Receptive Language Deficits Research Articles

Abnormal cortical activation during silent reading in adolescents with autism spectrum disorder

ObjectiveAutism spectrum disorder (ASD) is a developmental disorder characterized by communication deficits and social difficulties, and individuals with ASD frequently exhibit varied levels of language abilities. However, the neurophysiological mechanisms underlying their language deficits remain unclear. To gain insight into the neurophysiological mechanisms of receptive language deficits, we assessed cortical activation patterns in adolescents with ASD during silent word-reading. MethodsWe used magnetoencephalography to measure cortical activation during a silent word-reading task in 14 adolescent boys with high-functioning ASD and 17 adolescent boys with typical development (TD). ResultsCompared with participants with TD, those with ASD exhibited significantly decreased cortical activation in the left middle temporal gyrus, left temporoparietal junction, bilateral superior temporal gyrus, left posterior insula, and right occipitotemporal gyrus, and increased activation in the right anterior insula. Participants with ASD also exhibited a lack of left-lateralization in the central sulcus and abnormal right-lateralization in the anterior insula area. Furthermore, in participants with ASD, we found that abnormal activation of the right central sulcus correlated significantly with lower visual word comprehension scores, and that decreased activation of the right anterior insula correlated significantly with the severity of social interaction difficulties. ConclusionOur findings suggest that atypical cortical activation and lateralization in the temporal-frontal area, which is associated with higher-order language processing functions, such as semantic analysis, may play a crucial role in visual word comprehension and social interaction difficulties in adolescents with ASD.

Cognitive and behavioral profile of Williams Syndrome toddlers.

To verify indicators of cognitive development, receptive language skills and adaptive behavioral patterns in toddlers with Williams syndrome (WS). The sample comprised 8 children of both sex, aged between 48 and 72 months with WS. Instruments of data collection were Denver Developmental Screening Test II; Peabody Picture Vocabulary Test; Vineland Adaptive Behavior Scale; Child Behavior Checklist for Ages 1½-5and 6 to 18; Columbia Mental Maturity Scale (CMMS), and Behavior Problems Inventory-01. The major developmental impairments were associated with fine motor skills and personal care abilities. Deficits in receptive language and communication skills were reported according to the PPVT and Denver II, respectively. The caregivers reported behavioral and emotional problems associated to anxiety and depression, and attention problems scales of CBCL. The toddlers demonstrated deficits in adaptive functioning and behavioral, motor and cognitive difficulties such as inattention and hyperactivity, stereotypies and aggressive behavior.

Language development in the pediatric cochlear implant patient

ObjectiveTo access the long‐term outcomes of children implanted during most sensitive period for language development.Study designLiterature review.MethodAn initial PubMed search was carried out using the search terms language development and cochlear implant resulted in 1149 citations. A second search was carried out on the initial citations using the criterion of implantation in the period of birth to 24 months, which identified 386 articles. These were analyzed to determine those studies in which linguistic outcome was measured at least three or more years following implantation.ResultsTwenty‐one reports published from 2004 to 2017 that met the criteria. The range of follow‐up was from 3 years to an excess of 10 years. Four => 10‐year follow‐up reports were consistent in showing that the earlier the subject is implanted the better the outcome. Many, but not all, of these children did obtain age‐appropriate language. There were 17 reports with follow‐up from 3 to less than 10 years. In 7 of the 11 studies, the children's expressive language was reported to have reached an age level of less than 80%. The results for receptive language showed that 4 of the 11 studies found that the children achieved a receptive language age level of less than 80%. There were 8 studies which documented the effect of implantation before 12 months of age and between 12 and 24 months of age and they all found that the earlier the implantation, the better the outcome for language.ConclusionThe cochlear implant is efficacious in the amelioration of receptive and expressive language deficits in most congenitally deafened children implanted before the age of one. The language outcomes for those implanted after the age of one decline as the age of implantation increases.Level of EvidenceN/A.

Interictal epileptiform discharges have an independent association with cognitive impairment in children with lesional epilepsy.

The relative contribution of interictal epileptiform discharges (IEDs) to cognitive dysfunction in comparison with the underlying brain pathology is not yet understood in children with lesional focal epilepsy. The current study investigated the association of IEDs with intellectual functioning in 103 children with medication-resistant focal epilepsy. Hierarchical multiple regression analyses were used to determine the independent contribution of IED features on intellectual functioning, after controlling for effects of lesional pathology, epilepsy duration, and medication. Exploratory analyses were conducted for language and memory scores as well as academic skills available in a subset of participants. The results reveal that IEDs have a negative association with IQ with independent, additive effects documented for frequent and bilaterally distributed IEDs as well as discharge enhancement in sleep. Left-lateralized IEDs had a prominent effect on verbal intelligence, in excess of the influence of left-sided brain pathology. These effects extended to other cognitive functions, most prominently for sleep-enhanced IEDs to be associated with deficits in expressive and receptive language, reading, spelling and numerical skills. Overall, IED effects on cognition were of a magnitude similar to lesional influences or drug effects (topiramate use). This study demonstrates an association between IEDs and cognitive dysfunction, independent of the underlying focal brain pathology.

Characterising receptive language processing in schizophrenia using word and sentence tasks

ABSTRACTIntroduction. Language dysfunction is proposed to relate to the speech disturbances in schizophrenia, which are more commonly referred to as formal thought disorder (FTD). Presently, language production deficits in schizophrenia are better characterised than language comprehension difficulties. This study thus aimed to examine three aspects of language comprehension in schizophrenia: (1) the role of lexical processing, (2) meaning attribution for words and sentences, and (3) the relationship between comprehension and production.Methods. Fifty-seven schizophrenia/schizoaffective disorder patients and 48 healthy controls completed a clinical assessment and three language tasks assessing word recognition, synonym identification, and sentence comprehension. Poorer patient performance was expected on the latter two tasks.Results. Recognition of word form was not impaired in schizophrenia, indicating intact lexical processing. Whereas single-word synonym identification was not significantly impaired, there was a tendency to attribute word meanings based on phonological similarity with increasing FTD severity. Importantly, there was a significant sentence comprehension deficit for processing deep structure, which correlated with FTD severity.Conclusions. These findings established a receptive language deficit in schizophrenia at the syntactic level. There was also evidence for a relationship between some aspects of language comprehension and speech production/FTD. Apart from indicating language as another mechanism in FTD aetiology, the data also suggest that remediating language comprehension problems may be an avenue to pursue in alleviating FTD symptomatology.

Syntactic processing as a marker for cognitive impairment in amyotrophic lateral sclerosis

Despite recent interest in cognitive changes in patients with amyotrophic lateral sclerosis (ALS), investigations of language function looking at the level of word, sentence and discourse processing are relatively scarce. Data were obtained from 26 patients with sporadic ALS and 26 healthy controls matched for age, education, gender, anxiety, depression and executive function performance. Standardized language tasks included confrontation naming, semantic access, and syntactic comprehension. Quantitative production analysis (QPA) was used to analyse connected speech samples of the Cookie Theft picture description task. Results showed that the ALS patients were impaired on standardized measures of grammatical comprehension and action/verb semantics. At the level of discourse, ALS patients were impaired on measures of syntactic complexity and fluency; however, the latter could be better explained by disease related factors. Discriminant analysis revealed that syntactic measures differentiated ALS patients from controls. In conclusion, patients with ALS exhibit deficits in receptive and expressive language on tasks of comprehension and connected speech production, respectively. Our findings suggest that syntactic processing deficits seem to be the predominant feature of language impairment in ALS and that these deficits can be detected by relatively simple language tests.

Early-onset absence epilepsy associated with GLUT 1 deficiency

We describe a 4.5-year-old girl with an unremarkable personal and family history. At 16 mo of age, she had a single generalized tonic-clonic seizure while awake. At 3 yr of age, she started with daily absence seizures while awake and hyperki- netic and aggressive behavior. The interictal electroencephalography showed bilateral temporo-occipital spike and wave com- plexes. The ictal video-electroencephalography recordings showed symmetric or asymmetric and synchronized spike and wave paroxysms associated with absences. Neurological examination revealed motor clumsiness, ataxia, and receptive and expressive language deficits. Routine laboratory and imaging studies as well as blood and urine neuro-metabolic investigations were normal. The absences were refractory to antiepileptic drugs. Cerebrospinal fluid glucose concentration was 36 mg/dL, lac- tate concentration was 1 mmol/L, and cerebrospinal fluid/plasma glucose ratio was 0.49. Based on the electro-clinical manifes- tations, neurological examination, and the metabolic study results, glucose transporter-1 deficiency was diagnosed and the girl was started on the ketogenic diet with good response.

Language Impairment in Adolescents With Sydenham Chorea

BackgroundNeuropsychiatric comorbidities are frequent in Sydenham chorea. However, cognitive impairment in Sydenham chorea has not been sufficiently described. The objective of this study was to evaluate expressive and receptive language deficits in adolescents with Sydenham chorea. MethodsTwenty patients with Sydenham chorea were compared with 20 patients with rheumatic fever without chorea and 20 healthy controls. Participants were matched for age and gender. Participants were assessed with verbal fluency tasks (phonemic and semantic) and with verbal comprehension tasks (Token Test). Patients with Sydenham chorea were also assessed with the Universidade Federal de Minas Gerais Sydenham Chorea Rating Scale. ResultsPerformance in verbal fluency and in verbal comprehension tasks differed significantly (P < 0.01) among the three groups. Patients with Sydenham chorea performed significantly worse than healthy control group in phonemic and semantic verbal fluency tasks as well as in the Token Test. The group with rheumatic fever also performed worse than healthy controls in phonemic verbal fluency. Severity of motor signs in Sydenham chorea inversely correlated with performance in phonemic verbal fluency (words beginning with letter S, and total sum of words beginning with letters F, A, and S). ConclusionsAdolescents with Sydenham chorea show difficulties in verbal fluency and in verbal comprehension. Patients with rheumatic fever also have some degree of language impairment. Future studies must investigate language impairment in difference stages of Sydenham chorea (acute, persistent, and remission) and putative biological markers.

Pairing Speech Sounds With Vagus Nerve Stimulation Drives Stimulus-specific Cortical Plasticity.

Individuals with communication disorders, such as aphasia, exhibit weak auditory cortex responses to speech sounds and language impairments. Previous studies have demonstrated that pairing vagus nerve stimulation (VNS) with tones or tone trains can enhance both the spectral and temporal processing of sounds in auditory cortex, and can be used to reverse pathological primary auditory cortex (A1) plasticity in a rodent model of chronic tinnitus. We predicted that pairing VNS with speech sounds would strengthen the A1 response to the paired speech sounds. The speech sounds 'rad' and 'lad' were paired with VNS three hundred times per day for twenty days. A1 responses to both paired and novel speech sounds were recorded 24 h after the last VNS pairing session in anesthetized rats. Response strength, latency and neurometric decoding were compared between VNS speech paired and control rats. Our results show that VNS paired with speech sounds strengthened the auditory cortex response to the paired sounds, but did not strengthen the amplitude of the response to novel speech sounds. Responses to the paired sounds were faster and less variable in VNS speech paired rats compared to control rats. Neural plasticity that was specific to the frequency, intensity, and temporal characteristics of the paired speech sounds resulted in enhanced neural detection. VNS speech sound pairing provides a novel method to enhance speech sound processing in the central auditory system. Delivery of VNS during speech therapy could improve outcomes in individuals with receptive language deficits.

Speech and Language Issues in Children with Prader-Willi Syndrome

Background: Prader-Willi syndrome (PWS) is a genetic disorder caused by the lack of the paternal contribution of Chromosome 15q11.2-q13.2 region. It is associated with global developmental delays, including speech and language delay. There is no information regarding the prevalence of apraxia of speech in this syndrome, despite the fact that it is often recognized clinically. In this study, we sought to investigate the prevalence of apraxia in children with PWS and speech and language delay. Methods: Thirty children with genetically confirmed PWS, ages 22 months to 9 years of age, were evaluated by a certified speech-language pathologist due to physician concerns about speech and language development. Children were assessed by a variety of tests based on their age. Results: Sixteen children had receptive language deficits and 18 had expressive language deficits. Fourteen of the thirty children (47%) had results on evaluation that were consistent with apraxia, of which 57% were male, and 71% (p&lt;0.001) had deletion-type PWS. Conclusion: As expected, children with PWS who are referred for concerns about speech and language development are commonly found to have receptive and expressive language deficits. However, there was a high prevalence of apraxia in our patients, which has not previously been reported in this population. We recommend that children with PWS be evaluated for apraxia by a speech-language pathologist once their expressive language skills are developed enough for speech assessment. The diagnosis of apraxia will necessitate specific speech therapy techniques which may not otherwise be used for individuals with this syndrome, thus resulting in more severe and prolonged speech delays.

B-41 * Cognitive Impairments and Circumscribed Strength Areas in a Child with Prolonged Status Epilepticus and Developmental Delays

Objective: Anoxic brain injury has been implicated in severe intellectual, attention, memory, and behavioral impairments. The presented case delineates circumscribed areas of strength within the cognitive profile of an individual presenting with severe global deficits resulting from anoxia/ hypoxia in early childhood. Method: The client was a 9-year-11 month-old English dominant Latino male affected by developmental delays and a significant history of multiple episodes of tonic-clonic status epilepticus (40−75 minutes). Presenting problems included receptive and expressive language deficits, failure to develop reading or writing skills, attentional difficulties across domains, limited self-care abilities, tantrums, memory impairment, and frequent violation of social boundaries. Comprehensive and culturally sensitive neuropsychological instruments were used to obtain information regarding the client's current functioning. Data from neuroimaging studies conducted at 3 and 9 years of age is discussed. Although an MRI at 3 years of age revealed no structural abnormalities, an EEG conducted several months prior to the presented assessment revealed epileptiform discharges at the left temporal head region. Results: Neuropsychological test results revealed severely impaired performance across intellectual, verbal, memory, academic, visuomotor, and socio-emotional domains. Circumscribed strengths in associative learning were used as the basis for behavioral interventions with this child's impaired adaptive functioning. Additional areas of strength emphasized in intervention design included low level receptive language abilities, visual perception and recognition skill, and basic motor functioning. Conclusion(s): The described case illustrates the value of defining and subsequently capitalizing on specific strength areas in an otherwise depressed cognitive profile.

Sophisticated—Yet Simple—Apps for Grown-ups

Sophisticated—Yet Simple—Apps for Grown-ups

Habilidades de leitura em crianças com diagnóstico de hiperlexia: relato de caso

Hyperlexia is characterized by spontaneous and early acquisition of reading skills, manifested before the age of five, without any formal education. Expressive and receptive language deficit, excellent memory, delayed language skills, echolalia, perseverations, and difficulty to understand verbal contexts, are common symptoms in individuals with hyperlexia and global developmental disorders, including Asperger's syndrome. The aim of this study was to describe the reading skills of individuals with hyperlexia. The participants were six boys with a history of hyperlexia, perceived by relatives before 36 months of life, chronologically aged between four years and four months to five years and two months. The following evaluation procedures were applied: interview with the parents or responsible guardians, communicative behavior observation, Peabody Picture Vocabulary Test (PPVT), recognition of alphabet letters and numbers, School Performance Test, text reading, and reading comprehension. The participants showed different performances in the PPVT, and recognized letters, numbers and isolated words. There was difficulty in answering the writing subtest; the majority did not understand the text that they read and all of them demonstrated recognition of letters and numbers. Some participants were able to read words and texts. However, all participants presented alterations in other areas of language development, such as receptive vocabulary, communicative behavior and understanding of the material read, in addition to altered behaviors of social adaptation, both interactive and restrictive.

546 – Phelan-mcdermid syndrome and atypical bipolar disorder

Introduction The Phelan-McDermid syndrome (PMD) is characterized by intellectual disability (ID), childhood hypotonia, severely delayed or absent speech, and autistic features without major dysmorphisms and somatic anomalies. Haploinsufficiency of the SH3 and multiple ankyrin repeat domains 3 ( SHANK3 ) gene in 22q13.3 is thought to be causative for the PMD-phenotype comprising, in addition to sleep disturbances and deficits in language and communication, several behavioural abnormalities and increased reactivity to sensory stimuli. Data about neuropsychological dysfunctions or psychiatric symptoms are not available as yet. Objectives Investigating the possible psychopathological phenotype of PMD. Aims Diagnostic evaluation of one geriatric female and two male adult patients. Methods Detailed neuropsychiatric and neuropsychological examination of 3 patients with genetically proven PMD. Results In an adult male with a history of mood and behaviour fluctuations, psychiatric examination at the age of 24 disclosed symptoms of a severe major depression with irritability, loss of initiative, and marked sleep disturbances. Severe ID and marked deficits in expressive and receptive language were established. After addition of nortriptyline to valproic acid, depressive symptoms gradually remitted. His older brother has a similar history but with less severe symptomatology. The 70- years-old institutionalized female patient, had a history of affective and behavioural imbalance over many decades. Monotherapy with carbamazepine led to acceptable stabilization. Genetic evaluation in the two brothers and the female patient demonstated an identical loss of 2.15Mb and a 610kb deletion in 22q13.3, respectively, all including SHANK3 gene. Conclusions Provisonal diagnosis of atypical bipolar disorder could be made in all three patients.

Oral motor deficits in speech-impaired children with autism

Absence of communicative speech in autism has been presumed to reflect a fundamental deficit in the use of language, but at least in a subpopulation may instead stem from motor and oral motor issues. Clinical reports of disparity between receptive vs. expressive speech/language abilities reinforce this hypothesis. Our early-intervention clinic develops skills prerequisite to learning and communication, including sitting, attending, and pointing or reference, in children below 6 years of age. In a cohort of 31 children, gross and fine motor skills and activities of daily living as well as receptive and expressive speech were assessed at intake and after 6 and 10 months of intervention. Oral motor skills were evaluated separately within the first 5 months of the child's enrolment in the intervention programme and again at 10 months of intervention. Assessment used a clinician-rated structured report, normed against samples of 360 (for motor and speech skills) and 90 (for oral motor skills) typically developing children matched for age, cultural environment and socio-economic status. In the full sample, oral and other motor skills correlated with receptive and expressive language both in terms of pre-intervention measures and in terms of learning rates during the intervention. A motor-impaired group comprising a third of the sample was discriminated by an uneven profile of skills with oral motor and expressive language deficits out of proportion to the receptive language deficit. This group learnt language more slowly, and ended intervention lagging in oral motor skills. In individuals incapable of the degree of motor sequencing and timing necessary for speech movements, receptive language may outstrip expressive speech. Our data suggest that autistic motor difficulties could range from more basic skills such as pointing to more refined skills such as articulation, and need to be assessed and addressed across this entire range in each individual.

Language processing abnormalities in adolescents with psychotic-like experiences: An event related potential study

Language impairments are a well established finding in patients with schizophrenia and in individuals at-risk for psychosis. A growing body of research has revealed shared risk factors between individuals with psychotic-like experiences (PLEs) from the general population and patients with schizophrenia. In particular, adolescents with PLEs have been shown to be at an increased risk for later psychosis. However, to date there has been little information published on electrophysiological correlates of language comprehension in this at-risk group. A 64 channel EEG recorded electrical activity while 37 (16 At-Risk; 21 Controls) participants completed the British Picture Vocabulary Scale (BPVS-II) receptive vocabulary task. The P300 component was examined as a function of language comprehension. The at-risk group were impaired behaviourally on receptive language and were characterised by a reduction in P300 amplitude relative to the control group. The results of this study reveal electrophysiological evidence for receptive language deficits in adolescents with PLEs, suggesting that the earliest neurobiological changes underlying psychosis may be apparent in the adolescent period.

Commentary on “Motor Characteristics of Young Children Referred for Possible Autism Spectrum Disorder”

“How should I apply this information?” This retrospective chart analysis of young children who have been referred to an autism-evaluation clinic reveals that a delay in motor performance (gross and fine) may be regarded as an early childhood sign in this specific group of children. However, delay in motor performance does not distinguish young children with an autism spectrum disorder (ASD) from children who otherwise develop atypically. The clinical symptoms observed in these children include social emotional, receptive language, and expressive language deficits. When we find deficits in adaptive behavior and social interaction in combination with low motor outcomes in early development, we should be aware of the possibility of an ASD. This provides professionals the opportunity to diagnosis ASD early, even before early school age and consequently the opportunity to start with early intervention programs. “What should I be mindful about in applying this information?” The authors of this study conclude that children who are referred to an ASD clinic demonstrate a delay in gross and fine motor skills at 6 and 8 months, respectively. However, according to the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III) manual, developmental ages should only be presented when the scaled scores fall outside the normal ranges, that is, less than −2 SD. From the scaled scores (Table 1), one can conclude that the mean gross and fine motor development of these children is still within normal ranges, that is, between the 0 and −1 and −1 and −2 SD. It is therefore not appropriate to express motor delay in developmental ages for these children. Not only from a methodological point of view but also from a clinical standpoint, motor performance may best be expressed in scaled scores. Otherwise children will be, when only based on the developmental age, unjustifiably diagnosed as having delayed motor development while they are functioning within the normal variance. Table 1 shows that within the patient population diagnosed with an ASD, considerable variation is measured in gross and fine motor performance, thus implying that a number of children indeed have significant motor delay and should be looked upon in a more individualized manner. At least we should evaluate all children whether or not there is a delay in motor development, based on scaled scores or on developmental age, according to the BSID-III manual. We should look at both the quality and quantity of the motor performance and children's and parents' needs, before starting pediatric physical therapy intervention. The study describes a retrospective and cross-sectional analysis of medical charts; we therefore should interpret the findings with great care. It is not clear, for example, whether children who were diagnosed with an ASD had previous motor scores that were better or possibly worse than the scores at the time of diagnosis.1 This information is of great importance for clinicians to be able to draw the right conclusions from just 1 developmental assessment. Future studies should therefore use a longitudinal design with consecutive developmental assessments. In this article, a group of children younger than 3.5 years was evaluated. The study found a trend for gross motor skills to be moderately associated with functional difficulties (eg, adaptive and social emotional limitations) in children diagnosed with ASD. From these analyses, it is not clear in what direction this association points. The (fine) motor tasks in the BSID-III are complex tasks, and it is therefore more likely that the functional difficulties in this study will have a larger effect on motor skills than the opposite, that is, that motor impairment will limit adaptive and social emotional limitations, at least in early childhood. At a later age, that is, children at school age, it is more plausible that limited participation in physical activities due to motor problems may affect the functional skills (eg, adaptive and social emotional limitations). Table 1 shows that the main problems of these children seem to be in the social emotional, receptive language, and expressive language domains. Should we not focus on these functional limitations in the intervention of these children, which may influence indirectly development of motor performance? Further research is needed to answer that question. Janjaap van der Net, PT, PhD, PCS University Children's Hospital/UMC Utrecht, Utrecht, The Netherlands Maaike C. A. Sprong, PT, MSc, PCS University Children's Hospital/UMC Utrecht, Utrecht, The NetherlandsThe authors declare no conflict of interest.

The cerebellum, cognition, and behaviour

The cerebellum, cognition, and behaviour

Awareness of deficits during intracarotid anesthetic procedures in epilepsy: Comparisons of motor, naming, and comprehension awareness under amobarbital versus under etomidate

Awareness of deficits is often impaired following disruption of the right hemisphere. Intracarotid anesthetic procedures (IAPs) represent a unique method by which we can assess functioning of each hemisphere in isolation. We used this technique to explore deficits of awareness of specific functions—motor ability, naming, and comprehension—in patients with temporal lobe epilepsy. Some patients were injected with amobarbital, whereas others were injected with etomidate. We found that injection into the right hemisphere, or epileptogenic focus in the right hemisphere following injection in the left, resulted in the lowest levels of motor awareness. We also found a higher level of awareness for expressive language deficits and less awareness for receptive language deficits. Comparison of etomidate and amobarbital suggested more awareness following injection of etomidate. We discuss how these findings contribute to our understanding of the right hemisphere's special role in awareness, and how research in other disorders and in comparative neurology has shaped our conceptualization of the neuroanatomy of insight.

The relationship between acquired impairments of executive function and behaviour change in adults with Down syndrome

The latter stages of dementia in individuals with Down syndrome are well documented; however, earlier cognitive and behavioural changes have only recently been described. Holland et al. suggested such early signs of dementia in this population are behavioural and are similar to those seen in frontotemporal dementia, but there is, as yet, no evidence to determine whether such behavioural changes are associated with a declines in specific cognitive functions, including those associated with the frontal lobes. A longitudinal design of three time points across 16 months was used across 30 adults with Down syndrome aged 30 years and over. Measures of cognition (Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities), receptive language (British Picture Vocabulary Scales), adaptive behaviour (Vineland Adaptive Behavior Scales), behavioural excesses and behavioural deficits (Assessment for Adults with Developmental Disabilities) and measures of executive functioning were completed at each time point. Using a data-driven method, cognitive deterioration was determined using the Reliable Change Index on performance on the Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities across the duration of the study. Performance on the remaining measures were then compared between those with (n = 10) and those without (n = 20) cognitive deterioration. Only individuals with cognitive deterioration showed decreases on measures of executive function and significant changes in behaviour across the duration of the study, which was not solely due to declines in memory. There were no changes between the groups on levels of adaptive behaviour. Even in the early stages of cognitive deterioration, specific behavioural changes can be identified that are not present in those without cognitive deterioration. The differing effects of cognitive deterioration on behavioural excesses and deficits are discussed in relation to potentially differing underlying neuropathological causes.