Perioperative Assessment of Cerebellar Masses and the Potential for Cerebellar Cognitive Affective Syndrome.

The cerebellum was long perceived to be a region of limited importance with primary functions in the regulation of motor control. A degree of its functional topography in motor modulation has been traditionally appreciated. However, an evolving body of evidence supports its role in a range of cognitive processes, including executive decision making, language, emotional processing, and working memory. To this end, numerous studies of cerebellar stroke syndromes as well as investigations with functional magnetic resonance imaging and diffusion tensor imaging have given clinicians a better model of the functional topography within the cerebellum and the essential lanes of communication with the cerebrum. With this deeper understanding, neurosurgeons should integrate these domains into the perioperative evaluation and postoperative rehabilitation of patients with cerebellar tumors. This review aims to discuss these understandings and identify valuable tools for implementation into clinical practice.

Intellectual, educational, and situation-based social outcome in adult survivors of childhood medulloblastoma.

PURPOSE: To investigate intellectual and situation-based social outcome and educational achievement in adult survivors of childhood medulloblastoma and analyse factors influencing outcome Methods: We collected demographic, medical and cognitive data, and social and educational outcome at a mean time since the end of treatments of 14.9 years in 58 adults, aged 19-35 years, consecutively treated in a single cancer center between 1989 and 2005. RESULTS: Ten survivors had severe intellectual disability, 12 were still studying, 23 had a regular employment and 13 were unemployed. Full Scale Intellectual Quotient, assessed 6.6 years after the end of treatments, ranged from 46 to 131. It was strongly associated with educational achievement and significantly lower in patients who experienced postoperative cerebellar mutism, and when parental education level was low. CONCLUSION: These factors should be systematically considered at diagnosis in order to offer adequate and timely assessments and interventions.

A School Passport as Part of a Protocol to Assist Educational Reintegration After Medulloblastoma Treatment in Childhood.

BACKGROUND: Medulloblastoma is the most common malignant brain tumour in children and is treated with a combination of surgery, radiotherapy and chemotherapy. These children frequently experience long-term cognitive, social and physical sequelae, which significantly affect school reintegration. AIM: This study aimed to explore school-return experiences to create a more structured school reintegration protocol for children postmedulloblastoma. METHODS: A cohort of nine patients who had completed treatment and for whom full neuropsychometric data were available was included in the study (median time since diagnosis 8 years). Data were collected using qualitative parental questionnaires, semistructured interviews with teachers (n = 12) and healthcare professionals (HCPs) (n = 6) involved in their school reintegration. Thematic analysis was employed. A focus group with five HCPs was then used for data validation. RESULTS: This study uncovered the following four main subjects: (1) Information sharing; (2) education and empowerment (of educational professionals (EP) and parents); (3) communication between parents, HCPs and EPs; and (4) long-term difficulties. DISCUSSION: Implementation of a standardised protocol delivered within the structure of a school passport document would aid uniform follow-up. The proposed multistage protocol includes early communication and reintegration planning followed by meetings at school re-entry. Follow-up meetings are suggested to reduce information loss and reassess the child's needs. Hospital support at school transitions, inclusion of school data in long-term clinical follow-up and long-term rehabilitation are also recommended. Each stage would be supported by school passport documentation and would facilitate school and parental empowerment, paramount to the long-term sustainability of successful schooling.

Cerebellar mutism: the rehabilitation challenge in pediatric neuro-oncology: case studies.

Cerebellar Mutism Syndrome (CMS), also known as posterior fossa syndrome (CMS/PFS), refers to a transient loss of speech followed by dysarthria and is a common complication in the pediatric population following resection of a cerebellar tumor. Recognition and rehabilitation of CMS is critical in the post-operative phases of recovery. Two case studies, including neuropsychological assessments, along with the family and patient's perspectives are reviewed.

Time perception in children treated for a cerebellar medulloblastoma.

The aim of the present study was to investigate temporal abilities in children treated by surgery for a malignant tumor in the cerebellum. Children with a diagnosed medulloblastoma and age-paired control children were given a temporal discrimination task (bisection task) and a temporal reproduction task with two duration ranges, one shorter than 1s and the other longer than 4s. The motor and cognitive capacities of these children were also assessed by a battery of age-adapted neuropsychological tests. The results did not show any significant difference in performance between the children with or without cerebellar lesions in the temporal discrimination task. It was only in the temporal reproduction task that the children with cerebellar lesions reproduced longer and more variable durations than the other children, but only for the short stimulus durations (≤ 1 s). In addition, a hierarchical regression analysis revealed that the best predictor of variance in temporal performance was a significantly lower processing speed in children with cerebellar lesions in comparison to their controls. These results indicated that the major cause of deficits in temporal judgments in children with cerebellar lesions was due to their inability to reproduce accurately short temporal intervals in association with low processing speed, rather than to a specific deficit in the perception of time.

Enhancing psychosocial outcomes for young adult childhood CNS cancer survivors: importance of addressing vocational identity and community integration.

The purpose of this study was to examine the relationship between vocational identity, community integration, positive and negative affect, and satisfaction with life in a group of young adult central nervous system (CNS) cancer survivors. Participants in this study included 45 young adult CNS cancer survivors who ranged in age from 18 to 30 years (mean=22, SD=3.62), with a mean age at diagnosis of 8.8 years. Four standardized instruments were used to assess the individual's affect, satisfaction with life, vocational identity, and self-perceived level of community integration. Pearson correlation analyses were carried out to examine the relationships between the satisfaction with life and positive and negative affect, vocational identify, and community integration. A hierarchical linear regression was then performed to determine how well these variables predict satisfaction with life among CNS cancer survivors. Significant positive correlations were found between life satisfaction and positive affect (r=0.423, P<0.01), as well as life satisfaction and community integration (r=-505, P<0.001). A positive correlation between life satisfaction and vocational identity was only marginally significant (r=0.312, P<0.05). Regression results indicate that the model explained 29% of the variance with community integration making a unique contribution. The largest contribution of this study is that the findings provide initial evidence that addressing vocational identity and community integration may be important constructs in improving young adult CNS survivors' overall levels of satisfaction with life. These constructs have not been typically addressed in psychosocial cancer treatment programs.

Cognitive consequences of the treatment of medulloblastoma among children.

Progress in the treatment of medulloblastoma has resulted in increased survival among children. However, effective treatment, especially radiation therapy, produces negative consequences in the cognitive development of children, in terms of decreased intelligence quotients. Determining the factors underlying this decrease may influence the types of rehabilitation needed by children who undergo treatment for medulloblastoma. We review recent research on the impact of some factors that may underlie the cognitive deficits of pediatric and adolescent survivors, i.e., verbal comprehension, perceptual organization, attention, and processing speed. We assess eight pediatric survivors of medulloblastoma treatment with surgery, radiation, and chemotherapy. Children were assessed twice after diagnosis, using the Wechsler Intelligence Scale for Children-Third Edition. A large decrease in cognitive capacity was evident, as measured by intelligence quotients and factor indices. A raw score analysis of 12 subtests was performed, indicating a slower acquisition of functions and knowledge in the domains of verbal comprehension, perceptual organization, social perception, and psychomotor skills. We discuss issues of social reintegration, and propose that the rehabilitation of pediatric patients must include a program for social reinsertion, in addition to psychoeducational support.

Computerized tracking to train dexterity after cerebellar tumour: a single-case experimental study.

PRIMARY OBJECTIVE: In a child with pure ataxia after tumour removal, does dexterity training at the right elbow improve dexterity at the right elbow? Does improvement in dexterity transfer to improvement in upper limb activity? RESEARCH DESIGN: Single-case experimental study with 2-week baseline, 2-week intervention and 2-week follow-up phases and assessment by a blinded assessor. METHODS AND PROCEDURES: A 5-year old child 3 years after surgical resection of a low-grade cerebellar tumour underwent to dexterity training. Dexterity was measured using a Finger-to-Nose Test and upper limb activity was measured using the 9-Hole Peg Test. Measures were collected every 2 or 3 days over the 6-week period of the study. RESULTS: Finger-to-Nose Test showed visible but not statistical improvement between baseline and intervention phases in terms of smoothness of movement. On withdrawal of the intervention, visual analysis showed that scores remained at the same level. The 9-Hole Peg Test showed visible but not statistical improvement in terms of speed and smoothness of movement. On withdrawal of the intervention, visual analysis showed that improvement in speed was maintained during follow-up, but smoothness decreased. CONCLUSIONS: There is some evidence that dexterity can be trained and that improvement carries over to activity.

Video game-based exercises for balance rehabilitation: a single-subject design.

OBJECTIVES: To investigate whether coupling foot center of pressure (COP)-controlled video games to standing balance exercises will improve dynamic balance control and to determine whether the motivational and challenging aspects of the video games would increase a subject's desire to perform the exercises and complete the rehabilitation process. DESIGN: Case study, pre- and postexercise. SETTING: University hospital outpatient clinic. PARTICIPANTS: A young adult with excised cerebellar tumor, 1 middle-aged adult with single right cerebrovascular accident, and 1 middle-aged adult with traumatic brain injury. INTERVENTION: A COP-controlled, video game-based exercise system. MAIN OUTCOME MEASURES: The following were calculated during 12 different tasks: the number of falls, range of COP excursion, and COP path length. RESULTS: Postexercise, subjects exhibited a lower fall count, decreased COP excursion limits for some tasks, increased practice volume, and increased attention span during training. CONCLUSIONS: The COP-controlled video game-based exercise regime motivated subjects to increase their practice volume and attention span during training. This in turn improved subjects' dynamic balance control.

Attention and memory functioning among pediatric patients with medulloblastoma.

OBJECTIVE: To test the hypotheses that memory and attention deficits are prevalent in survivors of childhood medulloblastoma (MB) and that these deficits are associated with problems with academic achievement. METHODS: The medical charts of 38 child survivors of MB, who were administered the California Verbal Learning Test, Child Version (CVLT-C), Conners' Continuous Performance Test (CPT), and the Wechsler Individual Achievement Test (WIAT) as part of a comprehensive neurocognitive test battery, were retrospectively reviewed. RESULTS: Although no significant verbal memory deficits were found, 8 of 11 CPT variables were significantly below the standardization mean (p < or = .01). Additionally, stepwise regression analyses found that increased omission errors were significantly associated with lower reading and math performance (p < or = .01). CONCLUSIONS: These findings confirm previous reports of attention deficits among survivors of MB and provide a better understanding of how the dysfunction of particular attentional substrates (e.g., perceptual sensitivity, response bias) may result in learning problems in this population.

Functional recovery of children and adolescents after cerebellar tumour resection.

This study examined whether lesions to the cerebellum obtained in early childhood are better compensated than lesions in middle childhood or adolescence. Since cerebellar lesions might affect motor as well a cognitive performance, posture, upper limb and working memory function were assessed in 22 patients after resection of a cerebellar tumour (age at surgery 1-17 years, minimum 3 years post-surgery). Working memory was only impaired in those patients who had received chemo- or radiation therapy. Postural sway was enhanced in 64% of the patients during dynamic posturography conditions, which relied heavily on vestibular input for equilibrium control. Upper limb function was generally less impaired, but 54% of the patients revealed prolonged deceleration times in an arm pointing task, which probably does not reflect a genuine cerebellar deficit but rather the patients' adopted strategy to avoid overshooting. Age at surgery, time since surgery or lesion volume were poor predictors of motor or cognitive recovery. Brain imaging analysis revealed that lesions of all eight patients with abnormal posture who did not receive chemo- and/or radiation therapy included the fastigial and interposed nuclei (NF and NI). In patients with normal posture, NI and NF were spared. In 11 out of 12 patients with abnormal deceleration time, the region with the highest overlap included the NI and NF and dorsomedial portions of the dentate nuclei in 10 out of 12 patients. We conclude that cerebellar damage inflicted at a young age is not necessarily better compensated. The lesion site is critical for motor recovery, and lesions affecting the deep cerebellar nuclei are not fully compensated at any developmental age in humans.

Investigation and comparison of the effects of rehabilitation on balance and coordination problems in patients with posterior fossa and cerebellopontine angle tumours.

BACKGROUND: This study was planned to investigate and compare the effects of acute rehabilitation programme on balance and coordination problems in patients with posterior fossa and cerebellopontine angle tumours. METHODS: This study was carried out at Hacettepe University, School of Physical Therapy and Rehabilitation, neurosurgical rehabilitation unit on 40 patients whose ages varied between 18-60 years. Subjects were subdivided into two groups. Group I consisted of 20 patients with posterior fossa tumour, while the group II was composed of 20 patients with cerebellopontine angle tumour. Patients were evaluated according to the localisation of the tumour, the number of the surgical operations, the level of the surgical excision of the tumour, percent of having shunt, radiotherapy and chemotherapy. Balance problems were assessed according to Mokken's functional independence assessment scale in 5 selected positions and standing balance was also evaluated according to the method of Bohannon. The rehabilitation of the patients included balance and coordination training. The patients were re-evaluated before their discharge and the results of the two evaluations were compared. RESULTS: A significant improvement was found in the second group when pre- and post-treatment evaluations were compared. There were more coordination problems in group 1 in the pretreatment period. Both groups showed significant improvement of coordination problems after the treatment. However, this improvement was more obvious in the second group (p<0.05). There was a negative significant correlation between the decrease of balance problems and the amount of the tumour excided (I Group r=-0.51, II Group r=-0.57). CONCLUSIONS: It can be concluded that rehabilitation approaches are effective in balance and coordination problems observed in both tumour groups, but the responses to treatment are more obvious in the cerebellopontine angle tumours, which have better prognosis.

Recuperación funcional cerebelosa en niños hemisferectomizados / Functional recovery after cerebellar hemispherectomy in children

Este trabajo tiene la finalidad de evaluar la recuperación funcional clínica en niños sometidos a hemisferectomía cerebelosa unilateral por neoplasia extensa en dicha estructura. Se estudiaron cinco pacientes masculinos y cinco femeninos, con edades entre los dos y 15 años, quienes presentaron síndrome cerebeloso hemisférico preoperatorio; el diagnóstico histopatológico fue meduloblastoma en siete y astrocitoma en tres. Todos fueron evaluados clínicamente antes y despúes de la cirugía, con un periodo de seguimiento hasta de 18 meses. En un tiempo promedio de 6.5 meses poscirugía, se observó que 70 por ciento presentó recuperación clínica total de la función cerebelosa. La hemisferectomía cerebelosa en niños con neoplasia extensa de esta estructura, sin invasión al tallo cerebral, no condiciona secuelas neurológicas permanentes y en algunos casos evita una recidiva tumoral temprana

Neuropsychological sequelae of the treatment of children with medulloblastoma.

When a malignant tumor invades the child's cerebellum, the cost of successful treatment is often significant cognitive morbidity. A review of neuropsychological outcome revealed that survivors of childhood medulloblastoma (MB) have long-term deficits in intelligence, memory, language, attention, academic skills, psychosocial function, and a compromised quality of life. These deficits varied with chronological age at tumor diagnosis and/or adjuvant treatment, type and duration of presenting symptoms, tumor extension beyond the cerebellum, a history of adjuvant radiation treatment, and time since treatment. The effects on neuropsychological outcome of other factors, such as post-surgical hydrocephalus, were less clear. To understand the interaction between two factors predictive of outcome, age at diagnosis and time since treatment, we analyzed IQ results for a new sample of 25 surgically-treated and radiated MB survivors, and found that age at diagnosis and time since treatment made separable contributions to intellectual morbidity. PIQ appeared to measure some general effects of diffuse cerebral insult because it varied with chronological age of the child at tumor diagnosis but was relatively constant in magnitude, once established. VIQ, in contrast, was somewhat less sensitive to age at diagnosis in treated MB survivors, but declined with time since treatment. These results are important for understanding the academic attainments and continuing rehabilitation needs of childhood MB survivors, because they suggest that these children progressively fail to assimilate new verbally-based knowledge at a developmentally-appropriate rate.

Quality of long-term survival in young children with medulloblastoma.

The reported success of treatment for children with medulloblastoma must be balanced against the effect that treatment has on the quality of life of long-term survivors. The outcome of long-term survivors reported in previous studies has been conflicting. The authors evaluate the mental and behavioral skills of a group of medulloblastoma survivors from their institution, all of whom had survived for more than 5 years postdiagnosis. A review of the institutional records yielded 32 patients. Twenty-three families were interviewed by telephone and, of these, 13 subjects came to the hospital for detailed neuropsychological and neurological evaluations. Intelligence quotient (IQ) was less than 90 for all participants tested, and patients diagnosed before the age of 3 years had lower IQ scores on average than those diagnosed later. Mean IQ and achievement test scores in reading, spelling, and mathematics were all higher in survivors who had undergone shunting. Achievement test results were often not in accord with intellectual potential, and individual intellectual skills varied widely. Perceptual-motor task performance was below average in more than 50% of the participants, but motor dexterity was more severely affected than perception. Problems in learning and a delay in both physical growth and development were seen in a majority of participants. This study directs attention to the serious difficulties faced by long-term survivors of medulloblastoma and their families, and underscores the importance of routine neuropsychological testing. Moreover, the study provides further impetus to seek alternatives to irradiation in the treatment of malignant brain tumors.

Rehabilitation outcome in children after treatment of primary brain tumor.

Functional outcome studies after treatment of primary brain tumors in children are lacking. This study of 30 children, ages 3 to 20 years (x 10.8 yrs) was aimed at documenting the effects of rehabilitation on functional outcome after treatment of primary brain tumors. Functional measurements were documented by a modified functional independence measure for children (WeeFIM). The paired t-test, Wilcoxon test, chi 2 analysis, and Friedman test were used to assess significance of data. Statistically significant improvements were documented from admission to discharge, and discharge to follow-up in total WeeFIM scores (p = 0.001 and p = 0.0001) and specifically in the subgroups of self care, mobility, and locomotion. Though no significant improvement was noted in sphincter control from admission to discharge (p = 0.15), significant gains were seen at follow-up (p = 0.006). Borderline improvements at discharge in communication (p = 0.054) and social cognition (p = 0.051) became significant at follow-up (p = 0.01 and p = 0.004). At admission, 7 patients (23%) were independent in self care, 5 (17%) in mobility and 1 (3%) in locomotion compared with 18 (60%), 20 (67%), and 15 (50%) respectively at discharge. At follow-up there was further improvement with 95% independent in self care, 100% in mobility and 70% in locomotion. This study affirms the beneficial effect of comprehensive rehabilitation on functional outcome in children with residual disabilities after treatment of their primary brain tumors.

Facial reanimation with the VII-XII anastomosis: analysis of the functional and psychologic results.

The VII-XII anastomosis has been employed for more than a decade by The Otology Group, P.C., in the facial reanimation of patients undergoing extirpation of tumors involving the cerebellopontine angle and the skull base. A retrospective review based upon a detailed questionnaire and submitted photographic documentation from 61 patients forms the basis for this review. Details and analysis of the functional results include onset of function, synkinetic activity, corneal irritation and associated ophthalmologic problems, facial tone and symmetry, and volitional mimetic function. From a psychosocial perspective, evaluation was made regarding workplace and home acceptance, self consciousness, adaptation, and overall satisfaction. Because of the nature of its technical performance and reliability, the VII-XII anastomosis is an important technique for the otolaryngologist to be familiar with. Cognizance of the functional and psychologic results with this procedure will ensure optimal (yet realistic) rehabilitation for this patient population.

[Immediate and remote results of treatment of cerebellar astrocytoma]. / Bezposrednie i odlegle wyniki leczenia gwiazdziaków mózdzku.

Cerebellar astrocytoma accounted for 10% of all brain tumours treated at the Department of Neurosurgery, Medical Academy in Cracow in the years 1946 to 1968. It accounted for 16.6% of all gliomas, and 57% of subtentorial gliomas. Table I shows the distribution of the tumour according to age groups. The male:female sex ratio was near 1.0. In 124 cases the tumour was situated in the cerebellar hemispheres and in 91 in the vermis. The present study is based on an analysis of 215 cases with 124 tumours in the hemispheres and 91 in the vermis. In the hemispheres 77.8% of astrocytomas had cavities, while 22.2% were solid. In the vermis 60.6% of the tumours had cavities and 39.4% had no cavities. Infiltration of the brain stem or adherence to the floor of the fourth ventricle are mentioned in the protocols of 19 operations. The most frequent tumour in childhood and adolescence was pilocytic astrocytoma, in adulthood fibrillary and protoplasmic astrocytomas prevailed. In 10 cases of the last mentioned variety evidence of anaplasia was found. In the first four years when all operations were performed under local analgesia or rectal general anaesthesia the operative mortality was 21.5%, and in the subgroup of 40 first cases it was even 25%. After introduction of endotracheal anaesthesia the operative mortality fell to 13%, and in the subgroup of 40 last cases it was 9%. Detailed data about follow-up observations are available in 93 cases. Thirteen of them were disabled because of complete or nearly complete loss of vision. Nine of them completed schools for the blind and work in gainful occupations ad two founded families. Three patients are completely disabled because of equilibrium disturbances and ataxia. Two children attended a special school. The remaining 85 patients regarded themselves as healthy. This group comprised 66 patients operated upon at the age from 2 to 14 years, 12 were treated at the age from 15 to 21 years and 7 above that age. Some of them had high school education, others were doing heavy manual work, and many others attended secondary or high schools. Among the female patients 13 were mothers who gave birth to 23 children. (Table I). The fates of our patients confirm the view that in most cases cerebellar astrocytoma is surgically curable. The limit of radical operation is finding of brain stem infiltration observed in about 11% of cases. Partial removal of the tumour does not protect against recurrence but in some cases symptom-free periods of many years' duration were reported.