A systematic review of neuropsychological outcomes following posterior fossa tumor surgery in children

Hanzlik, Emily; Woodrome, Stacey E.; AbdelBaki, Mohamed S.; Geller, Thomas J.; Elbabaa, Samer K.

doi:10.1007/s00381-015-2867-3

Cited by 65 publications

(46 citation statements)

References 32 publications

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“…Recently, studies have shown that children with pre- and postnatal acquired, as well as hereditary cerebellar diseases, have similar neuropsychological and behavioral phenotypes. Specifically, deficits in attention, verbal memory, executive functioning, visual-spatial functioning, expressive language, internalizing and externalizing problems, and socialization have been demonstrated in these populations [Bolduc et al, 2011; Brossard-Racine et al, 2015; Hanzlik et al, 2015; Lassaletta et al, 2015]. The cognitive and behavioral deficits described in those with JS may thus, in part, reflect the underlying cerebellar dysfunction related to involvement of the hindbrain malformation in the disorder, but other structures may also be involved.…”

Section: Discussionmentioning

confidence: 99%

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Summers

Snow

Wiggs

et al. 2017

American J of Med Genetics Pt A

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Objectives Joubert Syndrome (JS) is a genetically heterogeneous ciliopathy characterized by hypo-dysplasia of the cerebellar vermis, a distinct hindbrain/midbrain malformation (molar tooth sign), and intellectual disability. We evaluated the neuropsychological profiles of 76 participants with JS in the context of molecular genetics and clinical covariates. Methods Evaluations included neuropsychological testing, structured parental interviews, DNA sequencing, brain magnetic resonance imaging (MRI), electroencephalography (EEG), ophthalmologic examination, and assessment for renal and hepatic disease. Results On average, participants manifested Full Scale Intelligence Quotients (FSIQ) in the moderately to profoundly low range (M=64.3±15.3). Of the Wechsler index scores, verbal comprehension was least affected and processing speed was most affected. Receptive language was rated as better than expressive language on the Vineland Adaptive Behavior Scales-Second Edition. Those with abnormal EEG had a significantly lower FSIQ (n=15; M=50.7±12.9) compared to participants with normal EEG (n=39; M=64.7±16.3; p=.004). Participants taking psychiatric medications manifested a lower FSIQ (n=20; M=54.8±13.2) than those not taking them (n=42; M=65.0±17.2; p=.022). These correlations were also present in the TMEM67-related JS sub-cohort (n=14). Based on parental assessment, psychiatric and behavioral problems were significantly more common than in the general population for all measures (p<.004 for all). Conclusions The majority (65%) of individuals with JS have some degree of intellectual disability. Abnormal EEG is associated with lower neuropsychological function. Processing speed is a weakness, while verbal comprehension and receptive language are relative strengths. These findings may guide parents, teachers, therapists, and doctors to determine appropriate therapies, accommodations and academic goals for individuals with JS.

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Section: Discussionmentioning

confidence: 99%

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Summers

Snow

Wiggs

et al. 2017

American J of Med Genetics Pt A

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“…Advances in the treatment of pediatric brain cancers have led to substantial improvement in survival rates . However, survivors bear the burden of treatment‐related complications that impair long‐term functioning and quality of life . Longitudinal studies have reported that childhood survivors exhibit progressive decline in intelligence quotient (IQ) scores over time as a result of acquiring new information and skills at slower rates than their same‐age peers .…”

Section: Introductionmentioning

confidence: 99%

“…2,3 However, survivors bear the burden of treatmentrelated complications that impair long-term functioning and quality of life. [4][5][6][7][8] Longitudinal studies have reported that childhood survivors exhibit progressive decline in intelligence quotient (IQ) scores over time 9,10 as a result of acquiring new information and skills at slower rates than their same-age peers. 11 Core cognitive domains ABBREVIATIONS: ACTs, Auditory Consonant Trigrams; IQ, intelligence quotient; NPS, Neurological Predictor Scale; OSDMT, Oral Symbol Digit Modality Test such as working memory, information processing speed and attention are hypothesized to be the foundational skills required to acquire and retain information.…”

Section: Introductionmentioning

confidence: 99%

The Neurological Predictor Scale: A predictive tool for long‐term core cognitive outcomes in survivors of childhood brain tumors

Taiwo

King

2016

Pediatric Blood & Cancer

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“…Most of the different types of posterior fossa tumors occur in childhood, including ependymomas, medulloblastomas, and pilocytic astrocytomas. Thirty percent of ependymomas are diagnosed in children under 3 years of age, most often located in the posterior fossa, and medulloblastomas and pilocytic astrocytomas account for 15–30% and 40% of all pediatric brain tumors, respectively2. Radiotherapy is one of the most effective treatments for brain tumors, but it is also a well-known risk factor for central nervous system injury3.…”

mentioning

confidence: 99%

Radiation induces progenitor cell death, microglia activation, and blood-brain barrier damage in the juvenile rat cerebellum

Zhou

Boström

et al. 2017

Sci Rep

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Posterior fossa tumors are the most common childhood intracranial tumors, and radiotherapy is one of the most effective treatments. However, irradiation induces long-term adverse effects that can have significant negative impacts on the patient’s quality of life. The purpose of this study was to characterize irradiation-induced cellular and molecular changes in the cerebellum. We found that irradiation-induced cell death occurred mainly in the external germinal layer (EGL) of the juvenile rat cerebellum. The number of proliferating cells in the EGL decreased, and 82.9% of them died within 24 h after irradiation. Furthermore, irradiation induced oxidative stress, microglia accumulation, and inflammation in the cerebellum. Interestingly, blood-brain barrier damage and blood flow reduction was considerably more pronounced in the cerebellum compared to other brain regions. The cerebellar volume decreased by 39% and the migration of proliferating cells to the internal granule layer decreased by 87.5% at 16 weeks after irradiation. In the light of recent studies demonstrating that the cerebellum is important not only for motor functions, but also for cognition, and since treatment of posterior fossa tumors in children typically results in debilitating cognitive deficits, this differential susceptibility of the cerebellum to irradiation should be taken into consideration for future protective strategies.

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A systematic review of neuropsychological outcomes following posterior fossa tumor surgery in children

Cited by 65 publications

References 32 publications

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

The Neurological Predictor Scale: A predictive tool for long‐term core cognitive outcomes in survivors of childhood brain tumors

Radiation induces progenitor cell death, microglia activation, and blood-brain barrier damage in the juvenile rat cerebellum

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