Characterising the Long-Term Language Impairments of Children Following Cerebellar Tumour Surgery by Extracting Psycholinguistic Properties from Spontaneous Language.

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.

Case report: the effects of cerebellar tDCS in bilingual post-stroke aphasia.

Transcranial Direct Current Stimulation may be a useful neuromodulation tool for enhancing the effects of speech and language therapy in people with aphasia, but research so far has focused on monolinguals. We present the effects of 9 sessions of anodal cerebellar tDCS (ctDCS) coupled with language therapy in a bilingual patient with chronic post-stroke aphasia caused by left frontal ischemia, in a double-blind, sham-controlled within-subject design. Language therapy was provided in his second language (L2). Both sham and anodal treatment improved trained picture naming in the treated language (L2), while anodal ctDCS in addition improved picture naming of untrained items in L2 and his first language, L1. Picture description improved in L2 and L1 after anodal ctDCS, but not after sham.

Executive functions in primary progressive aphasia: A meta-analysis.

Executive functions (EFs) refer to a set of cognitive processes, specifically shifting, inhibition, updating of working memory, and are involved in the cognitive control of behavior. Conflicting results have been reported regarding impairments of EFs in Primary Progressive Aphasia (PPA). We performed a multi-level meta-analysis to confirm whether deficits of EFs exist in this population, focusing on a common EFs composite, and the components shifting, inhibition and updating separately. We included 141 studies that report on 294 EFs tasks. The overall mean weighted effect size was large (d = -1,28), indicating poorer EFs in PPA as compared to age-matched cognitively healthy controls. Differences between effect sizes of the EFs components were not significant, indicating all components are affected similarly. Overall, moderator analysis revealed that PPA variant and disease duration were significant moderators of performance, while task modality and years of education were not. The non-fluent/agrammatic PPA and the logopenic PPA variants were similarly affected, but the semantic variant was affected to a lesser extent. We discuss implications for clinical and research settings, and future research.

A Systematic Review of Transcranial Direct Current Stimulation in Primary Progressive Aphasia: Methodological Considerations.

A variety of tDCS approaches has been used to investigate the potential of tDCS to improve language outcomes, or slow down the decay of language competences caused by Primary Progressive Aphasia (PPA). The employed stimulation protocols and study designs in PPA are generally speaking similar to those deployed in post-stroke aphasic populations. These two etiologies of aphasia however differ substantially in their pathophysiology, and for both conditions the optimal stimulation paradigm still needs to be established. A systematic review was done and after applying inclusion and exclusion criteria, 15 articles were analyzed focusing on differences and similarities across studies especially focusing on PPA patient characteristics (age, PPA variant, language background), tDCS stimulation protocols (intensity, frequency, combined therapy, electrode configuration) and study design as recent reviews and group outcomes for individual studies suggest tDCS is an effective tool to improve language outcomes, while methodological approach and patient characteristics are mentioned as moderators that may influence treatment effects. We found that studies of tDCS in PPA have clinical and methodological and heterogeneity regarding patient populations, stimulation protocols and study design. While positive group results are usually found irrespective of these differences, the magnitude, duration and generalization of these outcomes differ when comparing stimulation locations, and when results are stratified according to the clinical variant of PPA. We interpret the results of included studies in light of patient characteristics and methodological decisions. Further, we highlight the role neuroimaging can play in study protocols and interpreting results and make recommendations for future work.

Reintegration into school of childhood brain tumor survivors: a qualitative study using the International Classification of Functioning, Disability and Health - Children and Youth framework.

PURPOSE: The purpose of this study was to describe experiences of childhood brain tumor survivors (CBTS) and key figures in their environment after returning to school, using the International Classification of Functioning, Disability and Health - Children and Youth framework. METHODS: We conducted semi-structured interviews with five children, nine parents, 28 teachers, and 14 health professionals at three predetermined times over a two-year period. A qualitative content analysis was performed by linking meaningful units from transcribed interviews to ICF codes. RESULTS: Children experienced diverse body dysfunctions that could impede school participation, mostly related to knowledge acquisition and application, executive functioning and social life. Support at school and professional aftercare were essential to alleviate the child's difficulties. The teacher's attitude, parental involvement, and practices of collaboration belonging to the child's supportive network further influenced the reintegration process. Also, child-specific factors including emotional reactions to illness, age, and balance between school life and leisure time were considered to affect school readjustment. CONCLUSIONS: The International Classification of Functioning, Disability and Health framework is a useful instrument to systematically describe experiences of CBTS and key figures regarding school life, providing a common language to communicate for stakeholders in education and healthcare following the child's return.IMPLICATIONS FOR REHABILITATIONThe International Classification of Functioning, Disability and Health - Children and Youth framework is a useful instrument to describe experiences regarding school life of childhood brain tumor survivors (CBTS) and key figures in their environment.The ICF-CY model can be combined with a standardized assessment of neurocognition or performance to achieve a comprehensive view of the child's participation at school, by exploring both personal and environmental factors.Childhood brain tumor survivors mostly show impairments in acquiring and applying knowledge, executive functioning and social life, which may require increased support at school and professional aftercare.A positive attitude of school staff, high parental involvement in school life and adequate collaboration between parents, education and healthcare are important to prioritize in the context of reintegration into school of CBTS.

Post-operative cerebellar mutism syndrome: rehabilitation issues.

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.

Posterior Fossa Society Consensus Meeting 2018: a synopsis.

PURPOSE: The Posterior Fossa Society (PFS) was founded 4 years ago to systematically gather and exchange information on the post-operative (CMS) and cerebellar cognitive affective syndrome (CCAS). With its consensus meetings, the PFS orchestrates research studies in the field of cerebellar injury and progresses the knowledge of post-operative pediatric cerebellar mutism syndrome (CMS). In this article, we captured the 3-day program of presentations, group discussions, interactive workshops, and dialogue, highlighting the key topic areas of CMS and its research advances. METHODS: This synopsis is based on the third consensus meeting which was held in Reykjavik, Iceland, in August 2018. RESULTS: Three working groups have been defined to drive the future research priorities on post-surgical CMS: (i) refining definition and symptoms scoring of CMS; (ii) understanding the pathogenesis and enhancing risk-stratification strategies; and (iii) developing rehabilitation approaches and protocols. CONCLUSIONS: The third consensus meeting highlighted a unanimous desire for data-driven information to advance the knowledge and guide future research efforts. The PFS constitutes an established and expanding network of multi-disciplinary expertise that can facilitate the development of collaborative studies and produce official guidelines on the topic.

Reintegration Into School After Treatment for a Brain Tumor: The Child's Perspective.

This multiple case study investigated perspectives of childhood brain tumor survivors on reintegration into school over a 2-year period. Semistructured interviews were conducted with 5 children at 3 times to obtain an extensive view of their overall school experience. Thematic analysis of data resulted in 4 themes: "school life and participation," "peer relations and friendships," "performance and difficulties," and "support and follow-up." Childhood brain tumor survivors consider school attendance as part of a normal disease-free life. Social contact and friendships represent their main motivating factors for returning to school. Attitudes and feelings regarding performance, difficulties, and support vary among survivors and change over time. In conclusion, continuity in learning and social contact established before the return facilitate the reintegration process. A comprehensive assessment of their academic and psychosocial functioning should be organized on reentry. Systematic follow-up by parents, school staff, and health professionals throughout the child's school career is required.

Attending school after treatment for a brain tumor: Experiences of children and key figures.

Reintegration into school is a milestone for childhood brain tumor survivors, as well as for their parents, teachers, and healthcare providers. We explored their experiences following the school re-entry by conducting semi-structured interviews. Thematic analysis resulted in four main themes: "school performance," "psychosocial well-being," "support and approach," and "communication and collaboration." Children were pleased to return to school despite confrontation with adverse outcomes. Parents, teachers, and healthcare providers identified current and future concerns and challenges, as well as opportunities for academic and personal development. Their experiences highlight the importance of coordinated and systematic follow-up in close collaboration with healthcare providers.

Short-term perspectives of parents and teachers on school reintegration of childhood brain tumour survivors.

Objective: To discover short-term changes in perspectives of parents and teachers of childhood brain tumour survivors on school reintegration, in order to reveal similarities and differences between them over time. Methods: Semi-structured interviews were conducted with parents and teachers of five children at the start and the end of a 1-year period following the child's school re-entry. Results: Thematic analysis of data resulted in three main themes: 'the child's performance and wellbeing', 'the school's attitude and approach' and 'communication and working together'. Parental concerns about child-specific changes and the school's approach to the child could either decrease or increase over time. Teachers remained focused on assessing their pupil's learning potential and finding ways of appropriate support. Their different perspectives on communication and working together became more pronounced. Conclusions: This study emphasizes the importance of clear communication and collaboration, coordinated follow-up and availability of healthcare professionals during the child's school reintegration.

Acquired epileptic opercular syndrome related to a heterozygous deleterious substitution in GRIN2A.

Epileptic encephalopathies with continuous spike-and-waves during sleep (CSWS) are characterized by cognitive or language impairment, and are occasionally associated with pathogenic variants of the GRIN2A gene. In these disorders, speech dysfunction could be either related to cerebral dysfunction caused by the GRIN2A deleterious variant or intense interictal epileptic activity. Here, we present a patient with apraxia of speech, clearly linked to severity of epilepsy, carrying a GRIN2A variant. A 6-year-old boy developed acute regression of expressive language following epileptic seizures, leading to complete mutism, at which time EEG revealed CSWS. MEG showed bilateral superior parietal and opercular independent CSWS onsets and PET with fluorodeoxyglucose demonstrated significant increase in relative glucose metabolism in bilateral superior parietal regions. Corticosteroids induced a regression of CSWS together with impressive improvement in speech abilities. This case supports the hypothesis of a triggering role for epileptic discharges in speech deterioration observed in children carrying a deleterious variant of GRIN2A. When classic antiepileptic drugs fail to control epileptic activity, corticosteroids should be considered. Multimodal functional neuroimaging suggests a role for opercular and superior parietal areas in acquired epileptic opercular syndrome. [Published with video sequences on www.epilepticdisorders.com].

Perceptual Accent Rating and Attribution in Psychogenic FAS: Some Further Evidence Challenging Whitaker's Operational Definition.

A 40-year-old, non-aphasic, right-handed, and polyglot (L1: French, L2: Dutch, and L3: English) woman with a 12-year history of addiction to opiates and psychoactive substances, and clear psychiatric problems, presented with a foreign accent of sudden onset in L1. Speech evolved toward a mostly fluent output, despite a stutter-like behavior and a marked grammatical output disorder. The psychogenic etiology of the accent foreignness was construed based on the patient's complex medical history and psychodiagnostic, neuropsychological, and neurolinguistic assessments. The presence of a foreign accent was affirmed by a perceptual accent rating and attribution experiment. It is argued that this patient provides additional evidence demonstrating the outdatedness of Whitaker's (1982) definition of foreign accent syndrome, as only one of the four operational criteria was unequivocally applicable to our patient: her accent foreignness was not only recognized by her relatives and the medical staff but also by a group of native French-speaking laymen. However, our patient defied the three remaining criteria, as central nervous system damage could not conclusively be demonstrated, psychodiagnostic assessment raised the hypothesis of a conversion disorder, and the patient was a polyglot whose newly gained accent was associated with a range of foreign languages, which exceeded the ones she spoke.

Unassisted assessment of stroke severity using telemedicine.

BACKGROUND AND PURPOSE: Quantification of stroke severity through telemedicine consultation is challenging and relies on professional support at the patient's bedside. We aimed to develop a novel scale for assessing stroke severity through telemedicine without assistance from a third party (Unassisted TeleStroke Scale [UTSS]). METHODS: Stroke severity was assessed in 45 patients with suspicion of acute stroke by bedside examination using the National Institutes of Health Stroke Scale (NIHSS) and by teleconsultation using the UTSS. Scale reliability was evaluated by intrarater and interrater variability, internal consistency, and rater agreement. Concurrent and predictive validity were tested by relating the UTSS with the NIHSS and long-term outcome (modified Rankin Scale and mortality at 6 months). Clinimetric analysis of the UTSS was obtained via the Rasch model. RESULTS: The mean examination time for the UTSS was 3.1 minutes (SD, 1.1) versus 8.5 minutes for the NIHSS (SD, 2.6; P<0.001). Both UTSS and NIHSS showed excellent intrarater variability (r=0.97 and 0.98; P<0.001) and interrater variability (r=0.96 and 0.98; P<0.001), as well as excellent internal consistency and rater agreement. The UTSS correlated strongly with the NIHSS and was identified as an independent predictor of stroke outcome in logistic regression analysis. Rasch analysis indicated that the UTSS represents a unidimensional scale of stroke severity. CONCLUSIONS: The UTSS is a rapid, reliable, and valid tool for unassisted assessment of stroke severity through telemedicine.

The cerebellum: its role in language and related cognitive and affective functions.

The traditional view on the cerebellum as the sole coordinator of motor function has been substantially redefined during the past decades. Neuroanatomical, neuroimaging and clinical studies have extended the role of the cerebellum to the modulation of cognitive and affective processing. Neuroanatomical studies have demonstrated cerebellar connectivity with the supratentorial association areas involved in higher cognitive and affective functioning, while functional neuroimaging and clinical studies have provided evidence of cerebellar involvement in a variety of cognitive and affective tasks. This paper reviews the recently acknowledged role of the cerebellum in linguistic and related cognitive and behavioral-affective functions. In addition, typical cerebellar syndromes such as the cerebellar cognitive affective syndrome (CCAS) and the posterior fossa syndrome (PFS) will be briefly discussed and the current hypotheses dealing with the presumed neurobiological mechanisms underlying the linguistic, cognitive and affective modulatory role of the cerebellum will be reviewed.

Acquired auditory agnosia in childhood and normal sleep electroencephalography subsequently diagnosed as Landau-Kleffner syndrome: a report of three cases.

AIM: We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. METHOD: Case histories including EEG, positron emission tomography findings, and long-term outcome were reviewed. RESULTS: Auditory agnosia occurred between the age of 2 years and 3 years 6 months, after a period of normal language development. Initial awake and sleep EEG, recorded weeks to months after the onset of language regression, during a nap period in two cases and during a full night of sleep in the third case, was normal. Repeat EEG between 2 months and 2 years later showed epileptiform discharges during wakefulness and strongly activated by sleep, with a pattern of continuous spike-waves during slow-wave sleep in two patients. Patients were diagnosed with LKS and treated with various antiepileptic regimens, including corticosteroids. One patient in whom EEG became normal on hydrocortisone is making significant recovery. The other two patients did not exhibit a sustained response to treatment and remained severely impaired. INTERPRETATION: Sleep EEG may be normal in the early phase of acquired auditory agnosia. EEG should be repeated frequently in individuals in whom a firm clinical diagnosis is made to facilitate early treatment.

The pace of prosodic phrasing couples the listener's cortex to the reader's voice.

We studied online coupling between a reader's voice and a listener's cortical activity using a novel, ecologically valid continuous listening paradigm. Whole-scalp magnetoencephalographic (MEG) signals were recorded from 10 right-handed, native French-speaking listeners in four conditions: a female (Exp1f) and a male (Exp1m) reading the same text in French; a male reading a text in Finnish (Exp 2), a language incomprehensible for the subjects, and a male humming Exp1 text (Exp 3). The fundamental frequency (f0) of the reader's voice was recorded with an accelerometer attached to the throat, and coherence was computed between f0 time-course and listener's MEG. Similar levels of right-hemisphere-predominant coherence were found at ˜0.5 Hz in Exps 1-3. Dynamic imaging of coherent sources revealed that the most coherent brain regions were located in the right posterior superior temporal sulcus (pSTS) and posterior superior temporal gyrus (pSTG) in Exps 1-2 and in the right supratemporal auditory cortex in Exp 3. Comparison between speech rhythm and phrasing suggested a connection of the observed coherence to pauses at the sentence level both in the spoken and hummed text. These results demonstrate significant coupling at ∼0.5 Hz between reader's voice and listener's cortical signals during listening to natural continuous voice. The observed coupling suggests that voice envelope fluctuations, due to prosodic rhythmicity at the phrasal and sentence levels, are reflected in the listener's cortex as rhythmicity of about 2-s cycles. The predominance of the coherence in the right pSTS and pSTG suggests hemispherical asymmetry in processing of speech sounds at subsentence time scales.

Auditory-perceptual speech analysis in children with cerebellar tumours: a long-term follow-up study.

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.

Cerebellar-induced apraxic agraphia: a review and three new cases.

Apraxic agraphia is a writing disorder due to a loss or lack of access to motor engrams that program the movements necessary to produce letters. Clinical and functional neuroimaging studies have demonstrated that the neural network responsible for writing includes the superior parietal region and the dorsolateral and medial premotor cortex. Recent studies of two cases with atypical lesion localisations in the left thalamus and the right cerebellum support the hypothesis that the written language network is larger than previously assumed. The aim of this study is twofold: (1) to provide a survey of cases of apraxic agraphia published between 1973 and June 2010, and (2) to provide further evidence for a role of the cerebellum in writing via three additional cases who presented with apraxic agraphia after ischemic damage in the cerebellum. Functional neuroimaging studies by means of brain perfusion SPECT showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, syntax, naming, writing and executive functioning.