Language outcomes in children who underwent surgery for the removal of a posterior fossa tumor: A systematic review.

BACKGROUND: Children who underwent posterior fossa tumor removal may have spoken or written language impairments. The present systematic review synthesized the literature regarding the language outcomes in this population. Benefits of this work were the identification of shortcomings in the literature and a starting point toward formulating guidelines for postoperative language assessment. METHODS: A systematic literature search was conducted, identifying studies with patients who had posterior fossa surgery before 18 years of age. Included studies were narratively synthesized to understand language outcomes by language function (e.g., phonology, morphosyntax) at a group and individual level. Furthermore, the influence of several mediators (e.g., postoperative cerebellar mutism syndrome (pCMS), tumor type) was investigated. A critical evaluation of the language assessment tools was conducted. RESULTS: The narrative synthesis of 66 studies showed that a broad spectrum of language impairments has been described, characterized by a large interindividual heterogeneity. Patients younger at diagnosis, receiving treatment for a high-grade tumor and/or radiotherapy and diagnosed with pCMS seemed more prone to impairment. Several gaps in language assessment remain, such as a baseline preoperative assessment and the assessment of pragmatics and morphosyntax. Further, there were important methodological differences in existing studies which complicated our ability to accurately guide clinical practice. CONCLUSION: Children who had posterior fossa surgery seem to be at risk for postoperative language impairment. These results stress the need for language follow-up in posterior fossa tumor survivors.

Application of the Rotterdam postoperative cerebellar mutism syndrome prediction model in patients undergoing surgery for medulloblastoma in a single institution.

OBJECTIVE: Postoperative cerebellar mutism syndrome (CMS) develops in up to 40% of children with medulloblastoma. The Rotterdam model (RM) has been reported to predict a 66% risk of CMS in patients with a score of ≥ 100. The aim of this study was to retrospectively apply the RM to an independent cohort of patients with newly diagnosed medulloblastoma and study the applicability of the RM in predicting postoperative CMS. METHODS: Participants had to have their first tumor resection at the authors' institution and be enrolled in the SJMB12 protocol (NCT01878617). All participants underwent structured serial neurological evaluations before and then periodically after completing radiation therapy. Imaging was reviewed by the study neurologist who was blinded to CMS status when reviewing the scans and retrospectively applied RM score to each participant. RESULTS: Forty participants were included (14 females and 26 males). Four (10%) patients had CMS. The median age at tumor resection was 11.7 years (range 3.5-17.8 years). Tumor location was midline in 30 (75%), right lateral in 6 (15%), and left lateral in 4 (10%). The median Evans index was 0.3 (range 0.2-0.4), and 34 (85%) patients had an Evans index ≥ 0.3. Five participants required a ventricular shunt. The median tumor volume was 51.97 cm3 (range 20.13-180.58 cm3). Gross-total resection was achieved in 35 (87.5%) patients, near-total resection in 4 (10%), and subtotal in 1. The median RM score was 90 (range 25-145). Eighteen participants had an RM score of ≥ 100, and of these 16.7% (n = 3) had CMS. Of the 22 patients with an RM score < 100, 1 child developed CMS (4.5%, CI 0.1%-22.8%); 3 of the 18 patients with an RM score ≥ 100 developed CMS (16.7%, CI 3.6%-41.4%). The observed rate of CMS in the cohort of children with an RM score ≥ 100 was significantly lower than the observed rate in the original RM cohort (66.7%, CI 51%-80.0%, p < 0.001). A greater risk of CMS in patients with an RM score ≥ 100 could not be confirmed (p = 0.31). CONCLUSIONS: At the authors' institution, the incidence of CMS in patients who had an RM ≥ 100 was significantly lower than the RM cohort. These findings raise questions regarding generalizability of RM; however, fewer cases of CMS and a relatively small cohort limit this conclusion.

Cerebellar Mutism Syndrome After Posterior Fossa Tumor Surgery in Children-A Retrospective Single-Center Study.

OBJECTIVE: Cerebellar mutism syndrome (CMS) is a well-known complication after posterior fossa tumor surgery in pediatric patients. We evaluated the incidence of CMS in our institute and analyzed its association with multiple risk factors, such as tumor entity, surgical approach, and hydrocephalus. METHODS: All pediatric patients who had undergone intra-axial tumor resection in the posterior fossa between January 2010 and March 2021 were included in the retrospective analysis. Various data points, including demographic, tumor-associated, clinical, radiological, surgery-associated, complications, and follow-up data, were collected and statistically evaluated for an association with CMS. RESULTS: A total of 63 surgeries in 60 patients were included. The median patient age was 8 years. Pilocytic astrocytoma was the most common tumor type (50%), followed by medulloblastoma (28%) and ependymomas (10%). Complete, subtotal, and partial resection was achieved in 67%, 23%, and 10%, respectively. A telovelar approach had been used the most often (43%) compared with a transvermian approach (8%). Of the 60 children, 10 (17%) had developed CMS and showed marked improvement but with residual deficits. The significant risk factors were a transvermian approach (P = 0.03), vermian splitting when added to another approach (P = 0.002), an initial presentation with acute hydrocephalus (P = 0.02), and hydrocephalus present after tumor surgery (P = 0.004). CONCLUSIONS: Our CMS rate is comparable to those described in the literature. Despite the limitations of the retrospective study design, we found that CMS was not only associated with a transvermian approach but was also associated with a telovelar approach, although to a lesser extent. Acute hydrocephalus at the initial presentation necessitating urgent management was significantly associated with a greater incidence of CMS.

Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study.

The aim of this study was to explore the association between sex and cerebellar mutism syndrome and to examine other potential risk factors. This ambispective cohort study examined 218 pediatric patients (132 boys) with a posterior fossa tumor who underwent tumor resection from July 2013 to March 2021. The patients' demographics and tumor characteristics were examined and statistically analyzed to explore the associations among the variables. Multivariable and subgroup analyses were conducted to validate the independent risk factors for cerebellar mutism syndrome (CMS). The male and female patients did not differ significantly in terms of age, tumor size, tumor location, tumor consistency, VP shunt placement before resection, extent of resection, or surgeon, as well as with respect to the presence of hydrocephalus or paraventricular edema. The overall incidence of CMS was 32.6%. The incidence of CMS was significantly higher in male patients than that in female patients (41.7% vs. 18.6%; P = 0.001). In the multivariable analysis, male sex (adjusted odds ratio [OR], 3.27; P = 0.001), solid tumor consistency (adjusted OR, 5.61; P = 0.001), midline location (adjusted OR, 3.78; P = 0.004), and hydrocephalus (adjusted OR, 2.56; P = 0.047) were independent risk factors for the CMS. Chi-square analysis revealed that solid tumor consistency and midline location were associated with medulloblastoma (P < 0.001). Male patients had a higher risk of developing CMS after a posterior fossa tumor resection. Midline location, solid tumor consistency, and hydrocephalus were independent risk factors for CMS.

Clinical characteristics according to sex and symptom severity in children with selective mutism: a four-center study.

INTRODUCTION: Clinical information regarding selective mutism (SM), a persistent and debilitating psychiatric disorder, in children is extremely limited. We aimed to examine sociodemographic characteristics and comorbid psychiatric conditions and identify clinical variables associated with sex and SM severity among children with SM. METHODS: We analyzed the medical records of 49 children who received treatment for SM in four different tertiary hospitals in Turkey between 2016 and 2021. Children's charts were reviewed to examine clinical characteristics, comorbidities, and response to treatment. RESULTS: Thirty-one children were female, and 18 were male (female:male ratio is 1.7:1). Most children (73.5%) with SM displayed onset of SM in 3-6 years. However, most children (57.1%) were diagnosed between the ages of 7-11. The mean time from onset to diagnosis was 1.69 ± 1.37 years. Females displayed a later onset of SM (6.42 ± 2.40 vs. 4.89 ± 0.96; p= 0.013) and higher comorbidity rates (71% vs. 38.9%, p= 0.039) than males. The vast majority of children received two or more psychiatric diagnoses. Children in the severe group had a longer duration of illness, higher rates of psychiatric comorbidity, speech delay, and treatment resistance. CONCLUSION: Our study suggests that SM may have different clinical features according to sex and symptom severity of SM. More information about children with SM is needed to understand the development and maintenance of SM.

Postoperative speech impairment and cranial nerve deficits after secondary surgery of posterior fossa tumours in childhood: a prospective European multicentre study.

PURPOSE: Brain tumours constitute 25% of childhood neoplasms, and half of them are in the posterior fossa. Surgery is a fundamental component of therapy, because gross total resection is associated with a higher progression-free survival. Patients with residual tumour, progression of residual tumour or disease recurrence commonly require secondary surgery. We prospectively investigated the risk of postoperative speech impairment (POSI) and cranial nerve dysfunction (CND) following primary and secondary resection for posterior cranial fossa tumours. METHODS: In the Nordic-European study of the cerebellar mutism syndrome, we prospectively included children undergoing posterior fossa tumour resection or open biopsy in one of the 26 participating European centres. Neurological status was assessed preoperatively, and surgical details were noted post-operatively. Patients were followed up 2 weeks, 2 months and 1 year postoperatively. Here, we analyse the risk of postoperative speech impairment (POSI), defined as either mutism or reduced speech, and cranial nerve dysfunction (CND) following secondary, as compared to primary, surgery. RESULTS: We analysed 426 children undergoing primary and 78 undergoing secondary surgery between 2014 and 2020. The incidence of POSI was significantly lower after secondary (12%) compared with primary (28%, p = 0.0084) surgery. In a multivariate analysis adjusting for tumour histology, the odds ratio for developing POSI after secondary surgery was 0.23, compared with primary surgery (95% confidence interval: 0.08-0.65, p = 0.006). The frequency of postoperative CND did not differ significantly after primary vs. secondary surgery (p = 0.21). CONCLUSION: Children have a lower risk of POSI after secondary than after primary surgery for posterior fossa tumours but remain at significant risk of both POSI and CND. The present findings should be taken in account when weighing risks and benefits of secondary surgery for posterior fossa tumours.

Risk factors for postoperative cerebellar mutism syndrome in pediatric patients: a systematic review and meta-analysis.

OBJECTIVE: A review article assessing all the risk factors reported in the literature for postoperative cerebellar mutism syndrome (pCMS) among children remains absent. The authors sought to perform a systematic review and meta-analysis to evaluate this issue. METHODS: PubMed, Embase, and Web of Science were queried to systematically extract potential references. The articles relating to pCMS were required to be written in the English language, involve pediatric patients (≤ 18 years of age), and provide extractable data, which included a comparison group of patients who did not develop pCMS. The quality of the included studies was evaluated using the Newcastle-Ottawa Scale. Data were pooled using RevMan 5.4, and publication bias was assessed by visual inspection for funnel plot asymmetry. The study protocol was registered through PROSPERO (ID: CRD42021256177). RESULTS: Overall, 28 studies involving 2276 patients were included. Statistically significant risk factors identified from univariate analysis were brainstem invasion (OR 4.28, 95% CI 2.23-8.23; p < 0.0001), fourth ventricle invasion (OR 12.84, 95% CI 4.29-38.44; p < 0.00001), superior cerebellar peduncle invasion (OR 6.77, 95% CI 2.35-19.48; p = 0.0004), diagnosis of medulloblastoma (OR 3.26, 95% CI 1.93-5.52; p < 0.0001), medulloblastoma > 50 mm (OR 8.85, 95% CI 1.30-60.16; p = 0.03), left-handedness (OR 6.57, 95% CI 1.25-34.44; p = 0.03), and a vermis incision (OR 5.44, 95% CI 2.09-14.16; p = 0.0005). On the other hand, a tumor located in the cerebellar hemisphere (OR 0.23, 95% CI 0.06-0.92; p = 0.04), cerebellar hemisphere compression (OR 0.23, 95% CI 0.11-0.45; p < 0.0001), and intraoperative imaging (OR 0.36, 95% CI 0.18-0.72; p = 0.004) reduced the risk of pCMS. CONCLUSIONS: This study provides the largest and most reliable review of risk factors associated with pCMS. Although some risk factors may be dependent on one another, the data may be used by surgeons to better identify patients at risk for pCMS and for intervention planning.

Postoperative speech impairment and surgical approach to posterior fossa tumours in children: a prospective European multicentre cohort study.

BACKGROUND: Brain tumours are the most common solid tumours in childhood. Half of these tumours occur in the posterior fossa, where surgical removal is complicated by the risk of cerebellar mutism syndrome, of which postoperative speech impairment (POSI) is a cardinal symptom, in up to 25% of patients. The surgical approach to midline tumours, mostly undertaken by transvermian or telovelar routes, has been proposed to influence the risk of POSI. We aimed to investigate the risk of developing POSI, the time course of its resolution, and its association with surgical approach and other clinical factors. METHODS: In this observational prospective multicentre cohort study, we included children (aged <18 years) undergoing primary surgery for a posterior fossa tumour at 26 centres in nine European countries. Within 72 h of surgery, the operating neurosurgeon reported details on the tumour location, surgical approach used, duration of surgery, use of traction, and other predetermined factors, using a standardised surgical report form. At 2 weeks, 2 months, and 1 year after surgery, a follow-up questionnaire was filled out by a paediatrician or neurosurgeon, including neurological examination and assessment of speech. Speech was classified as mutism, reduced speech, or habitual speech. POSI was defined as either mutism or severely reduced speech. Ordinal logistic regression was used to analyse the risk of POSI. FINDINGS: Between Aug 11, 2014, and Aug 24, 2020, we recruited 500 children. 426 (85%) patients underwent primary tumour surgery and had data available for further analysis. 192 (45%) patients were female, 234 (55%) patients were male, 81 (19%) patients were aged 0-2 years, 129 (30%) were aged 3-6 years, and 216 (51%) were aged 7-17 years. 0f 376 with known postoperative speech status, 112 (30%) developed POSI, 53 (14%) developed mutism (median 1 day [IQR 0-2]; range 0-10 days), and 59 (16%) developed reduced speech after surgery (0 days [0-1]; 0-4 days). Mutually adjusted analyses indicated that the independent risk factors for development of POSI were younger age (linear spline, p=0·0087), tumour location (four levels, p=0·0010), and tumour histology (five levels, p=0·0030); surgical approach (six levels) was not a significant risk factor (p=0·091). Tumour location outside the fourth ventricle and brainstem had a lower risk of POSI (with fourth ventricle as reference, odds ratio (OR) for cerebellar vermis 0·34 [95% CI 0·14-0·77] and OR for cerebellar hemispheres 0·23 [0·07-0·70]). Compared with pilocytic or pilomyxoid astrocytoma, a higher risk of POSI was seen for medulloblastoma (OR 2·85 [1·47-5·60]) and atypical teratoid rhabdoid tumour (10·30 [2·10-54·45]). We did not find an increased risk of POSI for transvermian surgical approach compared with telovelar (0·89 [0·46-1·73]). Probability of speech improvement from mutism reached 50% around 16 days after mutism onset. INTERPRETATION: Our data suggest that a midline tumour location, younger age, and high-grade tumour histology all increase the risk of speech impairment after posterior fossa tumour surgery. We found no evidence to recommend a preference for telovelar over transvermian surgical approach in the management of posterior fossa tumours in children in relation to the risk of developing POSI. FUNDING: The Danish Childhood Cancer Foundation, the Swedish Childhood Cancer Foundation, the UK Brain Tumour Charity, the Danish Cancer Society, Det Kgl Kjøbenhavnske Skydeselskab og Danske Broderskab, the Danish Capitol Regions Research Fund, Dagmar Marshall Foundation, Rigshospitalet's Research Fund, and Brainstrust.

Cerebellar Mutism Syndrome: An Overview of the Pathophysiology in Relation to the Cerebrocerebellar Anatomy, Risk Factors, Potential Treatments, and Outcomes.

Cerebellar mutism syndrome (CMS) is one the most disabling postoperative neurological complications after posterior fossa surgery in children. CMS is characterized by a transient mutism with a typical onset demonstrated within 2 days postoperatively accompanied by associated ataxia, hypotonia, and irritability. Several hypotheses for the anatomical basis of pathophysiology and risk factors have been suggested. However, a definitive theory and treatment protocols have not yet been determined. Animal histological and electrophysiological studies and more recent human imaging studies have demonstrated the existence of a compartmentalized representation of cerebellar function, the understanding of which might provide more information on the pathophysiology. Damage to the dentatothalamocortical pathway and cerebrocerebellar diaschisis have been described as the anatomical substrate to the CMS. The risk factors, which include tumor type, brainstem invasion, tumor localization, tumor size, and vermal splitting technique, have not yet been clearly elucidated. The efficacy of potential pharmacological and speech therapies has been studied in small trials. Long-term motor speech deficits and associated cognitive and behavioral disturbances have now been found to be common among CMS survivors, affecting their development and requiring rehabilitation, leading to significant financial effects on the healthcare system and distress to the family. The aim of the present review was to outline the cerebellar anatomy and function and its connections in relationship to the pathophysiology and to refine the risk factors and treatment strategies for CMS.

Selective Mutism and Its Relations to Social Anxiety Disorder and Autism Spectrum Disorder.

In current classification systems, selective mutism (SM) is included in the broad anxiety disorders category. Indeed, there is abundant evidence showing that anxiety, and social anxiety in particular, is a prominent feature of SM. In this article, we point out that autism spectrum problems in addition to anxiety problems are sometimes also implicated in SM. To build our case, we summarize evidence showing that SM, social anxiety disorder (SAD), and autism spectrum disorder (ASD) are allied clinical conditions and share communalities in the realm of social difficulties. Following this, we address the role of a prototypical class of ASD symptoms, restricted and repetitive behaviors and interests (RRBIs), which are hypothesized to play a special role in the preservation and exacerbation of social difficulties. We then substantiate our point that SM is sometimes more than an anxiety disorder by addressing its special link with ASD in more detail. Finally, we close by noting that the possible involvement of ASD in SM has a number of consequences for clinical practice with regard to its classification, assessment, and treatment of children with SM and highlight a number of directions for future research.

Cerebellar mutism syndrome: current approaches to minimize risk for CMS.

PURPOSE: Cerebellar mutism syndrome (CMS) is a serious source of morbidity following posterior fossa surgery in the pediatric population. However, methods for effectively decreasing its incidence and impact remain unclear. It is our aim to examine the impact of adjusting surgical factors, namely the use of a telovelar approach and avoidance of cavitronic ultrasonic aspirator, on the incidence of CMS in our population as well as outlining potential pre-, intra-, and postoperative factors that may contribute to its development. METHODS: Retrospective review was performed to identify patients undergoing posterior fossa surgery for resection of a medulloblastoma. Demographic, surgical, and postoperative data were collected. These data were analyzed for possible correlations to the risk of developing CMS via univariate analysis. For factors found to be significant, a multivariate analysis was performed to assess their independence. RESULTS: Seven of 65 patients (10.8%) developed CMS postoperatively. Factors found to be significantly associated with a higher risk of CMS were the degree of retraction utilized during the procedure (p = 0.0000) and incision of the vermis (p = 0.0294). Although they did not reach the threshold of statistical significance, tumor vascularity (p = 0.19), adoption of a transvermian approach (p = 0.19), and lack of intraoperative imaging (p = 0.17) exhibited strongly suggestive trends towards a correlation with CMS. DISCUSSION: In an effort to reduce the incidence and severity of CMS in our population, our institution adopted surgical practices that minimize tissue trauma and mitigate postoperative edema. This included the use of a telovelar over a transvermian approach to obviate the need for vermian incision, avoidance of the CUSA, and minimization of heavy retraction during surgery. This was successful in reducing the incidence of CMS from 39% in our medulloblastoma patients to 10.8%. The development of CMS after posterior fossa surgery appears to be a "two-hit" phenomenon requiring a combination of existing predisposition, surgical injury, and postoperative exacerbation. Therefore, it is critical to identify the factors involved at each stage and investigate treatments to target them appropriately.

Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors.

PURPOSE: A preliminary survey of pediatric neurosurgeons working at different centers around the world suggested differences in clinical practice resulting in variation in the risk of pediatric cerebellar mutism (CM) and cerebellar mutism syndrome (CMS) after posterior fossa (PF) tumor resection. The purposes of this study were (1) to determine the incidence and severity of CM and CMS after midline PF tumor resection in children treated at these centers and (2) to identify potentially modifiable factors related to surgical management (rather than tumor biology) that correlate with the incidence of CM/CMS. METHODS: Attending pediatric neurosurgeons at British Columbia's Children's Hospital (BCCH) and neurosurgeons who completed a pediatric neurosurgery fellowship at BCCH were invited to provide data from the center where they currently practiced. Children aged from birth to less than 18 years who underwent initial midline PF tumor resection within a contemporary, center-selected 2-year period were included. Data was obtained by retrospective chart and imaging review. Modifiable surgical factors that were assessed included pre-resection surgical hydrocephalus treatment, surgical positioning, ultrasonic aspirator use, intraoperative external ventricular drain (EVD) use, surgical access route to the tumor, and extent of resection. CM was defined as decreased or absent speech output postoperatively and CMS as CM plus new or worsened irritability. RESULTS: There were 263 patients from 11 centers in 6 countries (Canada, Germany, the Netherlands, India, Indonesia, and the USA). Median age at surgery was 6 years (range < 1 to 17 years). The overall incidence of postoperative CM was 23.5% (range 14.7-47.6% for centers with data on ≥ 20 patients). The overall incidence of CMS was 6.5% (range 0-10.3% for centers contributing data on ≥ 20 patients). A multivariate logistic regression on the full data set showed no significant association between pre-resection surgical hydrocephalus treatment, prone position, ultrasonic aspirator use, EVD use, telovelar approach, complete or near total resection, or treating center and either postoperative CM or CMS. CONCLUSIONS: While there was variation in surgical management of midline PF tumors among centers participating in this study, the factors in management that were examined did not predict postoperative CM or CMS.

Visual Complications of Pediatric Posterior Fossa Tumors: Analysis of Outcomes.

OBJECTIVE: Visual abnormalities are common among children with posterior fossa tumors, resulting from disruption of ocular afferent and efferent systems due to the tumor or surgery. This study describes the visual complications and outcomes associated with these tumors. METHODS: Clinical and radiographic data of patients who underwent index surgery for resection of posterior fossa tumor from 2007 to 2016 were reviewed. Descriptive statistics, univariate, and multivariate regression were performed to assess factors contributing to visual acuity and postoperative strabismus. RESULTS: There were 182 patients who underwent posterior fossa craniotomy for neoplasm were included. Ophthalmologic symptoms were the fourth most common presenting complaint; initial ophthalmologic examination was abnormal in 40% of patients. Evaluation of visual acuity demonstrated a good outcome in 88% of patients following treatment. The most common postoperative oculomotor finding was esotropia (29%) which resolved spontaneously in more than half of patients. A good outcome was obtained in all patients who underwent surgery for esotropia. Hypertropia was noted in 14% of the cohort and less than half resolved spontaneously; less than half undergoing strabismus surgery for hypertropia had a good outcome. Multivariate analysis confirmed the association between cerebellar mutism and postoperative esotropia and hypertropia. Clinically significant pathological nystagmus was seen in 8% of the cohort. CONCLUSIONS: Our results indicate a good visual outcome in the majority of pediatric patients undergoing resection of posterior fossa tumors. Ophthalmologic complications should be appropriately evaluated and addressed to allow for the best possible vision to survivors of posterior fossa tumors.

Actualización de un modelo tentativo del mutismo selectivo / Update of a tentative model of selective mutism

El mutismo selectivo es un trastorno del comportamiento infanto-juvenil de inicio temprano (generalmente entre los 3 y los 5 años) que consiste en la resistencia al habla o su inhibición en situaciones sociales concretas, caracterizadas por la presencia de extraños, personas a las que no se habla o a las que se ha dejado de hablar, lugares en los que no se habla o donde el niño piensa que puede ser escuchado por quien no quiere, etc. Su escasa prevalencia lo convierte en un trastorno de la conducta raro y la revisión de la literatura confirma que la ansiedad es la alteración prominente en quienes lo presentan, lo que ha llevado a que en la última edición del Manual diagnóstico y estadístico de los trastornos mentales (Asociación Americana de Psiquiatría, DSM-5; APA, 2013) haya sido reclasificado como un trastorno de ansiedad. Este hecho, así como los avances en su investigación y los retos que estos plantean respecto de su evaluación y tratamiento hacen necesaria una revisión y actualización de las propuestas explicativas existentes. Este es el marco en el que se inserta el trabajo que presentamos

Selective mutism is a disorder of early-onset behavior (usually between 3 and 5 years) consisting in a resistance to speech or its inhibition in specific social situations, characterized by the presence of strangers, people who are not spoken to or to whom the child has stopped talking, places where the child doesn't speak or where the child thinks he/she can be heard by unwanted people, etc. Its low prevalence makes it a rare behavior disorder and the review of the literature confirms that anxiety is the prominent alteration in those who present it, which has led to the result that in the last edition of the Diagnostic and Statistical Manual of Mental Disorders (American Psychiatric Association, DSM-5; APA, 2013) the behavior has been reclassified as an anxiety disorder. This fact, as well as the advances in its research and the challenges that it poses regarding its evaluation and treatment, make it necessary to review and update the existing explanatory proposals. This is the framework of the work presented here

Development of a pre-operative scoring system for predicting risk of post-operative paediatric cerebellar mutism syndrome.

BACKGROUND: Despite previous identification of pre-operative clinical and radiological predictors of post-operative paediatric cerebellar mutism syndrome (CMS), a unifying pre-operative risk stratification model for use during surgical consent is currently lacking. The aim of the project is to develop a simple imaging-based pre-operative risk scoring scheme to stratify patients in terms of post-operative CMS risk. METHODS: Pre-operative radiological features were recorded for a retrospectively assembled cohort of 89 posterior fossa tumour patients from two major UK treatment centers (age 2-23yrs; gender 28 M, 61 F; diagnosis: 38 pilocytic astrocytoma, 32 medulloblastoma, 12 ependymoma, 1 high grade glioma, 1 pilomyxoid astrocytoma, 1 atypical teratoid rhabdoid tumour, 1 hemangioma, 1 neurilemmoma, 2 oligodendroglioma). Twenty-six (29%) developed post-operative CMS. Based upon results from univariate analysis and C4.5 decision tree, stepwise logistic regression was used to develop the optimal model and generate risk scores. RESULTS: Univariate analysis identified five significant risk factors and C4.5 decision tree analysis identified six predictors. Variables included in the final model are MRI primary location, bilateral middle cerebellar peduncle involvement (invasion and/or compression), dentate nucleus invasion and age at imaging >12.4 years. This model has an accuracy of 88.8% (79/89). Using risk score cut-off of 203 and 238, respectively, allowed discrimination into low (38/89, predicted CMS probability <3%), intermediate (17/89, predicted CMS probability 3-52%) and high-risk (34/89, predicted CMS probability ≥52%). CONCLUSIONS: A risk stratification model for post-operative paediatric CMS could flag patients at increased or reduced risk pre-operatively which may influence strategies for surgical treatment of cerebellar tumours. Following future testing and prospective validation, this risk scoring scheme will be proposed for use during the surgical consenting process.

Unable to Speak: Selective Mutism in Youth.

It is important for psychiatric nurses to be familiar with the clinical presentation and recommended treatment for selective mutism (SM), as it is a childhood anxiety disorder that is not commonly studied. This article provides a brief overview of its diagnostic criteria, prevalence, assessment, and history. Special attention is given to misconceptions regarding the disorder and differentiation of trauma and oppositional disorders. Two vignettes illustrate varied presentations of SM, with and without comorbid social phobia. Empirically supported behavioral and psychopharmacological treatment is outlined, and considerations for nursing are provided. [Journal of Psychosocial Nursing and Mental Health Services, 56(2), 14-18.].

Cerebellar mutism syndrome in children with brain tumours of the posterior fossa.

BACKGROUND: 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. METHODS: 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. DISCUSSION: 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. TRIAL REGISTRATION: Clinicaltrials.gov : NCT02300766 , date of registration: November 21, 2014.

Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery.

OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis. Tumor type, cerebellar midline location, and brainstem involvement are risk factors for pCMS that have been identified repeatedly, but they do not explain its latent onset. Ambiguous or negative results for other factors, such as hydrocephalus, postoperative meningitis, length of vermian incision, and tumor size, have been reached. The aim of this study was to identify perioperative clinical, radiological, and laboratory factors that also increase risk for the development of pCMS. The focus was on factors that might explain the delayed onset of pCMS and thus might provide a time window for taking precautionary measures to prevent pCMS or reduce its severity. The study was focused specifically on children who had undergone surgery for medulloblastoma. METHODS In this single-center retrospective cohort study, the authors included 71 children with medulloblastoma, 28 of whom developed pCMS after primary resection. Clinical and laboratory data were collected prospectively and analyzed systematically. Variables were included for univariate and multivariate analysis. RESULTS Univariate regression analysis revealed 7 variables that had a significant influence on pCMS onset, namely, tumor size, maximum tumor diameter > 5 cm, tumor infiltration or compression of the brainstem, significantly larger decreases in hemoglobin (p = 0.010) and hematocrit (p = 0.003) in the pCMS group after surgery than in the no-pCMS group, significantly more reported incidents of severe bleeding in the tumor bed during surgery in the pCMS group, preoperative hydrocephalus, and a mean body temperature rise of 0.5°C in the first 4 days after surgery in the pCMS group. Multiple regression analysis revealed that tumor size, tumor infiltration into or compression of the brainstem, and higher mean body temperature in the first 4 postoperative days were independent and highly significant predictors for pCMS. CONCLUSIONS The authors confirmed earlier findings that tumor-associated preoperative conditions, such as a maximum tumor diameter ≥ 5 cm and infiltration into or compression of the brainstem, are associated with a higher risk for the development of pCMS. Most importantly, the authors found that a 0.5°C higher mean body temperature in the first 4 postoperative days increased the odds ratio for the development of pCMS almost 5-fold. These data suggest that an important focus for the prevention of pCMS in children who have undergone medulloblastoma surgery might be rigorous maintenance of normothermia as standard care after surgery.