houston, Texas;

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Avner Meoded Section of Neuroradiology, Department of Radiology, Baylor College of Medicine, Houston, Texas; and

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Stephen Kralik Section of Neuroradiology, Department of Radiology, Baylor College of Medicine, Houston, Texas; and

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Kimberly Davis Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Marina Ma Section of Physical Medicine and Rehabilitation, Department of Pediatrics, Baylor College of Medicine, Houston, Texas

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Emily A. H. Warren Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Mark D. McCurdy Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Howard L. Weiner Division of Pediatric Neurosurgery, Department of Neurosurgery, Baylor College of Medicine, Houston, Texas;

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William Whitehead Division of Pediatric Neurosurgery, Department of Neurosurgery, Baylor College of Medicine, Houston, Texas;

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Michael E. Scheurer Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Lisa Rodriguez Section of Physical Medicine and Rehabilitation, Department of Pediatrics, Baylor College of Medicine, Houston, Texas

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Amy Daigle Section of Physical Medicine and Rehabilitation, Department of Pediatrics, Baylor College of Medicine, Houston, Texas

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Murali Chintagumpala Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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Lisa S. Kahalley Section of Psychology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas;

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OBJECTIVE

Patients who experience postoperative pediatric cerebellar mutism syndrome (CMS) during treatment for medulloblastoma have long-term deficits in neurocognitive functioning; however, the consequences on functional or adaptive outcomes are unknown. The purpose of the present study was to compare adaptive, behavioral, and emotional functioning between survivors with and those without a history of CMS.

METHODS

The authors examined outcomes in 45 survivors (15 with CMS and 30 without CMS). Comprehensive neuropsychological evaluations, which included parent-report measures of adaptive, behavioral, and emotional functioning, were completed at a median of 2.90 years following craniospinal irradiation.

RESULTS

Adaptive functioning was significantly worse in the CMS group for practical and general adaptive skills compared with the group without CMS. Rates of impairment in practical, conceptual, and general adaptive skills in the CMS group exceeded expected rates in the general population. Despite having lower overall intellectual functioning, working memory, and processing speed, IQ and related cognitive processes were uncorrelated with adaptive outcomes in the CMS group. No significant group differences or increased rates of impairment were observed for behavioral and emotional outcomes.

CONCLUSIONS

Survivors with CMS, compared with those without CMS, are rated as having significant deficits in overall or general adaptive functioning, with specific weakness in practical skills several years posttreatment. Findings from this study demonstrate the high risk for ongoing functional deficits despite acute recovery from symptoms of CMS, highlighting the need for intervention to mitigate such risk.

ABBREVIATIONS

ABAS-II = Adaptive Behavior Assessment System, Second Edition; ABAS-3 = Adaptive Behavior Assessment System, Third Edition; BASC-2 = Behavior Assessment System for Children, Second Edition; CMS = cerebellar mutism syndrome; CSI = craniospinal irradiation; FSIQ = full-scale IQ; GAC = General Adaptive Composite; PSI = Processing Speed Index; VCI = Verbal Comprehension Index; WMI = Working Memory Index.
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