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Child's Nervous System : ChNS :... Oct 2015Cerebellar mutism (CM) is defined as a peculiar form of mutism that may complicate the surgical excision of posterior cranial fossa tumor. The incidence is variable in... (Review)
Review
INTRODUCTION
Cerebellar mutism (CM) is defined as a peculiar form of mutism that may complicate the surgical excision of posterior cranial fossa tumor. The incidence is variable in the literature, occurring in up to one third of cases in some series. Commonly occurring peculiar features of CM are delayed onset following surgery, limited duration, and spontaneous recovery usually associated with dysarthria.
METHODS
A review has been performed concerning anatomical substrates and circuits actually considered to be involved in the development of cerebellar mutism, as well as risk factors for its development that have been documented in the literature. Attention has also been given to the long-term prognosis and the possibilities of rehabilitation that can be considered in these children, which has been compared with the authors' institutional experience.
RESULTS AND CONCLUSIONS
Tumor infiltration of the brainstem seems to represent the most relevant feature related to the development of CM, along with the histological diagnosis of medulloblastoma. On the other hand, hydrocephalus does not represent an independent risk factor. The higher rate of CM in children seems to be related to the higher incidence in children of tumors with malignant histology and brain stem involvement. Surgical technique does not seem to have a definite role; in particular, the use of a telovelar approach as compared to vermian split to reach the fourth ventricle extension of the tumor has not been demonstrated to prevent the development of cerebellar mutism. Concerning long-term prognosis, around one third of the children who develop cerebellar mutism after surgery have a persistent dysarthria, the remaining ones showing a residual phonological impairment. Long-term dysarthric features tend to be more severe and less prone to recovery in children presenting at diagnosis with associated combined procedural memory and defective neurocognitive functions.
Topics: Cerebellum; Humans; Infratentorial Neoplasms; Mutism; Neurosurgical Procedures; Postoperative Complications
PubMed: 26351234
DOI: 10.1007/s00381-015-2803-6 -
Handbook of Clinical Neurology 2018Cerebellar mutism most commonly, but not exclusively, develops in children after surgery for midline cerebellar or intraventricular tumors in the posterior fossa,... (Review)
Review
Cerebellar mutism most commonly, but not exclusively, develops in children after surgery for midline cerebellar or intraventricular tumors in the posterior fossa, typically medulloblastoma. Cerebellar mutism syndrome (CMS) comprises a complex set of neurologic and neurocognitive signs and symptoms, the cardinal and central component of which is an initially profound but usually reversible speech disorder. As such, CMS is currently recognized as an extreme form of the so-called cerebellar cognitive affective syndrome (Schmahmann syndrome). The putative cause of CMS is a substantial surgical injury to the proximal components of the bilateral efferent cerebellar pathways, disrupting cerebellar input to the supratentorial brain. The resultant cerebellocerebral diaschisis may lead to supratentorial cortical perfusion depression with frontal predominance. The speech disorder is, therefore, likely an apraxia. As our understanding of the mechanism and the clinical spectrum of CMS evolves, clinically useful preoperative risk stratification schemes, adjustments to surgical strategies and techniques, and possible early therapeutic-rehabilitative measures are being sought and developed to reduce the burden of this severe and particularly handicapping chronic morbidity on affected individuals and their families.
Topics: Cerebellar Diseases; Cerebellum; Child; Humans; Medulloblastoma; Mutism; Neurosurgical Procedures; Postoperative Complications
PubMed: 29891065
DOI: 10.1016/B978-0-444-64189-2.00018-4 -
Brain : a Journal of Neurology Nov 2023Cerebellar mutism syndrome is a disorder of speech, movement and affect that can occur after tumour removal from the posterior fossa. Projections from the fastigial...
Cerebellar mutism syndrome is a disorder of speech, movement and affect that can occur after tumour removal from the posterior fossa. Projections from the fastigial nuclei to the periaqueductal grey area were recently implicated in its pathogenesis, but the functional consequences of damaging these projections remain poorly understood. Here, we examine functional MRI data from patients treated for medulloblastoma to identify functional changes in key brain areas that comprise the motor system for speech, which occur along the timeline of acute speech impairment in cerebellar mutism syndrome. One hundred and twenty-four participants, all with medulloblastoma, contributed to the study: 45 with cerebellar mutism syndrome, 11 patients with severe postoperative deficits other than mutism, and 68 without either (asymptomatic). We first performed a data-driven parcellation to spatially define functional nodes relevant to the cohort that align with brain regions critical for the motor control of speech. We then estimated functional connectivity between these nodes during the initial postoperative imaging sessions to identify functional deficits associated with the acute phase of the disorder. We further analysed how functional connectivity changed over time within a subset of participants that had suitable imaging acquired over the course of recovery. Signal dispersion was also measured in the periaqueductal grey area and red nuclei to estimate activity in midbrain regions considered key targets of the cerebellum with suspected involvement in cerebellar mutism pathogenesis. We found evidence of periaqueductal grey dysfunction in the acute phase of the disorder, with abnormal volatility and desynchronization with neocortical language nodes. Functional connectivity with periaqueductal grey was restored in imaging sessions that occurred after speech recovery and was further shown to be increased with left dorsolateral prefrontal cortex. The amygdalae were also broadly hyperconnected with neocortical nodes in the acute phase. Stable connectivity differences between groups were broadly present throughout the cerebrum, and one of the most substantial differences-between Broca's area and the supplementary motor area-was found to be inversely related to cerebellar outflow pathway damage in the mutism group. These results reveal systemic changes in the speech motor system of patients with mutism, centred on limbic areas tasked with the control of phonation. These findings provide further support for the hypothesis that periaqueductal grey dysfunction (following cerebellar surgical injury) contributes to the transient postoperative non-verbal episode commonly observed in cerebellar mutism syndrome but highlights a potential role of intact cerebellocortical projections in chronic features of the disorder.
Topics: Humans; Medulloblastoma; Speech; Mutism; Cerebellar Neoplasms; Cerebellum; Cerebellar Diseases; Mesencephalon; Postoperative Complications
PubMed: 37343136
DOI: 10.1093/brain/awad209 -
Cureus Jan 2021Pediatric akinetic mutism syndrome is a clinical disease resulting from cerebellar injury and characterized by the absence of speech or reduced speech, emotional... (Review)
Review
Pediatric akinetic mutism syndrome is a clinical disease resulting from cerebellar injury and characterized by the absence of speech or reduced speech, emotional lability, there may also be hypotonia, oropharyngeal dysfunction/dysphagia, bladder and intestinal incontinence, or other behavioral disorders and neurological signals. It is described as the most recurrent complication in children, after posterior fossa tumor surgery, mainly related to cerebellar midline injuries. An increasing number of research and prospective reviews have provided valuable information on cerebellar mutism syndrome in recent years. The purpose of this review was to elucidate the pathophysiological basis and the predictive factors for this syndrome. Most cases of mutism are due to injury cerebellar tracts and cerebellar-cerebral circuits, involving particularly distinct points of the dentate-thalamus-cortical and dentato-rubro-thalamus-cortical. Advanced neuroimaging techniques, such as tractography and perfusion studies, have contributed to demonstrating changes in these pathways in patients with pediatric cerebellar mutism.
PubMed: 33542880
DOI: 10.7759/cureus.12593 -
Frontiers in Cell and Developmental... 2022Cerebellar mutism syndrome (CMS) is a common complication following surgical resection of childhood tumors arising in the posterior fossa. Alteration of linguistic... (Review)
Review
Cerebellar mutism syndrome (CMS) is a common complication following surgical resection of childhood tumors arising in the posterior fossa. Alteration of linguistic production, up to muteness and emotional lability, generally reported at least 24 h after the intervention, is the hallmark of post-operative CMS. Other associated traits include hypotonia and other cerebellar motor signs, cerebellar cognitive-affective syndrome, motor deficits from the involvement of the long pathways, and cranial neuropathies. Recovery usually takes 6 months, but most children are burdened with long-term residual deficits. The pathogenic mechanism is likely due to the damage occurring to the proximal efferent cerebellar pathway, including the dentate nucleus, the superior cerebellar peduncle, and its decussation in the mesencephalic tegmentum. Proven risk factors include brain stem invasion, diagnosis of medulloblastoma, midline localization, tumor size, invasion of the fourth ventricle, invasion of the superior cerebellar peduncle, left-handedness, and incision of the vermis. Currently, rehabilitation is the cornerstone of the treatment of patients with cerebellar mutism syndrome, and it must consider the three main impaired domains, namely speech, cognition/behavior, and movement.
PubMed: 36531947
DOI: 10.3389/fcell.2022.1082947 -
Children (Basel, Switzerland) Dec 2023Hearing loss is the most common sensory deficit and one of the most common congenital abnormalities. The estimated prevalence of moderate and severe hearing loss in a... (Review)
Review
Hearing loss is the most common sensory deficit and one of the most common congenital abnormalities. The estimated prevalence of moderate and severe hearing loss in a normal newborn is 0.1-0.3%, while the prevalence is 2-4% in newborns admitted to the newborn intensive care unit. Therefore, early detection and prompt treatment are of utmost importance in preventing the unwanted sequel of hearing loss on normal language development. The problem of congenital deafness is today addressed on the one hand with hearing screening at birth, on the other with the early (at around 3 months of age) application of hearing aids or, in case of lack of benefit, by the cochlear implant. Molecular genetics, antibody tests for some viruses, and diagnostic imaging have largely contributed to an effective etiological classification. A correct diagnosis and timely fitting of hearing aids or cochlear implants is useful for deaf children. The association between congenital deafness and "mutism", with all the consequences on/the consideration that deaf mutes have had since ancient times, not only from a social point of view but also from a legislative point of view, continued until the end of the nineteenth century, with the development on one side of new methods for the rehabilitation of language and on the other of sign language. But we need to get to the last decades of the last century to have, on the one hand, the diffusion of "universal newborn hearing screening", the discovery of the genetic causes of over half of congenital deafness, and on the other hand the cochlear implants that have allowed thousands of children born deaf the development of normal speech. Below, we will analyze the evolution of the problem between deafness and deaf-mutism over the centuries, with particular attention to the nineteenth century.
PubMed: 38255364
DOI: 10.3390/children11010051 -
Child's Nervous System : ChNS :... Jun 2020Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing... (Review)
Review
INTRODUCTION
Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase.
AIM OF THIS NARRATIVE REVIEW
The impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular.
Topics: Cerebellar Diseases; Cerebellar Neoplasms; Cerebellum; Child; Humans; Mutism; Postoperative Complications; Quality of Life
PubMed: 31222445
DOI: 10.1007/s00381-019-04229-6 -
Current Opinion in Neurology Apr 2017Mutism 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)
Review
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.
Topics: Cerebellar Diseases; Cerebellar Neoplasms; Diffusion Tensor Imaging; Humans; Mutism; Risk Factors
PubMed: 28118304
DOI: 10.1097/WCO.0000000000000426 -
Clinical Schizophrenia & Related... Jun 2018Although recognized as a feature of schizophrenia since the time of Kraepelin, motor disorders have received relatively little attention, particularly as regard their...
Although recognized as a feature of schizophrenia since the time of Kraepelin, motor disorders have received relatively little attention, particularly as regard their phenomenology. This is particularly the case in the English-speaking literature, where 'automaticity', mutism, and autonomic instability, have been emphasized among the features of catatonia at the expense of more complex behaviors and mannerisms. The possible relationship between the content of thinking disorders, such as hallucinations and dereistic thinking, and involuntary and semi-voluntary movement disorders, has been little noted. That such gestures might have "intentionality", or meaning, has not been considered. The case is presented of an adolescent who developed involuntary movements which seemed to have meaning in reference to his interests. The thought content, phenomenology, and brain abnormalities underpinning catatonia merit further study.
PubMed: 29944421
DOI: 10.3371/CSRP.CL.061518 -
Behavioural Brain Research Feb 2024Over the preceding years, music therapy has gained tremendous attention due to new findings of music in management of various conditions like Alzheimer's, depression,... (Review)
Review
Over the preceding years, music therapy has gained tremendous attention due to new findings of music in management of various conditions like Alzheimer's, depression, anxiety, insomnia, etc. Music is a non-invasive, patient-friendly and pleasant form of therapy with minimal or no side effects. It activates the reward pathway of brain by influencing several processes such as dopamine release, reduction in cortisol levels, increase in estrogen and testosterone levels. This review article focuses on advantages and disadvantages of music therapy, mechanism of action of music in brain and its effective applications in the management of different diseases. The article covers history of music therapy in America, Egypt, and India with practice of music therapy. The advanced effects of music therapy in autism, cancer, post-operative pain, Parkinson's disease, selective mutism, stroke, heart problems, pregnancy, eating disorders, bone fractures and obsessive compulsive disorders are discussed. Also the effect of music therapy on the quality of sleep and brain waves has been discussed. This is an established profession in western countries like America, UK, Australia, and Canada, but not in low-income countries like India where it needs to be standardized.
Topics: Pregnancy; Female; Humans; Music Therapy; Anxiety; Emotions; Music; Obsessive-Compulsive Disorder
PubMed: 37944563
DOI: 10.1016/j.bbr.2023.114750