REhabilitation Approaches in CHildren with cerebellar mutism syndrome (REACH): An international cross-disciplinary survey study.

OBJECTIVE: Pediatric cerebellar mutism syndrome (pCMS) can occur following resection of a posterior fossa tumor and, although some symptoms are transient, many result in long-lasting neurological deficits. A multi-disciplinary rehabilitation approach is often used in cases of pCMS; however, there have been no clinical trials to determine gold standards in rehabilitation practice in this population, which remains a research priority. The purpose of this study was to identify and compare intervention practices used in pCMS throughout the disciplines of occupational and physical therapy, speech-language pathology, and neuropsychology across geographic regions. METHODS: A 55-question e-survey was created by an international multidisciplinary research group made up of members of the Posterior Fossa Society and sent to rehabilitation professionals in pediatric neuro-oncology centers in the US, Canada, and Europe. RESULTS: Although some differences in the type of intervention used in pCMS were identified within each discipline, many of the targeted interventions including dose, frequency, and intensity were similar within disciplines across geographic regions. In addition, there were common themes identified across disciplines regarding challenges in the rehabilitation of this population. CONCLUSION: These results provide a foundation of current practices on which to build future intervention-based clinical trials.

Postoperative Pediatric Cerebellar Mutism After Posterior Fossa Surgery: A Case Report.

Cerebellar mutism syndrome (CMS) is a common complication of posterior fossa surgery that can confound the postanesthetic examination and have long-lasting impacts. There is confusion surrounding its precise description, diagnostic features, and associated morbidity. Here, we discuss the most up-to-date knowledge of CMS drawing from a clinical case in the context of 3 new reports: (1) an international consensus paper presenting a new proposed working definition by the Iceland Delphi Group, (2) a knowledge update by Gadgil et al, (3) and a review of neuroimaging-based data elucidating the etiology of CMS by Patay.

Cerebellar mutism syndrome: cause and rehabilitation.

PURPOSE OF REVIEW: Mutism of cerebellar origin may occur in the context of various causes but is most frequent in children after resection of a large midline cerebellar tumour. In this review, the endeavour to reach a consensus on name and definition of postoperative mutism of cerebellar origin and associated symptoms is highlighted. In addition, progress in understanding of cause and risk factors for the syndrome is discussed as well as the rehabilitation issues. RECENT FINDINGS: Consensus on the term cerebellar mutism syndrome (CMS) has been reached. The exact pathogenesis of CMS remains unclear. Recently, attention was drawn to the hypothesis that thermal injury might be an important mechanism in the pathogenesis of CMS. Diffusion tensor imaging tractography was found to visualize the damage to relevant pathways that are associated with persistent impairments after recovery of CMS. There is still no established treatment for CMS to date. SUMMARY: By reaching a consensus on terminology and description of CMS, a firm basis has been created for future research. The pathogenesis of CMS seems multifactorial and important risk factors have been found. However, CMS cannot be effectively prevented yet and no established or specific treatment is available, apart from very general rehabilitation and cognitive interventions.

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.

Behavioral intervention to treat selective mutism across multiple social situations and community settings.

We evaluated a behavioral intervention for a 9-year-old girl with selective mutism. The intervention consisted of role play and video self-modeling. The frequency of spoken initiations, responses to questions, and communication breakdowns was measured during three social situations (i.e., ordering in a restaurant, meeting new adults, and playing with new children) and in three community settings. Results demonstrated increases in spoken initiations and responses and decreases in communication breakdowns across all situations and settings.

Alteraciones del comportamiento, fiebre y mutismo. Viejo reto para médicos actuales / Behaviour changes, fever and mutism. An old challenge for present doctors

Presentamos el caso de una mujer de 65 años con fiebre, alteraciones del comportamiento y mutismo. En el diagnóstico diferencial del síndrome febril con alteraciones neuropsiquiátricas es fundamental realizar un estudio sistemático para llegar a un conocimiento temprano de la etiología del cuadro, que nos permita realizar un correcto tratamiento. En este caso, el diagnóstico temprano de catatonia maligna permitió aplicar tratamiento específico con terapia electroconvulsiva, con una pronta y favorable evolución. En este artículo, proponemos una conducta diagnóstica que se debe seguir que creemos puede resultar de utilidad en la práctica médica diaria (AU)

We present a case of a 65-year-old woman with fever, behaviour disorder and mutism. The differential diagnosis of fever and neuropsychiatric disorders requires a systematic study to get an early understanding of the aetiology, allowing us to provide the correct treatment. In our case, the early diagnosis of malignant catatonia enabled us to give our patient electroconvulsive therapy, achieving a spectacular improvement. In this article, we suggest an algorithm that could be useful in daily medical practice (AU)

Clinical case study: a 4-year-old boy with posterior fossa syndrome after resection of a medulloblastoma.

Posterior fossa syndrome can occur after neurosurgical resections of childhood posterior fossa tumors, such as medulloblastomas. Posterior fossa syndrome is characterized by transient mutism, emotional lability, cognitive deficits, weakness, ataxia, and cranial nerve dysfunction. Symptoms generally appear 1 to 3 days after surgery and persist for months to years. Neuroscience nurses play an integral role in helping affected children and their families through the diagnosis, treatment, and sequelae of this frightening syndrome.

Laryngotracheal separation in neonatal brainstem dysfunction.

INTRODUCTION: Neonatal brainstem dysfunction (NBD) associates four symptoms of variable presence and intensity: suction-swallowing dysfunction, abnormal laryngeal sensitivity and motility, gastroesophageal reflux, and cardiac vagal overactivity. MATERIALS AND METHODS: We report three cases of severe NBD with chronic aspiration which required surgical management. Successive failures and clinical deterioration led us to perform laryngotracheal separation. The surgical procedure consisted in suturing the distal segment of the trachea to the cervical skin after complete closure of the larynx. RESULTS: After surgery, these children did not present any pulmonary infection and were allowed oral nutrition. However, oral communication was no longer possible. Although it is a theoretically reversible procedure, the decision is ethically difficult in children free of mental deficiency, because of the vocal loss and the unpredictable NBD outcome. CONCLUSION: Laryngotracheal separation may be recommended after multidisciplinary decision for severe chronic aspiration in the particular case of children presenting with NBD.

Postoperative cerebellar mutism in adult patients with Lhermitte-Duclos disease.

Cerebellar mutism (CM) is a rare and severe form of speech and language impairment, mostly diagnosed in children and adolescents and rarely reported in adults. We here review the literature and summarize all anatomical structures related to the pathogenesis of this rare syndrome. We also report two illustrative cases of CM following surgical treatment of Lhermitte-Duclos disease (LDD; dysplastic gangliocytoma) in two adult patients. LDD is a rare benign cerebellar tumor. Surgical excision appears to be the only effective treatment. However, surgery is hampered by the difficulty to distinguish between tumor and healthy cerebellar tissue, which may result in extensive resection and cause neurological deficits such as CM. A review of the literature and our two cases suggest that lesions or functional impairment of paravermian structures including dentate nuclei, vermis, lateral hemispheres, and cerebellocortical pathways contribute to the development of CM. However, there is no single anatomical structure identified to be associated with CM. It is unknown whether some diseases such as LDD carry a higher risk of postoperative CM than others. As illustrated by our two cases, although there are no special means, optimal preoperative diagnosis might contribute to the prevention of this syndrome. Despite the severity, CM carries a favorable prognosis and generally resolves within a few months.To conclude, we review the clinical signs and particularly the pathophysiological observations and anatomical structures affected in the development of postoperative CM and contribute two cases illustrating the pathogenesis, prognosis, and possible prevention of this syndrome, to focus that CM might also occur in adults even in association with rare tumors.

Discharge management of an adolescent female with posterior fossa syndrome: a case report.

Posterior Fossa Syndrome (PFS) is a constellation of neurological, behavioural and psychological symptoms occurring in pediatric patients following surgical resection of posterior fossa brain tumours. The clinical presentation of PFS typically includes cerebellar mutism, bulbar dysfunction, ataxia, cranial nerve palsies, flaccid hemiparesis and emotional lability. The intent of this paper is to (a) provide an overview of PFS, (b) explore the case of a 16-year-old adolescent who presented with PFS following surgical resection of a fourth ventricle medulloblastoma, (c) reveal the complexity of her discharge, and (d) describe a discharge management framework used by the authors to guide the discharge process from a general pediatric unit in a tertiary care hospital.

The pathophysiologic mechanism of cerebellar mutism.

OBJECTIVE: Cerebellar mutism (CM) is a postoperative complication of mainly pediatric posterior fossa surgery. Multiple theories exist for explaining this phenomenon. We have made an attempt to further understand this entity given a particularly interesting case as it relates to multiple pathophysiologic pathways. METHODS: We have reviewed the details surrounding a particularly interesting case of CM. A retrospective analysis of this patient's clinical history and recovery is described. An extensive literature review has been performed in conjunction with an attempt to help elucidate details and a better understanding of CM. RESULTS: A thorough analysis of existing theories as to the pathophysiologic mechanism of CM has been performed as it relates to the details of this particular case. A case is described in which a child exhibiting CM abruptly improved and made a relatively quick recovery after the triggering of the melodic speech pathway by way of watching and beginning to sing along with a video. It appears that this incident involving a familiar song catalyzed various speech pathways, which apparently were in some state of shock. This phenomenon seems to be a temporary entity involving not only the mechanical coordination of speech production, but also the initiation of speech itself. CONCLUSIONS: Evidence exists for a pathophysiologic pathway for speech by way of coordinating phonation and articulation. In addition, there seems to exist a pathway by which the initiation of speech may be altered or halted by posterior fossa pathology, namely, vermian or dentate nuclear injury. In particular to this case, we found that the incidental appreciation of other forms of speech, melodic in this instance, may be the key to help stimulate and accelerate the recovery from CM.

Treating selective mutism in a paediatric rehabilitation patient by altering environmental reinforcement contingencies.

Selective mutism is a disorder which can cause severe social and academic impairment, and for which a wide variety of treatment approaches have been used, with varying degrees of success. Selective mutism can be conceptualized as the lack of generalization of a class of operant responses (e.g. audible and comprehensible verbalizations) across environmental contexts. The rehabilitation hospital setting, in which the patient is seen daily by multiple people in multiple settings, is particularly well-suited for implementing a systematic behavioural intervention to establish verbal behaviour and simultaneously reinforce its generalization. Data are presented on a 7-year-old female admitted to a rehabilitation hospital following orthopaedic surgery, who met the DSM-IV diagnostic criterion for selective mutism. Additional medical diagnoses included cerebral palsy, microcephaly, and mild mental retardation. A behavioural programme was developed and implemented to reinforce differentially first any communication, then verbal communication across staff and settings. Results were evaluated using a modified multiple baseline across settings design, and demonstrate that verbal, written, and tangible reinforcement effectively increased verbal behaviour where it previously rarely occurred. Results are discussed in terms of the relationship between selective mutism, social phobia and related disorders. The theoretical roles of behavioural phenomena (discriminative stimuli, stimulus generalization) in the development and treatment of these disorders are discussed.

[Establishing contact in the early stage of severe craniocerebral trauma: sound as the bridge to mute patients]. / Kontaktaufnahme im Frühstadium schwerer Schädel-Hirn-Traumen: Klang als Brücke zum verstummten Menschen.

Both from a theoretical perspective and by means of several case examples, the article focuses on the issue of overcoming the disturbed pre-verbal communication behaviour presented by patients in the early stage following severe craniocerebral trauma. In patients with brain lesion, a pre-verbal, emotionally-focussed tonal language almost invariably is capable of reaching the still healthy sections of the person. Hence, it is possible for music therapy to both establish contact with the seemingly non-responsive patient and re-stimulate the person's fundamental communication competencies and experience at the emotional, social and cognitive levels.

Effects of a pragmatic teaching strategy for requesting information by communication board users.

The effects of a pragmatic teaching strategy on 4 nonspeaking children's abilities to initiate requests for information (i.e., who, what, and where) using manual communication boards were investigated. A teaching strategy based on milieu teaching and time-delay procedures was developed and embedded in naturally structured communication tasks to evoke information requests. Results indicated that information requests using the communication board occurred only after the pragmatic teaching strategy was applied sequentially to the three request types during the experimental condition. This effect was demonstrated repeatedly across the 4 subjects. Although generalization across request types was not demonstrated, substantial generalization was found across partners and environments. A social validation measure confirmed the treatment effectiveness. Independent raters perceived significant differences when viewing before- and after-training videotapes on 7 of 10 communication variables for all subjects.