Standardized reporting of adverse events and functional status from the first 5 years of awake surgery for gliomas: a population-based single-institution consecutive series.

OBJECTIVE: To report our experience and investigate frequencies of adverse events and functional status from the first 5 years of performing awake surgery for gliomas in a single-center population-based setting. METHODS: We conducted a review of all patients with a glioma treated with awake surgery during the first 5 years following introduction of awake surgery at our center (February 2015 to February 2020). We assessed functional and radiological outcome, with adverse events classified according to the Landriel-Ibanez classification for neurosurgical complications, while neurological deficits were further subdivided into transient vs permanent. We sought to analyze our initial results and learning curve, as well as compare our results with literature. RESULTS: Forty-two patients were included. The median age was 38 years (range 18-66) and 13 (31%) were female. The indication for awake surgery was a presumed glioma in or near an eloquent area. The overall 30-day complication rate was 25 (59%), with 19 (45%) grade I complications, 3 (7%) grade II complications, and 3 (7%) grade III complications. Fifteen patients (36%) experienced transient neurological deficits, and 11 (26%) permanent neurological deficits. At 3-month follow-up, the Karnofsky Performance Score was 80 or higher for the entire cohort. The median extent of resection was 87%, with GTR achieved in 11 (26%). In search of potential learning curve difficulties, patients were divided into the 21 patients treated first (Early Group) versus the remaining 21 patients treated later (Late Group); no statistically significant difference in operating time, amount of tumor removed, or incidence of long-term postoperative neurological deficit was identified between groups. No awake surgery was aborted due to seizures. Comparison to the literature was limited by the diverse and unsystematic way in which previous studies have reported adverse events after awake craniotomy for gliomas. CONCLUSION: We provide a standardized report of adverse events and functional status following awake surgery for glioma during a single-center 5-year learning period, with similar rates of severe adverse events and functional outcome compared to literature without concerns of substantial learning curve difficulties. However, this comparison was flawed by non-standardized reporting of complications, highlighting a demand for more standardized reporting of adverse events after awake craniotomies.

Language ability in 5-12-year-old children with new-onset epilepsy.

PURPOSE: This clinically based study aimed to explore and describe language ability in 5-12-year-old children with new-onset epilepsy.Participants and methods: Twenty-one consecutively recruited children (eleven boys, ten girls) with new-onset epilepsy, were assessed using Clinical Evaluation of Language Fundamentals, fourth edition (CELF-4) and additional tests for verbal fluency/word retrieval and phonology. In addition, caregivers rated their child's speech, language, and communication in everyday context. Based on available tests and clinical observation, an overall evaluation of language ability was made to distinguish children with language disorders and children with language difficulties from those with language abilities within the normal range. Language disorder was diagnosed following the ICD-10 criteria. The cutoff for language difficulties was set at 1 standard deviation below the normative mean on the CELF-4 Core Language Score and additional indices. RESULTS: Out of twenty-one children, ten (47.5%) met the criteria for a language disorder diagnosis according to ICD-10. Another five (24%) had language difficulties but did not meet the criteria for a language disorder diagnosis according to ICD-10. Hence a total of fifteen (71.5%) children had an impaired language ability affecting different domains of language, including receptive language, language memory, and semantic processing. The remaining six (28.5%) children had average language ability. CONCLUSION: In this group of children with new-onset epilepsy, a large over-representation of co-existing language disorder and language difficulties was found. The findings suggest that specific language assessments for children with new-onset epilepsy are needed, to ensure that adequate interventions and support can be offered.

Coexisting disorders and problems in preschool children with autism spectrum disorders.

OBJECTIVES: To analyze cooccurring disorders and problems in a representative group of 198 preschool children with autism spectrum disorders (ASD) who had had interventions at a specialized habilitation center. METHODS: Parents and children were seen by a research team. Data were based on parental interviews, pediatric assessments, and tests of the child. Information on autistic symptoms, general cognitive function, speech and language, motor function, epilepsy, vision, hearing, activity level, behavior, and sleep was collected. RESULTS: Three ASD categories were used: (1) autistic disorder (AD), (2) autistic-like condition (ALC) or Asperger syndrome, and (3) one group with autistic symptoms/traits but not entirely all its criteria met for ASD. Children with autism had a mean of 3.2 coexisting disorders or problems, the ALC/Asperger group had a mean of 1.6, and children with autistic traits had a mean of 1.6. The most common disorder/problems in the total group pertained to language problems (78%), intellectual disability (ID) (49%), below average motor function (37%), and severe hyperactivity/ADHD (33%). CONCLUSIONS: The results accord with the concept of early symptomatic syndromes eliciting neurodevelopmental clinical examination (ESSENCE), and highlight the need of considering ASD in a broad perspective taking also other cooccurring developmental disorders into account.

Language/cognitive predictors of literacy skills in 12-year-old children on the autism spectrum.

AIM: Some school age children with autism spectrum disorder (ASD) struggle with literacy development, yet, the individual differences are major and not well understood. Moreover, literacy attainment is multi-faceted and literacy skills and difficulties manifest in more than one way. The aim of this study was to describe this variability and to identify language/cognitive predictors of different literacy skills. METHODS: We assessed different literacy skills (word reading accuracy, reading fluency, reading comprehension, and spelling), along with a set of language/cognitive predictor skills (listening comprehension, rapid automatized naming, phonological awareness and nonverbal cognitive ability), in 12-year-old children with ASD without intellectual disability recruited from a longitudinal study in Sweden. RESULTS: There was great heterogeneity (from floor to ceiling levels) in literacy skills, with a statistically increased prevalence of poor reading comprehension and reading fluency compared with population norms. In regression analyses, it was shown that concurrent language/cognitive predictor skills (e.g. phonological awareness) were differentially associated with literacy subskills. Moreover, a longitudinal analysis showed that preschool language problems were associated with poor word reading accuracy and spelling in middle school. CONCLUSIONS: The results confirm previous findings as well as provide new knowledge regarding profiles of literacy (difficulties) in children with ASD; interestingly, the identified predictors of literacy skills in ASD resembled those identified as important in general reading (and dyslexia) research, which might indicate that similar kinds of support and training would be beneficial.

High-level language outcomes three and twelve months after awake surgery in low grade glioma and cavernoma patients.

OBJECTIVE: Knowledge about the long-term outcome of high-level language ability in awake surgery patients with low-grade gliomas or cavernomas in language eloquent regions is limited, particularly regarding subtle changes in high-level language abilities. PATIENTS AND METHODS: The study group consisted of 27 patients with LGG or cavernoma which involved language eloquent regions in the left hemisphere. A comprehensive assessment battery was used to target subtle changes in overall high-level language ability as well as in language sub skills. Assessments were made preoperatively and at 3 and 12 months postoperatively. RESULTS: The results showed that overall high-level language ability had not decreased significantly at group level at 3 or 12 months postoperatively. The proportion of patients with a decline of 5 percent or more at follow up 3 and 12 months were 13% and 9% respectively. There was a marked decline in semantic fluency (animals and verbs) at 3 and 12 months postoperatively. Phonemic fluency, while not significantly reduced at three months, improved markedly in the interval between 3 and 12 months. At 12 months, the only significant decline relative to preoperative scores were seen in semantic fluency for animals and verbs. Verbal cognitive speed did not decline significantly postoperatively but approximately 40% of the patients had a decline of 5% or more at 12 months. CONCLUSIONS: Overall high-level language ability was not significantly affected postoperatively at 3 and 12 months in LGG and cavernoma awake surgery patients. Semantic word fluency had deteriorated postoperatively at 3 and 12 months follow-up. Taken together our results indicate a decline in processing speed of verbal material postoperatively in the patient group.

Phonological working memory with auditory presentation of pseudo-words -- an event related fMRI Study.

Phonological working memory (PWM) tasks consist of a sequence of stimulus-encoding, maintenance and response. Few previous functional magnetic resonance imaging (fMRI) studies of PWM have employed event-related designs that make it possible to analyze the activations associated with each phase of such a task. The exploration of the cortical activation that takes place during the maintenance of PWM has been of particular interest to us. It has been suggested that temporary storage is served by the inferior parietal cortex. However, to the best of our knowledge earlier studies have used visual stimuli that might generate different cortical activations than auditory stimuli, which, presumably, would be more dependent on the auditory association cortex in the temporal lobe. This study involved an auditory stimulus presentation and a forced two-choice task. We used a parametric event-related design with fMRI. The stimuli consisted of pseudo-words of five, seven and nine syllables in length. In the control task, acoustically analogous stimuli without phonetic or linguistic content were used for passive listening. The left inferior frontal gyrus was activated during the stimulus-encoding, maintenance and response, in agreement with previous studies. However, in contrast to previous studies using visual presentation of verbal material, the inferior parietal cortex was not activated during the maintenance in the present study, but only active in the comparison and decision phase. Significant activation of the auditory cortex in the middle temporal gyrus was observed during the maintenance phase, which, together with the inferior frontal gyrus activation might underly the maintenance of phonological information.

Speech and language profiles in 4- to 6-year-old children with early diagnosis of autism spectrum disorder without intellectual disability.

PURPOSE: This study aimed to present speech and language data from a community-representative group of 4- to 6-year-old children with early-diagnosed autism spectrum disorder (ASD) without intellectual disability (ID). METHODS: The study group comprised 83 children 4-6 years of age with ASD without ID. They had been diagnosed with ASD before age 4.5 years and had received intervention at a specialized habilitation center. At 2-year follow-up, their language abilities were evaluated comprehensively by two speech-language pathologists using a battery of assessments. Receptive and expressive language and phonology were examined. The phonology evaluation included measures of phonological speech production and of phonological processing. RESULTS: Results revealed that almost 60% had moderate-severe language problems. Nearly half exhibited combined expressive and receptive language problems, of which a majority also had phonology problems. Phonological speech problems were found in 21% of the total group. CONCLUSION: The findings underscore the importance of considering speech/language disorders in children with ASD without ID, since they usually attend mainstream classes but need specific educational adaptations.

Presurgical language lateralization assessment by fMRI and dichotic listening of pediatric patients with intractable epilepsy.

OBJECTIVE: The aim of this study was to evaluate the clinical use of a method to assess hemispheric language dominance in pediatric candidates for epilepsy surgery. The method is designed for patients but has previously been evaluated with healthy children. METHODS: Nineteen patients, 8-18 years old, with intractable epilepsy and candidates for epilepsy surgery were assessed. The assessment consisted of two functional MRI protocols (fMRI) intended to target frontal and posterior language networks respectively, and a behavioral dichotic listening task (DL). Regional left/right indices for each fMRI task from the frontal, temporal and parietal lobe were calculated, and left/right indices of the DL task were calculated from responses of consonants and vowels, separately. A quantitative analysis of each patient's data set was done in two steps based on clearly specified criteria. First, fMRI data and DL data were analyzed separately to determine whether the result from each of these assessments were conclusive or not. Thereafter, the results from the individual assessments were combined to reach a final conclusion regarding hemispheric language dominance. RESULTS: For 14 of the 19 subjects (74%) a conclusion was reached about their hemispheric language dominance. Nine subjects had a left-sided and five subjects had a right-sided hemispheric dominance. In three cases (16%) DL provided critical data to reach a conclusive result. CONCLUSIONS: The success rate of conclusive language lateralization assessments in this study is comparable to reported rates on similar challenged pediatric populations. The results are promising but data from more patients than in the present study will be required to conclude on the clinical applicability of the method.

Children with autism spectrum disorders who do not develop phrase speech in the preschool years.

There is uncertainty about the proportion of children with autism spectrum disorders who do not develop phrase speech during the preschool years. The main purpose of this study was to examine this ratio in a population-based community sample of children. The cohort consisted of 165 children (141 boys, 24 girls) with autism spectrum disorders aged 4-6 years followed longitudinally over 2 years during which time they had received intervention at a specialized autism center. In this study, data collected at the 2-year follow-up were used. Three categories of expressive language were defined: nonverbal, minimally verbal, and phrase speech. Data from the Vineland Adaptive Behavior Scales-II were used to classify expressive language. A secondary objective of the study was to analyze factors that might be linked to verbal ability, namely, child age, cognitive level, autism subtype and severity of core autism symptoms, developmental regression, epilepsy or other medical conditions, and intensity of intervention. The proportion of children who met the criteria for nonverbal, minimally verbal, and phrase speech were 15%, 10%, and 75%, respectively. The single most important factor linked to expressive language was the child's cognitive level, and all children classified as being nonverbal or minimally verbal had intellectual disability.

Preschoolers with Autism Spectrum Disorder Followed for 2 Years: Those Who Gained and Those Who Lost the Most in Terms of Adaptive Functioning Outcome.

Clinical predictors of 2-year outcome in preschoolers with ASD were studied in a population-based group of very young children with ASD (n = 208). Children who gained the most (n = 30) and lost the most (n = 23), i.e., increased or decreased their adaptive functioning outcome according to the Vineland Composite Score between study entry (T1) and follow-up (T2), 2 years later were compared. Individual factors that differed significantly between the two outcome groups were cognitive level, age at referral, not passing expected milestones at 18 months, autistic type behavior problems and regression. However, logistic regression analysis showed that only cognitive level at T1 (dichotomized into IQ < 70 and IQ ≥ 70) made a unique statistically significant contribution to outcome prediction (p = <.001) with an odds ratio of 18.01. The findings have significant clinical implications in terms of information at diagnosis regarding clinical prognosis in ASD.

Temporal resolution of auditory perception and verbal working memory in 15 children with language impairment.

We investigated temporal resolution of auditory perception (TRAP), verbal working memory, and speech perception in 15 children with language impairment (LI) in comparison with a control group of 99 typical children. A computerized two-choice test was used to assess these three abilities. No deficits in TRAP were found in the LI group, and the thresholds were similar for both study groups. It was interesting that the variability was high for both groups and that the control group's performance was poorer than reported in some previously published studies. There were significant differences in the two groups' performance on speech perception and verbal working memory. Working memory was the most sensitive of these two measures.

Language lateralization in children aged 10 to 11 years: a combined fMRI and dichotic listening study.

OBJECTIVE: The aims of this study were to develop and assess a method to map language networks in children with two auditory fMRI protocols in combination with a dichotic listening task (DL). The method is intended for pediatric patients prior to epilepsy surgery. To evaluate the potential clinical usefulness of the method we first wanted to assess data from a group of healthy children. METHODS: In a first step language test materials were developed, intended for subsequent implementation in fMRI protocols. An evaluation of this material was done in 30 children with typical development, 10 from the 1(st), 4(th) and the 7(th) grade, respectively. The language test material was then adapted and implemented in two fMRI protocols intended to target frontal and posterior language networks. In a second step language lateralization was assessed in 17 typical 10-11 year olds with fMRI and DL. To reach a conclusion about language lateralization, firstly, quantitative analyses of the index data from the two fMRI tasks and the index data from the DL task were done separately. In a second step a set of criteria were applied to these results to reach a conclusion about language lateralization. The steps of these analyses are described in detail. RESULTS: The behavioral assessment of the language test material showed that it was well suited for typical children. The results of the language lateralization assessments, based on fMRI data and DL data, showed that for 15 of the 17 subjects (88%) a conclusion could be reached about hemispheric language dominance. In 2 cases (12%) DL provided critical data. CONCLUSIONS: The employment of DL combined with language mapping using fMRI for assessing hemispheric language dominance is novel and it was deemed valuable since it provided additional information compared to the results gained from each method individually.

Language and communication skills in preschool children with autism spectrum disorders: contribution of cognition, severity of autism symptoms, and adaptive functioning to the variability.

This study examined the contribution of cognitive function, severity of autism, and adaptive functioning to the variability in language and communication skills in 129 preschool children (aged 24-63 months) with autism spectrum disorder (ASD). Participants were selected from a representative research cohort of 208 preschool children on the basis of caregiver completion of the MacArthur-Bates Communicative Development Inventories (CDI). The children were classified into three cognitive groups: (a) Normal intelligence; (b) Developmental delay; and (c) Intellectual disability. Autism symptom severity was measured by the Autistic Behavior Checklist (ABC), and adaptive functioning by the Daily Living Skills (DLS) and Socialization (Soc) subscales from the Vineland Adaptive Behavior Scales. For each of five CDI variables (Phrases understood, Words understood, Words produced, Gestures and actions, and Language use), the contribution of cognition, severity of autism symptoms, and adaptive functioning to the variability was examined. Cognition and age explained about half or more of the variance in the four verbal language CDI variables, but only about one fourth of the variance in the non-verbal communication variable Gestures and actions. Severity of autism symptoms and the two adaptive measures (DLS and Soc) each only accounted for a few percent more of the variance in the four CDI language variables; however, for Gestures and actions, an additional 11-21% of the variance was accounted for. In conclusion, for children with ASD, receptive and expressive language is mainly related to cognitive level, whereas non-verbal communication skills seem to also be related to severity of autism symptoms and adaptive functioning.

Teenage outcomes after speech and language impairment at preschool age.

AIM: Ten years ago, we published developmental data on a representative group of children (n = 25) with moderate or severe speech and language impairment, who were attending special preschools for children. The aim of this study was to perform a follow-up of these children as teenagers. METHODS: Parents of 23 teenagers participated in a clinical interview that requested information on the child's current academic achievement, type of school, previous clinical assessments, and developmental diagnoses. Fifteen children participated in a speech and language evaluation, and 13 participated in a psychological evaluation. RESULTS: Seven of the 23 teenagers had a mild intellectual disability, and another three had borderline intellectual functioning. Nine had symptoms of disorders on the autism spectrum; five of these had an autism spectrum disorder, and four had clear autistic traits. Six met criteria for attention-deficit hyperactivity disorder (ADHD)/subthreshold ADHD. Thirteen of 15 teenagers had a moderate or severe language impairment, and 13 of 15 had a moderate or severe reading impairment. Overlapping disorders were frequent. None of the individuals who underwent the clinical evaluation were free from developmental problems. CONCLUSION: A large number of children with speech and language impairment at preschool age had persistent language problems and/or met the criteria for developmental diagnoses other than speech and language impairment at their follow-up as teenagers. Language impairment in young children is a marker for several developmental disorders, particularly intellectual disability and autism spectrum disorder.

Early intervention in 208 Swedish preschoolers with autism spectrum disorder. A prospective naturalistic study.

Early intervention has been reported to improve outcome in children with autism spectrum disorders (ASDs). Several studies in the field have been randomized controlled trials (RCTs). The aim of this study was to assess ASD outcome in a large naturalistic study. Two hundred and eight children, aged 20-54 months, with a clinical diagnosis of ASD were given intervention and monitored prospectively in a naturalistic fashion over a period of 2 years. The toddlers were considered representative of all but the most severely multiple disabled preschool children with ASD in Stockholm county. They fell into three cognitive subgroups: one with learning disability, one with developmental delay, and one with normal intellectual functioning. Data on intervention type and intensity were gathered prospectively in a systematic fashion. Intervention was classified into intensive applied behaviour analysis (ABA) and non-intensive, targeted interventions, also based on ABA principles. Children were comprehensively assessed by a research team before the onset of intervention, and then, again, 2 years later. Change in Vineland adaptive behaviour scales composite scores from intake (T1) to leaving the study (T2) was set as the primary outcome variable. The research team remained blind to the type and intensity of interventions provided. One hundred and ninety-eight (95%) of the original samples stayed in the study throughout the whole 2-year period and 192 children had a complete Vineland composite score results both at T1 and T2. Vineland composite scores increased over the 2-year period. This increase was accounted for by the subgroup with normal cognitive functioning. There was no significant difference between the intensive and non-intensive groups. Individual variation was considerable, but no child in the study was "problem-free" at follow-up. Our data do not support that children with ASD generally benefit more from the most intensive ABA intervention programs than from less intensive interventions or targeted interventions based on ABA.