Autism Spectrum Disorder in Children with an Early History of Paediatric Acquired Brain Injury.

Autism spectrum disorder (ASD) is a neurodevelopmental condition that arises from a combination of both genetic and environmental risk factors. There is a lack of research investigating whether early acquired brain injury (ABI) may be a risk factor for ASD. The current study comprehensively reviewed all hospital records at The Brain Injury Service, Kids Rehab at the Children's Hospital at Westmead (Australia) from January 2000 to January 2020. Of the approximately 528 cases, 14 children with paediatric ABI were subsequently given an ASD diagnosis (2.7%). For this ASD sample, the mean age at the time of the ABI was 1.55 years, indicating a high prevalence of early ABI in this diagnostic group. The mean age of ASD diagnosis was, on average, 5 years later than the average ASD diagnosis in the general population. Furthermore, 100% of children had at least one medical comorbidity and 73% had three or more co-occurring DSM-5 diagnoses. Although based on a small data set, results highlight early paediatric ABI as a potential risk factor for ASD and the potential for a delayed ASD diagnosis following early ABI, with comorbidities possibly masking symptoms. This study was limited by its exploratory case series design and small sample size. Nonetheless, this study highlights the need for longitudinal investigation into the efficacy of early screening for ASD symptomatology in children who have sustained an early ABI to maximise potential intervention.

Neuropsychological outcomes of childhood acute necrotizing encephalopathy.

Acute necrotizing encephalopathy (ANE) is a rare form of acute encephalopathy, predominantly occurring in childhood, which has a typical radiological phenotype including bilateral, symmetrical, diffusion-restricted lesions of the thalami; posterior putamen; cerebellum; and brainstem. To date, no study has systematically examined the long-term cognitive and psychological impact of ANE. The current study describes the neuropsychological outcomes of three paediatric cases of ANE, ranging from 18 months to 10 years post ANE. All three cases displayed inattention, fine motor difficulties and anxiety. Social difficulties were also reported in all cases. The severity of long-term impairment was associated with acute presentation, as well as convalescent neuroimaging. These findings highlight the need for detailed neuropsychological assessment and long-term rehabilitation.

Preliminary normative data on the BORB for children aged 3-8.

The Birmingham Object Recognition Battery (BORB) is a theoretically based test battery that is used in adult cognitive neuropsychology in research and for clinical assessment. It allows a detailed analysis of underlying impairments in individuals with brain injury who have visual object recognition difficulties. The BORB's usefulness in pediatrics is supported by numerous research studies. However, there is no published normative data for children, making clinical use of the test difficult. The aim of this brief report is to publish some preliminary normative data in 70 children aged between 3 and 8 years to assist both researchers and clinicians with interpretation of test scores. Results indicate that children's performance on individual BORB subtests varies according to task demands and age. For some subtests there is improvement in performance with increasing age. However, very young children (age 3-4 years) perform at adult levels on some subtests, or alternatively on other subtests they perform at the level of chance. The current paper supports the need for pediatric data for the BORB due to large normal individual variation in performance and varying age-related performance on individual BORB subtests.

Cognitive outcomes of pediatric stroke.

A limited number of longitudinal studies have investigated long-term neuropsychological development in the pediatric stroke population. This study retrospectively examines cognitive outcomes in 41 children with a history of stroke, with reference to age at stroke, laterality, region and mechanism of stroke. In the course of recovery, neuropsychological measures of intellectual functioning and memory were administered at two time points, whilst executive functioning, attention and academic skills were administered at one time point. As predicted, children with stroke performed significantly worse compared to normative expectations on all neuropsychological measures. Up to two thirds of children scored in the borderline impaired and impaired ranges on at least one domain of cognition. Performance on intellectual and memory assessment remained relatively stable over time. Younger age at stroke was found to be associated with poorer intellectual functioning. No effects of laterality of stroke on neuropsychological performance over time were found. Children with subcortical stroke demonstrated a greater improvement in immediate memory over time than children with cortical stroke. These findings reveal that children with stroke display long-term cognitive difficulties that typically remain stable over time. Attention and academic skills are particularly vulnerable to impairment. Further evidence that age at stroke is a significant factor in terms of cognitive outcome is provided, in support of the "early vulnerability" position.

Long-Term Neuropsychological Outcomes of Childhood Onset Acute Disseminated Encephalomyelitis (ADEM): a Meta-Analysis.

The long-term neurocognitive prognosis of childhood onset acute disseminated encephalomyelitis (ADEM) is unclear. This review and quantitative synthesis of the available literature examined whether there are long-term impacts of childhood ADEM on neurocognitive functioning. A search of online databases (MEDLINE, EMBASE, EBSCO CINAHL, PsycINFO and the Cochrane Database of Systematic Reviews) from their inception to October 2015 and reference lists identified 13 papers eligible for inclusion in the systematic review; seven of these were eligible for inclusion in meta-analyses. The systematic review indicated that, at a group level there is a positive long-term neuropsychological outcome from childhood onset ADEM. However, despite the apparent absence of long-term negative impacts of ADEM at a group level, at an individual level impairments in the areas of IQ, attention, executive functioning, processing speed, learning and memory, visuospatial skills and internalising symptoms were found in up to 43% of patients when aggregated across the studies. No significant negative effect of ADEM for any of the neuropsychological domains examined was found in meta-analyses. However, the effects for Processing Speed (r mean = -0.296 (CI 95% = -0.605-0.013)) and Internalising symptoms (r mean = 0.242 (CI 95% = -0.014-0.564)) approached significance (p = 0.06), suggesting a trend towards ADEM leading to long-term reduced processing speed and elevated internalising symptoms. Together, our findings suggest that despite a generally positive neurocognitive outcome post childhood ADEM there are a subset of individuals who can suffer from ongoing specific cognitive impairments. Clinical implications and research priorities are discussed.

The neuropsychological profile of children with basal ganglia encephalitis: a case series.

Inflammatory basal ganglia encephalitis (BGE) is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Unlike most brain injuries, BGE is a radiologically pure basal ganglia syndrome. The current study systematically describes the neuropsychological outcomes of four paediatric cases of BGE, and thus the neuropsychological outcomes of focal basal ganglia insult in childhood. Although all patients made significant motor recoveries, all four cases displayed executive dysfunction, fine motor difficulties, and anxiety. Three out of four cases displayed attention deficits. The case who received intravenous immunoglobulin (IVIg) treatment and steroids during the acute phase of the disease had the best cognitive outcome. These findings highlight the need for detailed neuropsychological assessment and long-term follow-up.

Cognitive remediation of attention deficits following acquired brain injury: A systematic review and meta-analysis.

BACKGROUND: Attention deficits are common after acquired brain injury (ABI) and adversely impact academic, vocational and social outcomes. The role of cognitive interventions in post-ABI attention rehabilitation remains unclear. OBJECTIVE: To evaluate effectiveness of cognitive interventions in treating attention deficits following ABI and to explore differences in treatment effect between ABI etiologies. METHODS: MEDLINE, EMBASE, PsycINFO and CENTRAL databases were searched for randomized controlled trials (RCTs). Studies were selected by three reviewers. Study quality was assessed using Cochrane Collaboration tool for RCTs. Effect sizes (Hedge's g) for each attentional domain were meta-analyzed with subgroup analysis by ABI etiology. RESULTS: Twelve RCTs with 584 participants were included, representing individuals with stroke, traumatic brain injury (TBI) and CNS-impacting malignancy. Cognitive rehabilitation improved divided attention in stroke survivors (g 0.67; 95% confidence interval, 0.35-0.98; p <  0.0001) but not other ABI populations. Sustained, selective and alternating attention, and inhibition were not significantly improved in any ABI population. Follow-up data showed no evidence of long-term benefit. CONCLUSION: Cognitive rehabilitation resulted in short-term improvements in divided attention following stroke, but not after TBI or CNS-impacting malignancy. Cognitive interventions did not significantly improve other attentional domains in participants with stroke, TBI or CNS-impacting malignancy.

Training of familiar face recognition and visual scan paths for faces in a child with congenital prosopagnosia.

In the current report we describe a successful training study aimed at improving recognition of a set of familiar face photographs in K., a 4-year-old girl with congenital prosopagnosia (CP). A detailed assessment of K.'s face-processing skills showed a deficit in structural encoding, most pronounced in the processing of facial features within the face. In addition, eye movement recordings revealed that K.'s scan paths for faces were characterized by a large percentage of fixations directed to areas outside the internal core features (i.e., eyes, nose, and mouth), in particular by poor attendance to the eye region. Following multiple baseline assessments, training focused on teaching K. to reliably recognize a set of familiar face photographs by directing visual attention to specific characteristics of the internal features of each face. The training significantly improved K.'s ability to recognize the target faces, with her performance being flawless immediately after training as well as at a follow-up assessment 1 month later. In addition, eye movement recordings following training showed a significant change in K.'s scan paths, with a significant increase in the percentage of fixations directed to the internal features, particularly the eye region. Encouragingly, not only was the change in scan paths observed for the set of familiar trained faces, but it generalized to a set of faces that was not presented during training. In addition to documenting significant training effects, our study raises the intriguing question of whether abnormal scan paths for faces may be a common factor underlying face recognition impairments in childhood CP, an issue that has not been explored so far.

Predicting generalization in the training of irregular-word spelling: treating lexical spelling deficits in a child.

This paper presents a single case study investigating the mechanisms underlying generalization of treatment benefits to untrained words in spelling. Brunsdon, Coltheart, and Nickels (2005) observed that untreated words that improved tended to be those whose errors were closest to being correct prior to treatment. These words also tended to be high in written frequency. The present study employed the same treatment techniques as those used by Brunsdon et al. with K.M., a developmental surface dysgraphic. During a first treatment the characteristics of words whose spelling improved without specific training were identified. These characteristics were then used in a second treatment to test whether it was possible to predict generalization. The results showed that treatment generalization to untreated irregular words was best predicted by neighbourhood size and frequency. We suggest that the processes underlying treatment generalization are based on the interaction between the orthographic lexicon and the graphemic buffer. Clinical implications are discussed.

Topographical disorientation: towards an integrated framework for assessment.

Topographical disorientation, the inability to find one's way in large-scale environments, is a relatively common disorder. However, there are relatively few cognitive neuropsychological studies that investigate the nature of topographical cognition. Theoretical progress has been hindered by a number of factors including: terminological confusion; lack of theoretically driven assessment; the use of broad classifications for the nature of underlying impairments; and an ongoing failure to examine topographical skills in real-life settings. As a result, there is currently no well-established or widely accepted theoretical framework encompassing all aspects of this multifaceted area of cognition. In addition, there is a relative paucity of published case studies that include a comprehensive, theoretically based assessment of topographical disorientation, and treatment of the disorder has received virtually no formal investigation (with the exception of Davis & Coltheart, 1999). Thus, the current paper focuses on the development of a broad framework for understanding topographical cognition that integrates a number of recent theories of topographical orientation and mental imagery (Farah, 1984; Kosslyn, 1980; Riddoch & Humphreys, 1989). The aim of the paper is to present a preliminary framework that can be used as a basis for further refinement and development of theoretical proposals, and be employed by clinicians as a starting point for assessment planning.

Assessment and treatment of childhood topographical disorientation: a case study.

Topographical disorientation refers to individuals who are unable to find their way around large-scale environments in a normal manner. Childhood topographical disorientation is rarely investigated or reported. Treatment of topographical disorientation is also rare with only one reported treatment study in an adult (Davis & Coltheart, 1999) and no known description of treatment in a child. This paper reports a detailed case analysis of CA, a 6-year-old child with topographical disorientation, and a description of a treatment programme focused on training orientation in the school environment. Assessment of CA revealed mild to moderate visual agnosia in conjunction with severe impairments in general spatial learning and memory, topographical new learning and memory, and a total inability to learn new topographical routes. CA was also unable to use a mental image of his environment, a simple visual plan of his environment or a simple visual map, but was able to follow verbally mediated topographical instructions. The treatment programme focused on improving CA's topographical orientation in the school environment. The programme first involved training in recognition of major school buildings and landmarks and then focused on practical training in route finding along commonly used routes in the school environment. Clear benefits from treatment were evident. The assessment and treatment methods employed provide practical and useful ideas for management of this condition in other children.

Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome.

RATIONALE: The long-term outcome of idiopathic macrocephaly is presently unknown. METHODS AND RESULTS: In the current study (n=15), MRI conducted at long-term review showed regression of orbito-frontal extradural collections and normal or slightly enlarged ventricular space compared to infant examination. Head circumference had normalised in all but one participant. Neuropsychological assessments of nine participants showed general intellectual ability within the normal range in the majority of participants; however, specific deficits in attention were evident. Clinical interviews conducted with a smaller sub-group revealed anecdotal histories of behavioural difficulties and reading or arithmetic difficulties in half of the total sample. CONCLUSIONS: Prospective review studies such as this indicate that abnormal radiological findings in infancy are not necessarily predictive of neurodevelopmental problems and may reflect a normal variant. However, while overall intellectual ability may be within average limits in this diagnostic sample, considerable individual variations remain in specific areas of neuropsychological function.

Severe developmental letter-processing impairment: A treatment case study.

We report the case of ET, a 7-year-old child with a severe developmental letter-processing impairment. Detailed assessment revealed multiple impairments of the letter-processing system affecting abstract visual letter representation, semantics, and connections between the phonological processing modules and the orthographic buffer. Treatment methods focused on letter sounding through development of abstract visual representation and semantic representation of letters. Treatment resulted in dramatic and enduring improvements in cross-case matching, letter/number categorization, and letter sounding. Cognitive neuropsychological reading research has largely focused on word processing, with a relative neglect of single-letter processing. The current study investigated letter processing in greater detail than has been usual and outlines a broad theoretical framework for letter processing. ET's assessment and treatment data are used to support and question predictions from the framework. A number of theoretical implications are discussed with reference to ET's data and that of other reported cases of impaired single-letter processing. Finally, the paucity of investigation of letter processing in children is highlighted, particularly with regard to integrity of abstract letter representation in developmental dyslexia.

Developmental prosopagnosia: A case analysis and treatment study.

This paper reports a treatment case study focused on face perception impairments designed for AL, an 8-year-old child with prosopagnosia. AL's prosopagnosia was characterized by deficits at the level of structural encoding-that is, he was unable to achieve normal basic perception of faces. This impairment then impacted on all subsequent aspects of familiar- and unfamiliar-face processing. Detailed assessment of feature processing revealed impairments in perception of facial features with a dissociation between relatively good perception of the mouth feature and poor perception of eye and nose features. Interestingly, results also suggested at least partial internal representation of facial features despite long-standing deficits in perception of these features. A treatment programme focused on training in perception, and analysis of facial features and familiar-face naming was conducted. Treatment resulted in excellent face naming for familiar faces, a decreased reliance on nonfacial cues and a reduction in AL's tendency to misidentify unfamiliar faces as family members.

Treatment of irregular word spelling in developmental surface dysgraphia.

An increasing number of cognitive neuropsychological treatment studies of acquired dysgraphia have been published in recent years, but to our knowledge there are no corresponding studies of developmental dysgraphia. This paper reports a cognitive neuropsychological treatment programme designed for a child with developmental surface dysgraphia. The treatment aim was to improve functioning of the orthographic output lexicon, and so treatment methods targeted irregular word spelling. Treatment methods were based on previous successful treatments employed in cases of adult acquired surface dysgraphia (Behrmann, 1987; De Partz, Seron, & Van der Linden, 1992; Weekes & Coltheart, 1996). Results showed a significant treatment effect for both spelling and reading of irregular words that was largely stable over time and that generalised partially to spelling of untreated irregular words. Homophone words were not treated but some aspects of homophone reading and spelling also improved, though homophone confusion errors remained. Comparison of treatment effectiveness with and without mnemonics suggested that the mnemonic cue itself was not necessary to achieve treatment success for irregular word spelling. Analyses revealed that untreated irregular words whose spellings became correct as a result of treatment generalisation were those whose original misspellings were closest to being correct prior to treatment. Results also provided preliminary evidence that the mechanism underlying treatment generalisation involved improved access to orthographic representations, resulting in an increased tendency to employ orthography for spelling attempts and reduced reliance on phoneme to grapheme conversion.

Visual agnosia and prosopagnosia in childhood: a prospective case study.

Selective impairments in visual processing are well documented in adults but rarely reported in children. The few childhood cases reported are mostly retrospective accounts with little attention paid to developmental, assessment or management issues. We report a prospective case study of a boy with prosopagnosia and visual processing deficits of presumed developmental origin. At the age of 4 years, AL presented with a range of cognitive and visual recognition deficits. Subsequent assessments revealed an evolving pattern in visual recognition and dissociations between developing skills. At the age of 7 AL has impairments in early perceptual analysis, visual organisation and in complex visual processing. Although he can identify facial features and match faces he is unable to recognise familiar faces. His reading and spelling are developing normally. The nature of his deficits and his progress are discussed within a cognitive neuropsychological framework.