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1.
Pediatr Neurol ; 154: 15-19, 2024 May.
Article in English | MEDLINE | ID: mdl-38447508

ABSTRACT

BACKGROUND: Postoperative pediatric cerebellar mutism syndrome (CMS) may occur following a process affecting the posterior cranial fossa. Recent evidence demonstrates disabling and potentially lasting motor components of this syndrome, including ataxia, hemiparesis, and oculomotor dysfunction. These impairments may contribute to vestibular deficits. METHODS: This case series contributes data to quantify vestibular dysfunction in postoperative CMS. The pair consisted of one female and one male. RESULTS: Vestibular testing demonstrated both peripheral and central dysfunction. CONCLUSIONS: Given these findings, a thorough vestibular assessment may be indicated as part of a comprehensive evaluation following a postoperative CMS diagnosis. Further research is needed to understand the pathophysiology, treatment, and long-term outcomes of postoperative pediatric CMS.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Mutism , Child , Humans , Male , Female , Mutism/diagnosis , Mutism/etiology , Cerebellar Neoplasms/surgery , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Cerebellar Diseases/diagnosis , Cerebellar Diseases/etiology , Cranial Fossa, Posterior , Syndrome
2.
Cerebellum ; 23(2): 523-544, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37184608

ABSTRACT

Following cerebellar tumour surgery, children may suffer impairments of spontaneous language. Yet, the language processing deficits underlying these impairments are poorly understood. This study is the first to try to identify these deficits for four levels of language processing in cerebellar tumour survivors. The spontaneous language of twelve patients who underwent cerebellar tumour surgery (age range 3-24 years) was compared against his or her controls using individual case statistics. A distinction was made between patients who experienced postoperative cerebellar mutism syndrome (pCMS) and those who did not. Time since surgery ranged between 11 months and 12;3 years. In order to identify the impaired language processing levels at each processing level (i.e., lexical, semantic, phonological and/or morphosyntactic) nouns and verbs produced in the spontaneous language samples were rated for psycholinguistic variables (e.g., concreteness). Standard spontaneous language measures (e.g., type-token ratio) were calculated as well. First, inter-individual heterogeneity was observed in the spontaneous language outcomes in both groups. Nine out of twelve patients showed language processing deficits three of whom were diagnosed with pCMS. Results implied impairments across all levels of language processing. In the pCMS-group, the impairments observed were predominantly morphosyntactic and semantic, but the variability in nature of the spontaneous language impairments was larger in the non-pCMS-group. Patients treated with cerebellar tumour surgery may show long-term spontaneous language impairments irrespective of a previous pCMS diagnosis. Individualised and comprehensive postoperative language assessments seem necessary, given the inter-individual heterogeneity in the language outcomes.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Language Development Disorders , Mutism , Humans , Child , Male , Female , Child, Preschool , Adolescent , Young Adult , Adult , Cerebellar Neoplasms/complications , Cerebellar Neoplasms/surgery , Cerebellar Neoplasms/diagnosis , Postoperative Complications/diagnosis , Cerebellum/surgery , Cerebellum/pathology , Cerebellar Diseases/pathology , Mutism/diagnosis , Psycholinguistics , Language Development Disorders/etiology , Language Development Disorders/pathology
3.
Transcult Psychiatry ; 61(1): 15-29, 2024 02.
Article in English | MEDLINE | ID: mdl-37814531

ABSTRACT

Although the diagnosis of selective mutism (SM) is more prevalent among immigrant children, the link between the disorder and an immigration background has been elusive. Guided by ecocultural models of development, the current study aimed to construct a theory-based description of SM while considering individual, family, and contextual risk factors. Participants were 78 children with SM (38.4% with an immigration background), and 247 typically developed children (18.2% with an immigration background). Consistent with previous studies, our results suggest that anxiety was the most important predictor of SM symptoms, above and beyond immigration background. Immigration, especially if coupled with bilingual status and low family income, predicted increased levels of SM symptoms. Identifying multi-level predictors of SM may help researchers and clinicians to improve early identification and treatment of SM in culturally and linguistically diverse children.


Subject(s)
Emigrants and Immigrants , Mutism , Child , Humans , Mutism/diagnosis , Mutism/therapy , Anxiety Disorders/diagnosis , Anxiety/therapy , Emigration and Immigration
4.
J Neurosurg Pediatr ; 33(2): 174-178, 2024 Feb 01.
Article in English | MEDLINE | ID: mdl-38000061

ABSTRACT

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.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Medulloblastoma , Mutism , Child , Male , Female , Humans , Child, Preschool , Adolescent , Medulloblastoma/diagnostic imaging , Medulloblastoma/surgery , Medulloblastoma/epidemiology , Retrospective Studies , Mutism/etiology , Mutism/diagnosis , Mutism/epidemiology , Cerebellar Diseases/diagnosis , Cerebellar Diseases/etiology , Cerebellar Neoplasms/diagnostic imaging , Cerebellar Neoplasms/surgery , Postoperative Complications/diagnostic imaging , Postoperative Complications/etiology , Postoperative Complications/epidemiology
5.
Adv Tech Stand Neurosurg ; 46: 65-94, 2023.
Article in English | MEDLINE | ID: mdl-37318570

ABSTRACT

Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or severely reduced speech; however, many patients continue to have speech and language deficits. At this point, anti-cancer treatment with chemotherapy and radiotherapy may be of focus more than the prognosis of CMS; however, many patients continue to have speech and language problems for months and years to come, and they are at high risk of other neurocognitive sequelae as well.Without reliable measures to prevent or treat the syndrome, we may look towards improving the prognosis of speech and neurocognitive functioning in these patients. As speech and language impairment is the cardinal symptom and late effect of CMS, the effect of intense and early-onset speech and language therapy as a standard of care in these patients should be investigated in relation to its effect on regaining speech capacity.


Subject(s)
Brain Neoplasms , Cerebellar Diseases , Infratentorial Neoplasms , Mutism , Child , Humans , Mutism/diagnosis , Cerebellar Diseases/diagnosis , Brain Neoplasms/complications , Infratentorial Neoplasms/complications , Risk Assessment , Syndrome , Disease Progression , Postoperative Complications/diagnosis
6.
Pediatr. aten. prim ; 25(98): e49-e51, abr.- jun. 2023. tab
Article in Spanish | IBECS | ID: ibc-222206

ABSTRACT

El mutismo selectivo es un trastorno poco frecuente y a menudo infradiagnosticado, que puede afectar al rendimiento escolar y repercutir negativamente en su desarrollo. Ocurre antes de los 5 años y está caracterizado por la incapacidad de comunicarse en ámbitos donde el niño no se siente cómodo, como puede ser la escuela; en cambio, en otros lugares, como el domicilio, se comunica y actúa sin problemas. Genera inquietud familiar. Su tratamiento principal es la terapia cognitivo conductual. La combinación de esta terapia con la administración de fármacos se reserva para casos más graves. Se describe a continuación un caso diagnosticado hace 6 meses y en tratamiento desde entonces (AU)


Selective mutism is a less frequent disorder, often underdiagnosed, which negatively affects the child’s development and school performance. This disorder takes place before the age of five. It is characterized with the incapacity to speak in places where the child does not feel comfortable, as school, but at home they speak without problem. In fact, the detection occurs at school, and generates family distress due to their surprise for their child’s attitude. The treatment is based on cognitive-conductive therapy, and just in severe cases pharmacotherapy is needed. A case diagnosed 6 months ago and under treatment since then is described below. (AU)


Subject(s)
Humans , Female , Child, Preschool , Cognitive Behavioral Therapy , Mutism/therapy , Shyness , Diagnostic and Statistical Manual of Mental Disorders , Mutism/diagnosis , Anxiety/complications , Mutism/etiology
7.
Transcult Psychiatry ; 60(2): 313-331, 2023 04.
Article in English | MEDLINE | ID: mdl-36628442

ABSTRACT

Selective mutism is a childhood psychiatric disorder that has been associated with adverse psychological, social and educational outcomes. Although evidence suggests that culturally and linguistically diverse children might be overrepresented among children with selective mutism, a direct examination of how migration or minority status are associated with the development and persistence of the disorder is still scarce. Guided by eco-cultural perspectives of development, the current review aims to provide an overview of selective mutism in culturally and linguistically diverse children. A systematic literature review of selective mutism studies that included a group of culturally and linguistically diverse children yielded eight studies that met our inclusion criteria. Although these studies support the view that bilingualism and minority status might be associated with selective mutism, the role of sociocultural factors in the development and persistence of the disorder remained mostly unexamined. The review concludes with a discussion of potential directions for future research, including examination of the cultural and psychological meanings of silence and talk, socialization goals, gender inequality, and parental acculturation strategies.


Subject(s)
Multilingualism , Mutism , Humans , Child , Mutism/diagnosis , Mutism/psychology , Communication Barriers , Acculturation , Socialization
8.
J Autism Dev Disord ; 53(10): 3891-3900, 2023 Oct.
Article in English | MEDLINE | ID: mdl-35904647

ABSTRACT

The present study addressed the different contributors to social anxiety in children with Selective Mutism (SM), with and without co-occurring Autism Spectrum Disorder (ASD) (SM + ASD). Seventy-five parents completed an online composite questionnaire consisting of the symptoms of SM and ASD, anxiety and sensory measures. The results found the SM + ASD group showed significantly higher levels of social anxiety and sensory avoidance compared to the SM only group. However, a simple mediation model revealed sensory avoidance to be a mediator of this relationship between the diagnosis and social anxiety.. Therefore, higher levels of sensory avoidance may help to differentiate social anxiety between the groups and may also be a sign of ASD in children with SM who have and/or are yet to receive an ASD diagnosis.


Subject(s)
Autism Spectrum Disorder , Child Behavior Disorders , Mutism , Humans , Child , Autism Spectrum Disorder/diagnosis , Mutism/diagnosis , Anxiety Disorders/diagnosis , Anxiety
9.
Nord J Psychiatry ; 77(2): 158-164, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36384394

ABSTRACT

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.


Subject(s)
Child Behavior Disorders , Mutism , Humans , Child , Male , Female , Mutism/diagnosis , Mutism/epidemiology , Mutism/therapy , Child Behavior Disorders/psychology , Comorbidity , Time Factors , Turkey/epidemiology
10.
Eur Child Adolesc Psychiatry ; 32(10): 1821-1839, 2023 Oct.
Article in English | MEDLINE | ID: mdl-34853909

ABSTRACT

Selective mutism (SM) is an anxiety disorder (prevalence 1-2%), characterized by the consistent absence of speaking in specific situations (e.g., in school), while adequately speaking in other situations (e.g., at home). SM can have a debilitating impact on the psychosocial and academic functioning in childhood. The use of psychometrically sound and cross-culturally valid instruments is urgently needed.The aim of this paper is to identify and review the available assessment instruments for screening or diagnosing the core SM symptomatology. We conducted a systematic search in 6 databases. We identified 1469 studies from the last decade and investigated the measures having been used in a diagnostic assessment of SM. Studies were included if original data on the assessment or treatment of SM were reported. It was found that 38% of published studies on SM reporting original data did not report the use of any standardized or objective measure to investigate the core symptomatology. The results showed that many different questionnaires, interviews and observational instruments were used, many of these only once. The Selective Mutism Questionnaire (SMQ), Anxiety Disorders Interview Schedule (ADIS) and School Speech Questionnaire (SSQ) were used most often. Psychometric data on these instruments are emerging. Beyond these commonly used instruments, more recent developed instruments, such as the Frankfurt Scale of SM (FSSM) and the Teacher Telephone Interview for SM (TTI-SM), are described, as well as several interesting observational measures. The strengths and weaknesses of the instruments are discussed and recommendations are made for their use in clinical practice and research.


Subject(s)
Child Behavior Disorders , Mutism , Child , Humans , Mutism/diagnosis , Mutism/therapy , Mutism/psychology , Anxiety Disorders/diagnosis , Surveys and Questionnaires , Schools
11.
Child Care Health Dev ; 49(1): 106-118, 2023 01.
Article in English | MEDLINE | ID: mdl-35708465

ABSTRACT

BACKGROUND: This study investigates the severeness of participation barriers in patients surviving paediatric posterior fossa tumours (PFT) many years after treatment. In the past, aetiological studies on adverse outcome have primarily focused on pathobiological risk factors. The current analysis aims to investigate the importance of environmental variables. METHODS: On average, 10 years after diagnosis, educational and social difficulties of 42 patients surviving paediatric PFT (mean age 17 years) were inquired using a self-constructed questionnaire following the Psychosocial Resource-Orientated Assessment (PREDI). Educational or social participation barriers were defined by self- and/or proxy-reported difficulties in school or in peer relationships. Accordingly, the children and adolescents were categorized into groups of adequate and limited participation. Subsequently, the study identified potential pathobiological (treatment type, hydrocephalus, tumour relapse, gender, age at diagnosis, seizures and cerebellar mutism) and environmental (parental and maternal education, siblings, main language, discrepancies between personal and environmental values, regular physical activity and private living space) risk factors and investigated whether patients with adequate and limited participation differed in the number of risk factors. RESULTS: Almost one decade after treatment, two thirds of patients experienced educational and/or social difficulties. Patients with limited participation were more frequently associated with environmental factors such as low maternal education degree, siblings, main language other than German, discrepancies between societal and personal values and irregular physical activity, as well as the pathobiological risk factors treatment type, hydrocephalus, tumour relapse, cerebellar mutism and seizures. These variables significantly discriminated between patients with and without limited participation. CONCLUSIONS: Limited participation in patients treated for paediatric PFT is common. Next to pathobiological, also environmental risk factors play a major role in educational and social participation barriers. This highlights the fact that solely considering pathobiology is not sufficient when investigating risk factors for the emergence of late sequelae. Future aetiological studies must adopt a biopsychosocial perspective.


Subject(s)
Hydrocephalus , Infratentorial Neoplasms , Mutism , Adolescent , Child , Humans , Mutism/complications , Mutism/diagnosis , Neoplasm Recurrence, Local/complications , Infratentorial Neoplasms/complications , Hydrocephalus/complications , Seizures/complications
12.
Eur Child Adolesc Psychiatry ; 32(11): 2209-2221, 2023 Nov.
Article in English | MEDLINE | ID: mdl-35984502

ABSTRACT

Some long-term outcomes for participants with selective mutism (SM) are elevated rates of phobic disorders, particularly social phobia, persistent communicative problems, and reduced self-esteem. However, data on the long-term outcomes of SM are scarce. In this study, by analyzing interpersonal anxiety, communication skills, and self-esteem among those who experienced SM and felt cured (SM-C-group: 30 females, 6 males, mean age 28.0, SD = 7.42, range 19-47 years), those who experienced SM and did not feel cured (SM-NC-group: 37 females, 4 males, mean age 27.4, SD = 7.24, age range 19-50 years), and those who had not experienced SM (Non-SM-group: 30 females, 30 males, mean age 26.4, SD = 7.62, age range: 20-48 years), we examined the long-term outcomes of SM and the factors that influence the feeling of being cured of SM. Results showed that the SM-C-group and SM-NC-groups had significantly higher interpersonal anxiety and significantly lower communication skills than the Non-SM-group. Moreover, the SM-C-group showed significantly lower interpersonal anxiety and significantly higher communication skills than the SM-NC-group. However, while there was no significant difference in self-esteem between the SM-C and SM-NCgroups, there was a significant difference between the SM-NC and Non-SM groups. The SM-C and SM-NC groups did not differ on the retrospective symptom load (SMQ-J), but did on the Current level of difficulty with speaking. The results of the logistic regression analysis predicted that communication skills and self-esteem did not influence the feeling of being cured of SM, but interpersonal anxiety and Current level of difficulty to speak did. Therefore, it is speculated that the intensity of this interpersonal anxiety and whether people with SM still felt difficulty in talking to others may have affected the feeling of being cured from SM.


Subject(s)
Mutism , Phobic Disorders , Male , Female , Humans , Young Adult , Adult , Middle Aged , Mutism/therapy , Mutism/diagnosis , Retrospective Studies , Anxiety Disorders , Phobic Disorders/therapy , Anxiety
13.
BMC Psychol ; 10(1): 271, 2022 Nov 16.
Article in English | MEDLINE | ID: mdl-36384568

ABSTRACT

BACKGROUND: Silence in certain situations represents the core symptom of selective mutism (SM). However, it is unclear what additional symptoms are part of this disorder. Although knowledge of symptoms is essential for diagnostics and intervention, to date, only scarce research exists on circumscribed symptoms of SM. Given the large overlap between SM and social anxiety disorder (SAD), it remains also unclear which symptoms can differentiate both disorders. METHODS: A network analysis of potential symptoms of SM was performed based on a mixed sample of N = 899 children and adolescents with and without indication of SM (n = 629 with silence in certain situations). In a preliminary analysis, we demonstrated that children with and without silence in certain situations do not differ with respect to their network structure, justifying an analysis on the entire mixed sample. Possible communities (symptom clusters) within the network and thus potential latent variables were examined, and symptoms were analyzed in terms of their centrality (the extent to which they are associated with other symptoms in the network). To investigate the differentiability of symptoms of the SM network from symptoms of SAD, we computed a network that additionally contains symptoms of SAD. RESULTS: In the resulting network on symptoms of SM, silence was, as expected, the symptom with the highest centrality. We identified two communities (symptom cluster): (1) symptoms associated with the fear response of freezing, (2) symptoms associated with speech production and avoidance. SM network symptoms and SAD symptoms largely formed two separate symptom clusters, with only selectivity of speaking behavior (more talkative at home and taciturn or mute outside the home) falling into a common cluster with SAD symptoms. CONCLUSIONS: Silence appears to have been confirmed by analysis as a core symptom of SM. Additional anxiety-related symptoms, such as avoidance behavior or motor inhibition associated with freezing, seem to co-occur with silence. The two communities of SM potentially indicate different mechanisms of silence. The symptoms of SM appear to be distinguishable from those of SAD, although there seems to be overlap in terms of difficulty speaking in situations outside the home.


Subject(s)
Child Behavior Disorders , Mutism , Phobia, Social , Child , Humans , Adolescent , Mutism/diagnosis , Mutism/therapy , Mutism/complications , Syndrome , Phobia, Social/complications , Phobia, Social/diagnosis , Fear
14.
J Psychiatr Res ; 151: 710-715, 2022 07.
Article in English | MEDLINE | ID: mdl-35690008

ABSTRACT

A deficit in emotion regulation skills could be an important factor influencing the vulnerability and maintenance of symptoms in selective mutism (SM). Unfortunately, to date only a few studies have examined emotion regulation in SM. Therefore, the present study investigated whether SM is associated with dysfunctional emotion regulation strategies. We compared a sample of 28 children and adolescents with SM (M = 12.66 years, SD = 3.98; 18 females) to 33 controls without SM (M = 12.45 years, SD = 3.18; 21 females). Both groups were investigated for the assessment of SM, social anxiety and emotion regulation using self and parent report questionnaires. We assumed that the disorder is associated with less adaptive and more maladaptive strategies, especially maladaptive cognitive strategies. Instead of significant differences in these overall values, only significant differences in individual emotion regulation strategies were found. In terms of adaptive strategies, children and adolescents with SM reported less problem-oriented behaviour and less cognitive problem-solving. Instead, they reported the maladaptive strategy of abandonment more often than the control group. In contrast to other anxiety disorders, children and adolescents with SM did neither report maladaptive cognitive strategies nor seek support more frequently than the control group. Their emotion regulation strategies are qualitatively closely related to the symptoms of SM, which makes it difficult to determine their independent significance. Trial registration: This study is registered with the ClinicalTrials.gov number NCT04233905.


Subject(s)
Child Behavior Disorders , Emotional Regulation , Mutism , Adolescent , Anxiety Disorders/psychology , Child , Child Behavior Disorders/psychology , Female , Humans , Mutism/diagnosis , Mutism/etiology , Mutism/psychology , Surveys and Questionnaires
15.
Pediatr Neurol ; 132: 4-10, 2022 07.
Article in English | MEDLINE | ID: mdl-35598587

ABSTRACT

Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, occurs in a subset of children after posterior fossa tumor resection, most commonly medulloblastoma. Patients with this syndrome exhibit often transient, although protracted, symptoms of language impairment, emotional lability, cerebellar, and brainstem dysfunction. However, many patients experience persistent neurological deficits and lasting neurocognitive impairment. Historically, research and clinical care were hindered by inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty surrounding risk factors and etiology. Proposed diagnostic criteria include two major symptoms, language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement as well as other experts in this field. Risk factors most commonly associated with development of CMS include midline tumor location, diagnosis of medulloblastoma and specific tumor subtype, younger age at diagnosis, and preoperative language impairment. A proposed etiology of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Treatment for CMS remains supportive. Herein, we present a comprehensive overview of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. In addition, we identify essential multidisciplinary research priorities to advance diagnostics, prevention, and intervention efforts for patients with, or at risk for, development of CMS.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Language Development Disorders , Medulloblastoma , Mutism , Cerebellar Diseases/complications , Cerebellar Diseases/diagnosis , Cerebellar Neoplasms/complications , Child , Humans , Medulloblastoma/complications , Medulloblastoma/diagnosis , Medulloblastoma/therapy , Mutism/diagnosis , Mutism/etiology , Mutism/therapy , Postoperative Complications , Research , Syndrome
16.
Childs Nerv Syst ; 38(10): 1993-1997, 2022 10.
Article in English | MEDLINE | ID: mdl-35338372

ABSTRACT

Children undergoing surgical removal of tumors in the posterior cranial fossa can encounter a varied and complex constellation of neurological symptoms, called cerebellar mutism, defined as a disturbance in the planning and programming of motor language with preserved understanding, behavioral disorders such as inattention, visual-spatial disorganization, personality change, as well as ataxia and dysmetria. In the last years, several groups have been trying to establish risk factors or even predictive scores in order to be able at least in part to predict the appearance of speech disorders before surgery. We report on a child with pilocytic astrocytoma of the cerebellar vermis who had already been diagnosed with developmental linguistic delay two years earlier. This disorder initially worsened after surgery and later improved in the following 12 months. The aim of this paper is to emphasize the importance of preoperative neuropsychological evaluation. The present case, along with those reported in the literature, suggests that the risk of long-term cerebellar mutism is higher in children with preoperative speech disorders. In these patients a thorough assessment of cognitive and linguistic functions is therefore necessary to better evaluate the risk of cerebellar mutism after surgery.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Medulloblastoma , Mutism , Cerebellar Diseases/complications , Cerebellar Diseases/surgery , Cerebellar Neoplasms/surgery , Child , Humans , Language , Medulloblastoma/surgery , Mutism/diagnosis , Mutism/etiology , Postoperative Complications/psychology , Speech Disorders/complications , Syndrome
17.
Eur J Pediatr ; 181(3): 941-950, 2022 Mar.
Article in English | MEDLINE | ID: mdl-34651204

ABSTRACT

The post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1-17 years (median 8 years, IQR 3-10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30 days after the onset (T0) or until symptom remission. At day 30 (T1), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5-62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At T1, longer symptom persistence was associated with greater severity (p = 0.01). Greater severity at T0 predicted greater severity at T1 (p = 0.0001). Children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS (p = 0.025 and p = 0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions.


Subject(s)
Cerebellar Diseases , Cerebellar Neoplasms , Mutism , Adolescent , Cerebellar Neoplasms/surgery , Child , Child, Preschool , Humans , Infant , Mutism/diagnosis , Mutism/etiology , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Postoperative Period
18.
F1000Res ; 11: 847, 2022.
Article in Japanese | MEDLINE | ID: mdl-36851921

ABSTRACT

Background: The main characteristic of selective mutism (SM) is the failure to speak in specific social situations. Thus, assessing speech across social contexts is important for confirming a diagnosis of SM and for differentiating it from other disorders. The purpose of this review was to organize how the core symptom of SM, a lack of speech in specific social situations, has been assessed in previous studies. Methods: A systematic search of articles was conducted in three databases, Web of Science, PsycINFO, and PubMed and reviews of surveys or experimental studies that reported empirical data on individuals with SM were performed. We excluded review, qualitative, epidemiological, and intervention studies. The study summarized the diagnostic criteria, methods of confirming SM diagnosis, distinction of SM from other disorders, and methods of speech assessment. Results: A total of 447 articles were screened, where 60 articles were considered eligible. The results demonstrate that different interviews and questionnaires were used to establish the diagnosis of SM. However, the majority of interviews and questionnaires lacked validation. Only two (2/60) articles used validated methods of speech assessment to confirm SM diagnosis. Moreover, a consensus was lacking on the assessment method for differentiating SM from other disorders across studies. Specifically, 17 studies measured speech and are not intended for diagnosis. The majority of studies (16/17) used the questionnaire to assess the severity of the SM condition, and only one study conducted behavioral observation. Assessment methods based on the measurement of speech in real-life situations for individuals with SM were not established. Conclusion: We have the limitation that we did not review intervention studies. However, this systematic review revealed the problem that speech assessment methods for surveys or experimental studies of SM were not established. Future studies should establish methods of speech assessment across social situations to assess SM symptoms.


Subject(s)
Mutism , Humans , Mutism/diagnosis , Speech , Surveys and Questionnaires
19.
Int. j. clin. health psychol. (Internet) ; 21(3): 1-9, sep.-dec. 2021. tab
Article in English | IBECS | ID: ibc-211581

ABSTRACT

Background/Objective: Research on selective mutism (SM) has been limited by the absence of standardised, psychometrically sound and cross-culturally valid assessment measures. Our aim is to present the results of a study of the factor structure and the reliability and concurrent validity of the scores of the Selective Mutism Questionnaire (SMQ), translated and adapted into Spanish. The SMQ contains 17 items about children's difficulty in speaking adequately in the family, school and social environment (out-of-school and out-of-family), each of which is answered according to a Likert scale with four response alternatives on speech frequency. Method: The study involved 110 pairs of parents of children diagnosed with SM whose ages ranged from 3 to 10 years. Results: The results show that the data from the Spanish sample fit the factorial model obtained by Bergman et al., and that data on its reliability and validity are robust and confirm that it as a good instrument for assessing SM in Spanish-speaking children. Conclusions: Therefore, we can affirm that the SMQ is a good instrument to assess SM in Spanish-speaking children. (AU)


Antecedentes/Objetivo: La investigación sobre el mutismo selectivo (MS) se ha visto limitada por la ausencia de medidas de evaluación estandarizadas, psicométricamente sólidas y con validez transcultural. Nuestro objetivo es presentar los resultados de un estudio de la estructura factorial, fiabilidad y validez concurrente de las puntaciones del Selective Mutism Questionnaire (SMQ), traducido y adaptado al español. El SMQ contiene 17 ítems sobre la dificultad de los niños para hablar adecuadamente en el ámbito familiar, escolar y social (extraescolar y ajeno al ámbito familiar), cada uno de los cuales se contesta según una escala tipo Likert con cuatro alternativas de respuesta sobre la frecuencia del habla. Método: En el estudio participaron 110 parejas de padres de niños y niñas diagnosticados con MS. Resultados: Los resultados muestran que los datos de la muestra española se ajustan al modelo factorial obtenido por Bergman et al., así como que los datos sobre su fiabilidad y la validez son robustos. Conclusiones: Por todo ello podemos afirmar que el SMQ es un buen instrumento para valorar el MS en niños y niñas españoles de habla castellana. (AU)


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Psychometrics , Mutism/diagnosis , Mutism/drug therapy , Surveys and Questionnaires , Spain , Translating
20.
Acta Neurochir (Wien) ; 163(5): 1291-1298, 2021 05.
Article in English | MEDLINE | ID: mdl-32621064

ABSTRACT

BACKGROUND: After posterior fossa tumour surgery, up to 39% of children experience postoperative cerebellar mutism syndrome (CMS) characterized by mutism and other motor and cognitive impairments. There is a lack of knowledge on the patient-reported challenges and long-term needs. Consequently, no specific recommendations exist for rehabilitative and supportive interventions for patients with CMS. The aims of this study were to explore the patients' experiences related to the sequelae of CMS, to identify challenges and needs regarding support and rehabilitation in the period of growing from child to adult and to add perspectives for future developments of supportive care and rehabilitative guidelines. METHODS: Ten semi-structured interviews were conducted with young adults diagnosed with CMS as children. A thematic analysis identified four themes describing challenges impacting aspects of the participants' lives. RESULTS: Four main themes were identified and highlight the rehabilitative need for focus on verbal and non-verbal communication skills in addition to the physical impairments. We found that brain tumour survivors with CMS can benefit from social and educational rehabilitation, straightforward and truthful information, support in structuring their everyday lives and increased public knowledge of CMS. CONCLUSION: Children with CMS face a variety of challenges affecting many aspects of their everyday lives. They should be entitled to the elements of a current rehabilitation initiative for childhood cancer to support patients' social disability and educational decline. Finally, we identified a need for an official information publication.


Subject(s)
Cerebellum/pathology , Mutism/diagnosis , Adolescent , Child , Child, Preschool , Family , Female , Humans , Male , Psychological Distance , Syndrome , Time Factors , Young Adult
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