Search

Background: Cerebellar mutism syndrome (CMS) is a common and debilitating complication of posterior fossa tumor surgery in children. Affected children exhibit communication and social impairments that overlap phenomenologically with subsets of deficits exhibited by children with Autism spectrum disorder (ASD). Although both CMS and ASD are thought to involve disrupted cerebro-cerebellar circuitry, they are considered independent conditions due to an incomplete understanding of their shared neural substrates., Methods: In this study, we analyzed postoperative cerebellar lesions from 90 children undergoing posterior fossa resection of medulloblastoma, 30 of whom developed CMS. Lesion locations were mapped to a standard atlas, and the networks functionally connected to each lesion were computed in normative adult and pediatric datasets. Generalizability to ASD was assessed using an independent cohort of children with ASD and matched controls (n = 427)., Results: Lesions in children who developed CMS involved the vermis and inferomedial cerebellar lobules. They engaged large-scale cerebellothalamocortical circuits with a preponderance for the prefrontal and parietal cortices in the pediatric and adult connectomes, respectively. Moreover, with increasing connectomic age, CMS-associated lesions demonstrated stronger connectivity to the midbrain/red nuclei, thalami and inferior parietal lobules and weaker connectivity to the prefrontal cortex. Importantly, the CMS-associated lesion network was independently reproduced in ASD and correlated with communication and social deficits, but not repetitive behaviors., Conclusions: Our findings indicate that CMS-associated lesions may result in an ASD-like network disturbance that occurs during sensitive windows of brain development. A common network disturbance between CMS and ASD may inform improved treatment strategies for affected children., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

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., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: Approximately 7%-50% of children with medulloblastoma (MB) develop postoperative cerebellar mutism syndrome (pCMS). pCMS has a short-term negative impact on intelligence, but effects on long-term outcomes are contradictory. The aim of this study was to assess long-term effects of pCMS in MB patients on aspects of intelligence quotient (IQ) and its perioperative risk factors., Methods: In this single-center retrospective cohort study, 31 children were included (14 pCMS). Perioperative risk factors included brainstem invasion, vermis incision, hydrocephalus, tumor size, severity of pCMS, neurological symptoms, mean body temperature (BT) on days 1-4 post surgery, and age at resection. Age-appropriate Wechsler Intelligence tests were assessed at least 2 years after tumor resection., Results: Mean interval between tumor resection and neuropsychological evaluation was 3.9 years in pCMS and 4 years and 11 months in the no-pCMS group. No significant differences in IQ scores were found between groups. The pCMS group had a clinically relevant difference of 10 points when compared to age norms on verbal IQ (VIQ). Bilateral pyramidal and swallowing problems were risk factors for lower performance. In the overall group, tumor size, younger age at surgery, and raised mean BT were negatively correlated with aspects of IQ., Conclusions: We found a clinically significant reduction of VIQ in the pCMS patient group. pCMS patients with a larger tumor size, younger age at surgery, a higher mean BT in the first days after surgery, bilateral pyramidal symptoms, and swallowing problems 10 days post surgery are more at risk for VIQ deficits at long-term., (© 2021 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Selective mutism (SM) is classified under the category of anxiety disorders in DSM-5 [1], although concrete fears that underlie the condition are not specified contrary to all other anxiety disorders. Given the lack of studies systematically investigating fears in SM, content and frequency of concrete fears as well as related cognitions have remained unclear so far. One hundred and twenty-four participants [M = 13.25 years (SD = 3.24), range 8-18 years] with SM (n = 65), social phobia (SP n = 18) or with typical development (TD n = 51) took part in an online survey. Participants with SM (n = 65) answered an open-ended question concerning fears that might cause the consistent failure to speak in select situations. Additionally, participants with SM, SP and TD completed a survey containing 34 fear-related cognitions that might occur in speech-demanding situations. Open text answers were systematically evaluated by extracting higher-order categories using a Qualitative Content Analysis. Single item scores of the survey were compared between the three groups. 59% of all spontaneously reported fears were assigned to the cluster of social fears. Other reported fears represented the categories fear of mistakes (28%), language-related fears (8%) and voice-related fears (5%). The SM- and SP group only differed regarding the cognition that one's own voice might sound funny (SM > SP). Social fears and the fear of mistakes account for the majority of fears in SM. Therefore, future interventions should consider specifically targeting these types of fears.

Purpose: To determine the value of structural magnetic resonance imaging (MRI) in predicting post-operative paediatric cerebellar mutism syndrome (pCMS) in children undergoing surgical treatment for medulloblastoma., Methods: Retrospective cohort study design. Electronic/paper case note review of all children with medulloblastoma presenting to Great Ormond Street Hospital between 2003 and 2013. The diagnosis of pCMS was established through a scoring system incorporating mutism, ataxia, behavioural disturbance and cranial nerve deficits. MRI scans performed at three time points were assessed by neuroradiologists blinded to the diagnosis of pCMS., Results: Of 56 children included, 12 (21.4%) developed pCMS as judged by a core symptom of mutism. pCMS was more common in those aged 5 or younger. There was no statistically significant difference in pre-operative distortion or signal change of the dentate or red nuclei or superior cerebellar peduncles (SCPs) between those who did and did not develop pCMS. In both early (median 5 days) and late (median 31 months) post-operative scans, T2-weighted signal change in SCPs was more common in the pCMS group (p = 0.040 and 0.046 respectively). Late scans also showed statistically significant signal change in the dentate nuclei (p = 0.024)., Conclusions: The development of pCMS could not be linked to any observable changes on pre-operative structural MRI scans. Post-operative T2-weighted signal change in the SCPs and dentate nuclei underlines the role of cerebellar efferent injury in pCMS. Further research using advanced quantitative MRI sequences is warranted given the inability of conventional pre-surgical MRI to predict pCMS.

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.

Background: Posterior fossa syndrome (PFS) is a severe, postoperative complication occurring in about 25 % of pediatric patients undergoing surgery for midline tumors in the posterior fossa. In recent years, MRI-based research elucidated the cause of PFS and shed new light on its putative pathophysiology. This review highlights the major advances arising from research of this topic., Results: Postsurgical damage patterns in patients after posterior fossa surgery show that PFS results from bilateral surgical damage to the proximal efferent cerebellar pathways (pECPs). Surgical pECP disruption has other MRI-detectable effects that are more remote. Patients with PFS tend to develop global supratentorial cortical hypoperfusion, likely representing reversed, cerebello-cerebral diaschisis. Because this hypoperfusion is most prominent in frontal regions, cerebellar mutism may indicate a dominantly frontal lobe dysfunction, hence a peculiar form of speech apraxia. Injury to the pECP also leads to contralateral inferior olivary nucleus degeneration. When bilateral hypertrophic olivary degeneration (HOD) is observed after posterior fossa surgery, affected patients have clinical PFS. Therefore, it is suggested that bilateral HOD may be a sensitive and, in appropriate clinical settings, reliable a posteriori surrogate imaging indicator of bilateral disruption of the pECPs and consequently of PFS. Having such a "validation tool" presents new opportunities to develop better definitions for the phenotypes within the clinical spectrum of PFS., Conclusions: Anatomical and functional MRI techniques are suitable and valuable tools with which to detect structural changes and pathophysiological processes in the development and evolution of PFS and may be key, integral components of future clinical research endeavors.

Up to 39% of children operated for a posterior fossa tumor develop the cerebellar mutism syndrome. Although they are alert and cooperative, with normal language comprehension, they are unable to speak. In addition, patients may demonstrate apathy, bladder and bowel incontinence and long-term language and cognitive disturbances. This devastating syndrome is at the same time intriguing, because it confirms a role for the cerebellum in language and cognitive functions. Recent investigations have led to new insights regarding the cerebellar mutism syndrome. The commonly accepted hypothesis states that the mutism is caused by a hypofunction of cerebral hemispheres, due to damage to the superior cerebellar peduncle and functional disruption of the cerebello-cerebral circuitry. This article focuses on the evidence for and against this hypothesis and its clinical consequences., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Varicella (chickenpox) is a common childhood infection caused by the varicella-zoster virus, which is often self-limiting and usually benign. Although uncommon, neurologic complications of varicella have been documented that include postinfectious cerebellar ataxia, meningoencephalitis, Reye syndrome, myelitis, optic neuritis, stroke, Guillain-Barré syndrome, seventh cranial nerve palsy, and Ramsay-Hunt syndrome. In this case study, the authors describe a 7-year-old girl who presented with varicella skin rash with unsteady gait and anarthria on day 2, and her condition was attributed to cerebellar mutism. To date, this complication has never been reported in a child with primary varicella infection. Therefore, this case study documents a rare but serious complication of childhood chickenpox., (© The Author(s) 2013.)

Mutism of cerebellar origin is a well-described clinical entity that complicates operations for posterior fossa tumors, especially in children. This review focuses on the current understanding of principal pathophysiological aspects and risk factors, epidemiology, clinical characteristics, treatment strategies, and outcome considerations. The PubMed database was searched using the term cerebellar mutism and relevant definitions to identify publications in the English-language literature. Pertinent publications were selected from the reference lists of the previously identified articles. Over the last few years an increasing number of prospective studies and reviews have provided valuable information regarding the cerebellar mutism syndrome. Importantly, the clarification of principal terminology that surrounds the wide clinical spectrum of the syndrome results in more focused research and more effective identification of this entity. In children who undergo surgery for medulloblastoma the incidence of cerebellar mutism syndrome was reported to be 24%, and significant risk factors so far are brainstem involvement and midline location of the tumor. The dentate-thalamo-cortical tracts and lesions that affect their integrity are considered significant pathophysiological issues, especially the tract that originates in the right cerebellar hemisphere. Moderate and severe forms of the cerebellar mutism syndrome are the most frequent types during the initial presentation, and the overall neurocognitive outcome is not as favorable as thought in the earlier publications. Advanced neuroimaging techniques could contribute to identification of high-risk patients preoperatively and allow for more effective surgical planning that should focus on maximal tumor resection with minimal risk to important neural structures. Properly designed multicenter trials are needed to provide stronger evidence regarding effective prevention of cerebellar mutism and the best therapeutic approaches for such patients with a combination of pharmacological agents and multidisciplinary speech and behavior augmentation.

A 55-year-old female is presented with transient cerebellar mutism caused by a well-circumscribed left pontine infarction due to postoperative basilar perforator occlusion. Although conventional T2 imaging shows a well-demarcated lesion confined to the pontine region, diffusion tensor imaging shows an asymmetry in fractional anisotropy in the superior cerebellar peduncle. This supports the general hypothesis that cerebellar mutism is caused by functional disruption of the dentate-rubro-thalamic tract. Correlating postoperative anatomic changes to a heterogenic clinical syndrome remains challenging, however.

Background: The transcerebellomedullary fissure (trans-CMF) approach is safe and effective. Nevertheless, previous research documented a few differences in the use of this approach with regard to the opening portion of the fissure and roof of the ventricle. Here, we present a series of patients with fourth ventricular lesions and our experience using the trans-CMF approach., Methods: Fifty patients who underwent the trans-CMF approach were analyzed. The tela choroidea was simply incised in 32 patients: 27 unilaterally and 5 bilaterally. Both the tela and inferior medullary velum were cut in 18 patients: 16 unilaterally and 2 bilaterally. Unless the tumor extended below the C1 level, C1 was preserved intact. Brainstem mapping (BSM) and corticobulbar tract (CBT) motor-evoked potential (MEP) monitoring were used., Results: Gross total removal was achieved in 41 (82 %) cases, and sub-total removal was achieved in 9 (18 %) cases. Two deaths occurred 1-2 months postoperatively because of pulmonary complications. Four patients developed temporary mutism, all of whom underwent the bilateral trans-CMF approach (this rate is significantly higher than that of the unilateral approach, P < 0.05). No permanent neurological deficit occurred., Conclusion: The trans-CMF approach provides excellent access to fourth ventricular lesions without splitting the vermis. The opening portion of the fissure and roof of the ventricle should be determined by the location, extension and size of the lesion. In most cases, the unilateral trans-CMF approach with only a tela choroidea incision is adequate; this procedure is mini-invasive and possibly prevents postoperative mutism.

Objective: Fronto-cerebellar association fibers (FCF) are involved in neurocognitive regulatory circuitry. This may also be relevant for cerebellar mutism syndrome (CMS) as a complication following posterior fossa tumor removal in children. In the present study, we investigated FCF by diffusion tensor imaging in affected children and controls., Methods: Diffusion-weighted MR imaging at 3 T (GE) allowed tractography of FCF using a fiber tracking algorithm software (Brainlab 2.6) in 29 patients after posterior fossa tumor removal and in 10 healthy peers. Fiber tract volumes were assessed and fiber signals were evaluated in a semiquantitative manner along the anatomical course., Results: Volumes of FCF revealed significant diminished values in pediatric patients with symptoms of CMS (19.3 ± 11.7 cm(3)) when compared with patients without symptoms of CMS (26.9 ± 11.9 cm(3)) and with healthy peers (36.5 ± 13.82 cm(3)). In medulloblastoma patients, the volume of FCF was also significantly reduced in patients with symptoms of CMS despite having the same antitumor therapy. In semiquantitative analysis of the fiber tract signals, differences were observed in the superior cerebellar peduncles and midline cerebellar structures in patients with symptoms of CMS., Conclusion: Using DTI, which allows the visualization of fronto-cerebellar fiber tracts, lower FCF tract volumes and diminished fiber signal intensities at the level of the superior cerebellar peduncles and in midline cerebellar structures were identified in patients with postoperative symptoms of CMS. Our study refers to the role of a neural circuitry between frontal lobes and the cerebellum being involved in neurocognitive impairment after posterior fossa tumor treatment in children.

Mutism and Subsequent Dysarthria (MSD) and the Posterior Fossa Syndrome (PFS) have become well-recognized clinical entities which may develop after resection of cerebellar tumours. However, speech characteristics following a period of mutism have not been documented in much detail. This study carried out a perceptual speech analysis in 24 children and adolescents (of whom 12 became mute in the immediate postoperative phase) 1-12.2 years after cerebellar tumour resection. The most prominent speech deficits in this study were distorted vowels, slow rate, voice tremor, and monopitch. Factors influencing long-term speech disturbances are presence or absence of postoperative PFS, the localisation of the surgical lesion and the type of adjuvant treatment. Long-term speech deficits may be present up to 12 years post-surgery. The speech deficits found in children and adolescents with cerebellar lesions following cerebellar tumour surgery do not necessarily resemble adult speech characteristics of ataxic dysarthria., (Copyright © 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Epileptic discharges are not considered a part of the clinical picture of selective mutism, and electroencephalography is generally not recommended in its work-up. This report describes 6 children with selective mutism who were found to have a history of epilepsy and abnormal interictal or subclinical electroencephalography recordings. Two of them had benign epilepsy of childhood with centro-temporal spikes. The mutism was not related in time to the presence of active seizures. While seizures could be controlled in all children by medications, the mutism resolved only in 1. Although the discharges could be coincidental, they might represent a co-morbidity of selective mutism or even play a role in its pathogenesis. Selective mutism should be listed among the psychiatric disorders that may be associated with electroencephalographic abnormalities. It can probably be regarded as a symptom of a more complicated organic brain disorder.

Rotavirus is one of the most common causes of gastroenteritis in children and is known to accompany some neurological disorders such as encephalitis/encephalopathy and seizures. Although cerebellar disorders sometime occur as a complication of rotavirus gastroenteritis in Japan, few reports have addressed these issues. Here, we report three cases of insulted cerebellums in addition to encephalitis/encephalopathy associated with rotavirus. Similar to posterior fossa syndrome after surgery, mutism was a notable symptom that lasted about 1 month. Brain diffusion-weighted imaging (DWI) revealed chronological changes, i.e., marked hyperintensity in the bilateral dentate nucleus followed by the vermis and cerebellar hemisphere. The bilateral dentate nucleus is known to be a key lesion site for mutism, and these clinical and radiological findings may be tightly connected in rotavirus-associated cerebellitis/cerebellopathy., (Copyright © 2010 Elsevier B.V. All rights reserved.)

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.

Acute disseminated encephalomyelitis is a demyelinating process affecting multiple areas of the central nervous system, frequently including the cerebellum. Cerebellar insult may lead to absence of speech or cerebellar mutism. Cerebellar mutism often occurs in young children after posterior fossa tumor resection, and generally appears as part of a larger subset of neurobehavioral signs and personality changes known as posterior fossa syndrome. Information on the impact of widespread cerebellar involvement on speech production, behavior, and long-term outcomes in acute disseminated encephalomyelitis is limited. We describe cases of acute disseminated encephalomyelitis with predominantly cerebellar involvement, with specific attention to cerebellar mutism. We conducted a retrospective chart review of children diagnosed with acute disseminated encephalomyelitis between 2005-2009 at a pediatric multiple sclerosis and demyelinating disorders clinic. Of 19 patients diagnosed with acute disseminated encephalomyelitis, six (32%) manifested primary cerebellar involvement. Of these six, four (67%) exhibited acute language disturbance, with three (50%) exhibiting mutism. The three patients with cerebellar mutism experienced protracted speech and language deficits after follow-ups from 6 months to 4 years. Widespread cerebellar involvement in acute disseminated encephalomyelitis may result in cerebellar mutism, in addition to persistent neurocognitive and behavioral problems., (Copyright 2010 Elsevier Inc. All rights reserved.)

Object: The origin and long-term outcome of cerebellar mutism syndrome (CMS), a postoperative syndrome of diminished speech, hypotonia, and ataxia that affects approximately 25% of patients with medulloblastoma, is poorly elucidated. The current study was undertaken to determine factors associated with development of CMS, a means to determine its severity or cause, and outcomes of patients with this syndrome., Methods: The study included 28 children with medulloblastoma who either underwent an operation or were referred to the authors' institution soon after surgery. Eleven (39%) of these children had CMS. The preoperative, immediate postoperative, and 1-year postoperative MR images were reviewed by a neuroradiologist blinded to diagnosis of CMS. The severity of mutism and neurological and neurocognitive outcomes were examined., Results: Preoperative MR images showed no differences in tumor size, hydrocephalus, or peritumoral edema in patients with and without CMS. An association with brainstem invasion was significant (p < 0.05), and there was a trend toward an association with involvement of the cerebellomedullary angle (p = 0.08). Images obtained immediately postoperatively showed cerebellar edema in 92% of all patients; there were trends for more middle and superior cerebellar peduncle edema in patients with CMS (p = 0.05 and 0.07, respectively). At 1 year postoperatively, patients with CMS showed more moderate to severe atrophy/gliosis of total cerebellum (p < 0.01), vermis (p < 0.01), and brainstem (p < 0.05). Mean IQ was 16 points lower in patients with CMS (IQ = 84.2 +/- 15.8) compared with those without CMS (IQ = 100.4 +/- 17.4), with a trend toward significance (p = 0.07)., Conclusions: This study demonstrates that CMS is associated with postoperative damage to the cerebellum and brainstem, damage not predicted by immediate postoperative MR imaging, and with poorer associated functional outcome.

Background: Transient mutism as a consequence of posterior fossa surgery is a well-known phenomenon. However, it has rarely been reported after focal nonsurgically induced cerebellar damage., Case Report: We describe a 7-year-old child affected by a tumor arising from the quadrigeminal plate who developed transient cerebellar mutism after a spontaneous bleeding which extended to the upper cerebellar vermis. The recovery from mutism started about a week after the bleeding. At the time of the surgical treatment, 8 weeks after the spontaneous intratumoral bleeding, she was only dysarthric., Discussion: To our knowledge, this is the first pediatric case of presurgical cerebellar mutism due to a hemorrhage of a neoplastic lesion. Moreover, the focality of bleeding allows the confirmation of the role played by the upper vermis in speech control as well as exclusion of surgically induced lesions commonly suggested as possible cause of the cerebellar mutism.

Introduction: First described 15 years ago, primary progressive anarthria is a focal cortical atrophy defined as a rare progressive impairment of speech associated with orofacial apraxia and leading to mutism with a frontal lobe syndrome. The aim of this study was to analyze clinical and neuropsychological data and results of complementary tests in a series of patients presented with primary progressive anarthria., Material and Methods: We, retrospectively, studied five patients with primary progressive anarthria. We particularly analyzed the following parameters: age at onset, age at the diagnostic, disease time from onset to first consultation, the initial orientation, the neuropsychological and clinical data at the first visit, electromyography, brain MRI, and single photon emission computed tomography (SPECT) findings. Clinical and neuropsychological data were used to monitor disease course., Results: The mean age at onset of symptoms was 75.2+/-5.8 years. Patients were primarily referred to a specialist in memory disease (n=3) or a specialist in motor neuron disease (n=2). The time from onset to first consultation was 11.2+/-3 months. Anarthria was associated with dysexecutive syndrome and sometimes, with impaired comprehension. Electromyography was always normal. Cranial MRI showed temporal or left frontal atrophy (n=3). Spect revealed decreased cerebral blood flow predominating in the left frontal or temporal region (n=4)., Conclusion: Long delay for specialist consultation and inadequate initial orientation retard disease diagnosis, leading to severe incapacity. Complementary studies are required to confirm diagnostic and to rule out lateral amyotrophic sclerosis. During the early stages, involvement of the premotor cortex may be considered due to the speech apraxia. Secondary motor orofacial disturbances suggest an extension to the motor cortex. Primary progressive anarthria is a distinct individual entity within the spectrum of focal cortical atrophies.

The present study aims to demonstrate that errors when writing are more common than expected in patients affected by primary lateral sclerosis (PLS) with severe dysarthria or complete mutism, independent of spasticity. Sixteen patients meeting Pringle's et al. [34] criteria for PLS underwent standard neuropsychological tasks and evaluation of writing. We assessed writing abilities in spelling through dictation in which a set of words, non-words and short phrases were presented orally and by composing words using a set of preformed letters. Finally, a written copying task was performed with the same words. Relative to controls, PLS patients made a greater number of spelling errors in all writing conditions, but not in copy task. The error types included: omissions, transpositions, insertions and letter substitutions. These were equally distributed on the writing task and the composition of words with a set of preformed letters. This pattern of performance is consistent with a spelling impairment. The results are consistent with the concept that written production is critically dependent on the subvocal articulatory mechanism of rehearsal, perhaps at the level of retaining the sequence of graphemes in a graphemic buffer. In PLS patients a disturbance in rehearsal opportunity may affect the correct sequencing/assembly of an orthographic representation in the written process.

Patients with progressive nonfluent aphasia (PNFA) can become mute early in the course of the disease. Voxel-based morphometry showed that PNFA is associated with left anterior insula and inferior frontal atrophy. In PNFA with early mutism, volume loss was more prominent in the pars opercularis and extended into the left basal ganglia. Damage to the network of brain regions involved in both coordination and execution of speech causes mutism in PNFA.

Background: Mutism has been associated with injury to midline cerebellar structures secondary to degenerative disease, tumors, hemorrhage, or surgery. Typically, cerebellar mutism syndrome (CMS) has been seen in children and only rarely described in adults after surgery of the posterior fossa. This syndrome typically arises 48 hours after the initiating event and resolves approximately 7 to 8 weeks later. Characteristics of CMS include complete absence of speech without impaired consciousness, other cranial nerve deficits, or long tract signs., Case Description: The authors report on 2 patients each of whom developed cerebellar mutism after tumor resection using a posterior fossa approach. The first patient underwent gross total resection of a pineal region tumor via a supracerebellar approach. The second patient underwent posterior fossa decompression for a left cerebellar hemispheric renal cell carcinoma metastasis with adjacent hemorrhage. One patient displayed a variant of cerebellar mutism with severe ataxic dysarthria without complete absence of speech, whereas the other demonstrated frank mutism. After neuroimaging studies confirmed the absence of a surgically treatable postoperative cause for the patients' symptoms, they were managed in a supportive fashion (eg, speech therapy) and improved within 3.5 months and 1 year, respectively., Conclusion: It is paramount that neurosurgeons be aware of cerebellar mutism with regard to its very rare occurrence in adults, its time of onset, and typical self-limiting course.

The aim of the present study was to investigate the manifestations of mutism after surgery in children with cerebellar tumors. Speech impairment following cerebellar mutism in children was investigated based on standardized acoustic speech parameters and perceptual criteria. Mutistic and non-mutistic children after cerebellar surgery as well as orthopedic controls were tested pre-and postoperatively. Speech impairment was compared with the localization of cerebellar lesions (i. e. affected lobules and nuclei). Whereas both control groups showed no abnormalities in speech and behavior, the mutistic group could be divided into children with dysarthria in post mutistic phase and children with mainly behavioral disturbances. In the mutistic children involvement of dentate and fastigial nuclei tended to be more frequent and extended than in the nonmutistic cerebellar children. Cerebellar mutism is a complex phenomenon of at least two types. Dysarthric symptoms during resolution of mutism support the anarthria hypothesis, while mainly behavioral changes suggest an explanation independent from speech motor control.

Transient mutism after posterior fossa surgery in children or associated with cerebellar hemorrhage or trauma is a recognized phenomenon. However, its association with parainflammatory cerebellitis has been rarely described. We report on a previously healthy 3-year-old child with severe cerebellitis after acute gastroenteritis of unidentified cause. Severe ataxia and transient mutism were the prevailing clinical features. Magnetic resonance imaging revealed swelling of the cerebellum with protruding cerebellar tonsils at the level of the occipital foramen. Recovery from the acute illness was slow and incomplete. Residual cerebellar dysfunction manifested with dysphonic and dysarthric speech, as well as motor coordination problems and was associated with atrophy of the vermis and cerebellar hemispheres in follow-up studies.

Transient cerebellar eye closure (TCES) is a complication of cerebellar tumor surgery in children and is almost exclusively observed in the context of the syndrome of mutism and subsequent dysarthria. As knowledge about the course of transient cerebellar eye closure is absent, we describe in detail the clinical picture in a 14-year-old girl. The process of improvement of TCES is characterized by four distinct phases, can last more than 1 month and may be associated with severe persistent cerebellar dysfunction., (Copyright 2003 S. Karger AG, Basel)

Tacrolimus (FK506), an immunosuppressant, has been associated with mutism in adults after liver transplant. Speech arrest, agitation, tremor, ataxia, and downward gaze deviation in a 5-year-old female 13 days after orthotopic liver transplant are reported. FK506, which began to be administered 12 days earlier, rose to a level of 44 ng/mL (normal range, 10-20 ng/mL) 1 day before neurologic abnormalities began. FK506 dose level was maintained and then reduced. Three days later the patient could say a few single words and extra-ocular movement returned to normal. Four months later, she continued to exhibit decreased fluency and dysarthria with ataxia. One year later, decreased fluency and mild ataxia persists. Rapid identification of speech loss linked to FK506 may be important because reduction or cessation of the drug may be associated with reverse of speech loss.

Cerebellar mutism (anarthria) is a well-described complication of posterior fossa tumor resection. It is accompanied by a characteristic behavior including irritability and autistic features. This syndrome is typically reversible within days to months. Underlying pathophysiology is unknown. We describe two children who presented with a similar clinical finding after nonsurgical cerebellar involvement, hemolytic-uremic syndrome in one and cerebellitis in the other. Postmortem pathologic findings in the first patient indicated cerebellar ischemic necrosis. Single-photon emission computed tomography in the second patient revealed diffuse cerebellar hypoperfusion with no supratentorial abnormalities, refuting a phenomenon of diaschisis between cerebellar and frontal connections. These findings confirm that this clinical syndrome may occur in a nonsurgical, nontraumatic context. They are consistent with recent integrative hypotheses explaining cerebellar anarthria.

Three types of researches have been carried out on brain-mind relationships: 1. researches on anatomical correlates of special talents (for example, perfect pitch) or deficits (for example, dyslexia), 2. researches to examine the relationship between a given cognitive syndrome and the site of brain damage, 3. researches to localize human cognitive function in the brain in vivo using functional magnetic resonance imaging (fMRI) and positron emission tomography (PET). fMRI is a particularly important because it is noninvasive. A tutorial covering basic aspects of this methodology is presented, along with a survey of recent fMRI data related to clinical application. Future investigations of the three types enumerated above are expected to further clarify brain-mind relationships.

When a child does not speak, this may be because there is no wish to do so (elective or selective mutism), or the result of lesions in the brain, particularly in the posterior fossa. The characteristics of the former children are described, especially their shyness; and it is emphasized that mild forms are quite common and a definitive diagnosis should only be made if the condition is significantly affecting the child and family. In the case of mutism due to organic causes, the commonest of these is trauma to the cerebellum. Operations on the cerebellum to remove tumours can be followed by mutism, often after an interval of a few days, and it may last for several months or longer, to be followed by dysarthria. Other rarer causes are discussed, and also the differential diagnosis. The so-called posterior fossa syndrome consists of mutism combined with ataxia, cranial nerve palsies, bulbar palsies, hemiparesis, cognitive impairment and emotional lability, but the post-operative symptoms are often dominated by the lack of speech. The most accepted cause for the condition is vascular spasm with involvement of the dentate nucleus and the dentatorubrothalamic tracts to the brain-stem, and subsequently to the cortex. Diaschisis may be involved in causing the loss of higher cerebral functions, and possibly, complicating hydrocephalus. The treatment of elective mutism is reviewed, either using a psychotherapeutic approach or a variety of drugs, or both. These may well be ineffective, and it must be remembered that the condition often resolves on its own. The former treatment must concentrate on the training of social skills and activities of daily life and must be targeted to both the child, the family, and the school. Also, all kinds of punishment and insistence on speech must be discouraged. The drug, which seems to be most effective, is fluoxetine. Discovering more about the causes of mutism due to organic causes may well depend on studies using such techniques as magnetic resonance imaging and single photon emission tomography.

A 24-year-old female developed high fever and unconsciousness for several days at the age of 3. She was said to have meningitis. Subsequently her verbal development was markedly retarded so that she was kept at a deaf-and-dumb institute. At the age of 19, she was transfered to the mental hospital because she occasionally became very wild without any reasons. She suddendly died at the age of 24 due to paralytic ileus. Her autopsy showed bilateral cavities in the white matter of the anterior portion of the temporal lobes and the striatum. Microscopically, the tissues around the cavities were necrotic and devastated with loss of neurones. Glio-mesodermal reaction was mild. Besides marked thickening of meninges and micropolygyria of the cerebellar hemispheres were demonstrated.

Mutism following brain trauma is quite common, is usually transient, and recovery of speech is essentially the rule. Lasting total absence of speech without aphasia is highly unusual. Three such patients, two of traumatic and one due to vascular origin showing buccofacial apraxia (BFA) and computerized tomography (CT) evidence of bilateral frontal lesions are reported. It is suggested that complete lasting mutism associated with BFA is a result of bihemispheric lesions affecting mainly the opercular part of the inferior frontal gyrus and immediate adjacent regions.