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PubMed2004

Theory-of-mind development in oral deaf children with cochlear implants or conventional hearing aids.

BACKGROUND: In the context of the established finding that theory-of-mind (ToM) growth is seriously delayed in late-signing deaf children, and some evidence of equivalent delays in those learning speech with conventional hearing aids, this study's novel contribution was to explore ToM development in deaf children with cochlear implants. Implants can substantially boost auditory acuity and rates of language growth. Despite the implant, there are often problems socialising with hearing peers and some language difficulties, lending special theoretical interest to the present comparative design. METHODS: A total of 52 children aged 4 to 12 years took a battery of false belief tests of ToM. There were 26 oral deaf children, half with implants and half with hearing aids, evenly divided between oral-only versus sign-plus-oral schools. Comparison groups of age-matched high-functioning children with autism and younger hearing children were also included. RESULTS: No significant ToM differences emerged between deaf children with implants and those with hearing aids, nor between those in oral-only versus sign-plus-oral schools. Nor did the deaf children perform any better on the ToM tasks than their age peers with autism. Hearing preschoolers scored significantly higher than all other groups. For the deaf and the autistic children, as well as the preschoolers, rate of language development and verbal maturity significantly predicted variability in ToM, over and above chronological age. CONCLUSIONS: The finding that deaf children with cochlear implants are as delayed in ToM development as children with autism and their deaf peers with hearing aids or late sign language highlights the likely significance of peer interaction and early fluent communication with peers and family, whether in sign or in speech, in order to optimally facilitate the growth of social cognition and language.

Autistic Disorder↗
PubMed2004

Children with cochlear implants: changing parent and deaf community perspectives.

OBJECTIVE: To examine changing parent and deaf community perspectives related to pediatric cochlear implantation. DESIGN: This research is based primarily on 2 nonrandom study designs. In the first study, conducted by the Gallaudet University Research Institute, Washington, DC, in the spring of 1999, a 12-page questionnaire was distributed to 1841 parents of children with cochlear implants; 439 questionnaires were returned. In the second study, we conducted 56 interviews with parents of 62 children with implants (and 1 without). SUBJECTS: Parents of children with cochlear implants; Gallaudet University faculty, staff, students, and alumni. RESULTS: While parents frequently receive conflicting information about educational and communication options for their child, they generally support signing before and after implantation. The parents of a child with an implant have a great interest in their child's spoken language development, and most would like to have had their child receive an implant earlier. Children with implants are educated in a variety of educational settings. Mainstreamed children with implants often continue to require classroom support services, and children with implants are frequently not isolated from both deaf and hearing peers. Parents have mixed experiences when getting information from persons in the deaf community. COMMENT: Opposition to pediatric cochlear implantation within the deaf community is giving way to the perception that it is one of a continuum of possibilities for parents to consider. To ensure optimal use of the cochlear implant, parents need to remain involved in their child's social and educational development.

Adolescent↗
PubMed1999

A comparison of language achievement in children with cochlear implants and children using hearing aids.

English language achievement of 29 prelingually deaf children with 3 or more years of cochlear implant (CI) experience was compared to the achievement levels of prelingually deaf children who did not have such CI experience. Language achievement was measured by the Rhode Island Test of Language Structure (RITLS), a measure of signed and spoken sentence comprehension, and the Index of Productive Syntax (IPSyn), a measure of expressive (signed and spoken) English grammar. When the CI users were compared with their deaf age mates who contributed to the norms of the RITLS, it was found that CI users achieved significantly better scores. Likewise, we found that CI users performed better than 29 deaf children who used hearing aids (HAs) with respect to English grammar achievement as indexed by the IPSyn. Additionally, we found that chronological age highly correlated with IPSyn levels only among the non-CI users, whereas length of CI experience was significantly correlated with IPSyn scores for CI users. Finally, clear differences between those with and without CI experience were found by 2 years of post-implant experience. These data provide evidence that children who receive CIs benefit in the form of improved English language comprehension and production.

Achievement↗
PubMed2006

Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech.

Apraxia of speech (AOS) is a motor speech disorder characterized by slow speaking rate, abnormal prosody and distorted sound substitutions, additions, repetitions and prolongations, sometimes accompanied by groping, and trial and error articulatory movements. Although AOS is frequently subsumed under the heading of aphasia, and indeed most often co-occurs with aphasia, it can be the predominant or even the sole manifestation of a degenerative neurological disease. In this study we determine whether the clinical classifications of aphasia and AOS correlated with pathological diagnoses and specific biochemical and anatomical structural abnormalities. Seventeen cases with initial diagnoses of a degenerative aphasia or AOS were re-classified independently by two speech-language pathologists--blinded to pathological and biochemical findings--into one of five operationally defined categories of aphasia and AOS. Pathological diagnoses in the 17 cases were progressive supranuclear palsy in 6, corticobasal degeneration in 5, frontotemporal lobar degeneration with ubiquitin-only-immunoreactive changes in 5 and Pick's disease in 1. Magnetic resonance imaging analysis using voxel-based morphometry (VBM), and single photon emission tomography were completed, blinded to the clinical diagnoses, and clinicoimaging and clinicopathological associations were then sought. Interjudge clinical classification reliability was 87% (kappa = 0.8) for all evaluations. Eleven cases had evidence of AOS, of which all (100%) had a pathological diagnosis characterized by underlying tau biochemistry, while five of the other six cases without AOS did not have tau biochemistry (P = 0.001). A majority of the 17 cases had more than one yearly evaluation, demonstrating the evolution of the speech and language syndromes, as well as motor signs. VBM revealed the premotor and supplemental motor cortices to be the main cortical regions associated with AOS, while the anterior peri-sylvian region was associated with non-fluent aphasia. Refining the classification of the degenerative aphasias and AOS may be necessary to improve our understanding of the relationships among behavioural, pathological and imaging correlations.

Aged↗
PubMed2001

Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study.

A 62-year-old woman initially presented with slowly progressive nonfluent aphasia with minimal intellectual involvement. Echolalia and personality change were prominent whereas parkinsonian features and signs suggesting parietal lobe dysfunctions were not present. The patient's language deficit was consistent with transcortical motor aphasia. She did not manifest extrapyramidal signs. The patient was diagnosed as having Pick's disease or frontal lobe dementia. She died at age 65, 2 years and 9 months following disease onset. Neuropathological findings including cytoskeletal abnormalities, however, were clearly distinct from those of classical Pick's disease and were consistent with those reported in corticobasal degeneration (CBD). The distribution of her cortical lesions was accentuated in the frontal language-related area. The clinical manifestations in CBD are diverse, and primary progressive nonfluent aphasia should be considered as an initial symptom of CBD. Neuropathological examination of such patients should include cytoskeletal abnormality studies.

Aphasia, Primary Progressive↗
PubMed1996

Involvement of the white matter in hypomelanosis of Ito (incontinentia pigmenti achromiens).

We report our clinical and neuroradiologic findings in 13 patients affected by hypomelanosis of Ito. Seven patients were boys and six were girls; their ages ranged from 11 months to 16 years. Neurologic signs were present in all but two cases, and they consisted of language disabilities, seizures, hypotonia, mental retardation, and autistic behavior. MRI was performed in all patients. We observed anomalies of the white matter in seven of the 13 patients; all but one of these seven had neurologic signs that included seizures, hypotonia, language disabilities, and mental retardation. The abnormal signals in the white matter were mostly located in the parietal periventricular and subcortical regions of both hemispheres. Moreover, we found asymmetry of the cerebral hemispheres in one of our 13 patients and atrophy of the cerebellar vermis in another patient, with no involvement of the white matter in either. In the remaining four of the 13 patients results of MRI appeared normal. There was a relationship between the anomalies in the central nervous system at MRI, as a whole, and the neurologic manifestations, even though two patients with apparently normal images on MRI had partial and generalized tonic clonic seizures, respectively. A correlation was also found between white matter anomalies and neurologic signs; extended and deep changes in white matter images were associated with more severe neurologic abnormalities and delayed language milestones appeared to be a constant finding in this group of patients. These anomalies of the white matter, which did not progress over time, resembled those seen in other neurocutaneous syndromes. The hypothesis is presented that underlying disarray of cortical lamination or neuronal loss with subsequent wallerian degeneration and altered or delayed myelination could be the cause of the abnormal findings on MRI.

Adolescent↗
PubMed1988

A long-term follow-up case study of crossed aphasia assessed by single-photon emission tomography (SPECT), language, and neuropsychological testing.

A 65-year-old man with well-defined crossed aphasia secondary to right cerebral infarction 10 years previously was studied for current language and cognitive abilities and regional cerebral blood flow (rCBF) during cognitive activation measured by single-photon emission tomography (SPECT). Reversed hemispheric lateralization was demonstrated by qualitative aspects of the patient's constructional deficits, dominant parietal lobe signs, and absence of the neglect syndrome. Language activation procedures during SPECT produced focal increases in rCBF to both frontal lobes with a phoneme detection task and to right temporal and parietal lobes with a math task. The authors stress the complexities of assessing brain/language mechanisms in vivo and demonstrate variabilities in rCBF during language activation dependent on task selection.

Aged↗
PubMed1997

Boys with Asperger's disorder, exceptional verbal intelligence, tics, and clumsiness.

Five boys with both Asperger's disorder and Tourette syndrome, exceptional verbal intelligence, and clumsiness are reported. Each presented at early elementary school age with a prominent complaint of social difficulties with peers. History was notable for a flapping stereotypy and the neurological examination revealed motor and/or vocal tics and numerous motor soft signs. Highly specialized interests were characteristics. Language prosody and/or pragmatics was impaired. Despite exceptional verbal intelligence, the children were not, according to their teachers and parents, faring well either socially or academically. Motor difficulties, manifested psychometrically as a significant performance IQ disadvantage, interfered with school performance and social adjustment. Tics, although not noted by parents in the clinical history, compounded their social difficulties. Asperger's disorder in these highly verbal children overlaps with pervasive developmental disorder (PDD) on account of the socioemotional difficulties and stereotypies seen in both. Asperger's disorder and Tourette syndrome overlap in these children on account of the tics. Finally, Asperger's disorder and the right-hemisphere-based learning disorders overlap on account of the visuoperceptual and attentional deficits that can occur in both.

Child, Preschool↗
PubMed1995

Early onset Alzheimer's disease in a South American pedigree from Argentina.

We report the clinical, SPET, immunohistochemical and DNA features of an early-onset familial Alzheimer's disease (FAD) in an Argentine pedigree of South American indian ethnic background. Pedigree spans 5 generations comprising more than 110 biological relatives. Clinical data supported the diagnosis of early onset FAD (mean age at onset 38.9 years) in 10 family members, including 3 with pathological confirmation (mean age at death 48.5). The pattern of transmission suggested autosomal dominant inheritance. Prominent features were mood changes, early language impairment, myoclonus, seizures and cerebellar signs. SPET displayed bilateral frontal, temporo-parietal and cerebellar hypoperfusion in early stages and in an asymptomatic member at risk, suggesting that SPET may have predictive value in this family. Immunohistochemistry showed beta amyloid deposits within neuritic plaques and vessel walls and no anti-PrP immunoreactivity. DNA analysis showed no abnormalities in the beta amyloid precursor protein gene. The identification of additional genetic defects in well characterized independent FAD pedigrees will contribute to the understanding of the pathogenesis of Alzheimer's disease.

Adult↗
PubMedDate not supplied

Correlations between cognitive impairment, middle cerebral artery flow velocity and cortical glucose metabolism in the early phase of Alzheimer's disease.

In a previous transcranial Doppler (TCD) study, we demonstrated a decrease in blood flow velocity in the proximal tract of the middle cerebral artery (MCA) in patients with Alzheimer's disease (AD). In these patients there was also an asymmetry in blood flow velocity which positively correlated with the cognitive asymmetry often seen in the early phase of AD. In this study we found a correlation between the absolute values and asymmetry indexes of MCA blood flow velocity with adjusted metabolic values and asymmetry indexes of the relative cortical frontotemporoparietal (FTP) areas, evaluated by FDG-PET, and with neuropsychological asymmetry indexes. Patients with prevalent visuospatial deficits (right hemisphere dysfunction) showed significant decreases in right MCA blood flow velocity and right FTP cortical glucose hypometabolism, whereas in patients with prevalent language deficits (left hemisphere dysfunction), these signs were observed on the other side. In AD patients, the decrease of blood flow velocity in MCA might be due to reduced metabolic demands in the temporoparietal cortical areas primarily affected by AD.

Aged↗
PubMed2004

Verbal autopsy of 80,000 adult deaths in Tamilnadu, South India.

BACKGROUND: Registration of the fact of death is almost complete in the city of Chennai and not so in the rural Villupuram district in Tamilnadu, India. The cause of death is often inadequately recorded on the death certificate in developing countries like India. A special verbal autopsy (VA) study of 48,000 adult (aged >or= 25 yrs) deaths in the city of Chennai (urban) during 1995-97 and 32,000 in rural Villupuram during 1997-98 was conducted to arrive at the probable underlying cause of death to estimate cause specific mortality. METHODS: A ten day training on writing verbal autopsy (VA) report for adult deaths was given to non-medical graduates with at least 15 years of formal education. They interviewed surviving spouse/close associates of the deceased to write a verbal autopsy report in local language (Tamil) on the complaints, symptoms, signs, duration and treatment details of illness prior to death. Each report was reviewed centrally by two physicians independently. Random re-interviewing of 5% of the VA reports was done to check the reliability and reproducibility of the VA report. The validity of VA diagnosis was assessed only for cancer deaths. RESULTS: Verbal autopsy reduced the proportion of deaths attributed to unspecified and unknown causes from 54% to 23% (p < 0.0001) in urban and from 41% to 26% (p < 0.0001) in rural areas in Tamilnadu for adult deaths (>or= 25). The sensitivity of VA to identify cancer was 95% in the age group 25-69. CONCLUSION: A ten day training programme to write verbal autopsy report with adequate feed back sessions and random sampling of 5% of the verbal autopsy reports for re-interview worked very well in Tamilnadu, to arrive at the probable underlying cause of death reliably for deaths in early adult life or middle age (25-69 years) and less reliably for older ages (70+). Thus VA is practicable for deaths in early adult life or middle age and is of more limited value in old age.

Adult↗
PubMed2002

Recognition of cuneiform inscription signs by use of a hybrid-optoelectronic correlator device.

A hybrid-optoelectronic correlator device and an algorithm are proposed for recognizing cuneiform inscription signs. The device is based on the extended correlator architecture with three liquid-crystal display(s) (LCD)s and three light detectors: one CCD camera for capturing the input image, oneLCD for displaying the input image, two LCDs for the complex correlation filter (amplitude and phase parts), and two detectors for measuring the total and peak intensities of the output correlation information. The recognition algorithm is designed to allow automatic as well as real-time processing. The recognition results are given for the cuneiform signs impressed on an original clay tablet. The investigated tablet (VAT 12890 of the Pergamon Museum, Berlin, Germany) was found in Bogazköy (Hattusha) and dates from the 14th century B.C. It is a fragment of the Epic of Gilgamesh in the Akkadian language with a large number of the sign samples.

Journal Article↗
PubMed2002

[Clinical diagnosis of autism].

INTRODUCTION: The disorders of the autistic spectrum form a collection of symptoms due to dysfunction of the central nervous system with great variations in the degree of severity. Autism is considered to be a generalized disorder of development (DSM-IV). Autism is not defined as a specific disease, since it does not have a specific aetiology. DEVELOPMENT: There are many syndromes related to autism, but most of these disorders are not selective and show a combination of autistic symptoms together with symptoms of neurological dysfunction. There is no specific aetiology, although in recent years genetics have been shown to be important. The prevalence varies between 1 and 1.2/1,000. Boys are more often affected than girls, in a proportion of 3-4 to 1. Diagnosis is clinical and is based on alterations of social interaction, problems of communication and also a restricted range of activities and interests (DSM-IV). There are anomalies associated with behaviour problems, such as delay in speaking, mental retardation, sensorial defects and motor difficulties. CONCLUSIONS: Over 75% of autistic children have mental retardation, and this proportion is higher in severe cases, especially when the children have attention deficit with hyperactivity. These children have many of the typical signs of autism: stereotyped movements, inappropriate language, obsessive behaviour with little mental flexibility, naivety and little skill in social interaction. In these cases it is difficult to draw the line between mental retardation and autism.

Attention Deficit Disorder with Hyperactivity↗
PubMed1982

The dimension of inattention among children with school problems.

Within a population of children referred for school-related problems, youngsters with significant attention deficits were compared with children having other types of learning problems. Using data from parent and teacher questionnaires as well as results of multidisciplinary team assessments, each patient was assigned either to a group with significant attention deficits or to one with learning problems, but fewer, if any, problems with attention. Disagreement between observation sources was demonstrated. Children qualifying for the significant attention deficit group were characterized by a greater likelihood of having behavioral problems during the toddler and preschool years. They also had a higher prevalence of minor neurologic signs and difficulty on tests of language development. Their current behavioral and adjustment problems seemed to be more severe and widespread than those of the youngsters with whom they were compared. No major intergroup differences were observed with regard to age at referral, socioeconomic status, family history of learning and behavior problems, perinatal health risks, or psychosocial difficulties. There was considerable historic and symptomatic overlap between the two groups, suggesting that the clinical picture characteristic of significant attention deficits is relatively nonspecific and is either a primary or secondary finding in a large proportion of a heterogeneous population of children experiencing difficulties in school.

Adolescent↗
PubMed1998

[Neurological assessment of learning disorders].

INTRODUCTION: The neurological concept of learning is approached from a cybernetic point of view, taking into account that a child should recognize a fact, learn it semantically and decided whether it is worth storing; the dynamic aspect of memory is the true motor of the ability to learn and all this is modulated by the attention factor. DEVELOPMENT: The neurological evaluation of learning disorders is based on clinical examination which includes the so-called minor signs of the noetic functions, specifically language, the praxes, gnosias, perceptive-motor function, laterality and the lexical, graphic and calculation functions together with the modulating element, mentioned above, of the level of attention with or without hyperactivity. These semiological elements are grouped into three major categories of syndromes: motor syndrome, dyslexic-dysgraphic-dyscalculation syndrome and the hyperkinetic syndrome or attention deficit with hyperactivity. We also note the differential diagnosis. We review the neurophysiological biological markers (EEG and brain mapping, cerebral evoked potentials, neurometry) and those based on neuroimaging techniques (cerebral CT, MR, SPECT and PET). CONCLUSIONS: The contribution of neurological assessment is considered as part of the functions of a multi-disciplinary team which should deal with the diagnosis and treatment of children with learning disorders.

Attention Deficit Disorder with Hyperactivity↗
PubMedDate not supplied

Acquisition of graphic communication by a young girl without comprehension of spoken language.

PURPOSE: To describe a graphic-mode communication intervention involving a girl with intellectual impairment and autism who did not develop comprehension of spoken language. The aim was to teach graphic-mode vocabulary that reflected her interests, preferences, and the activities and routines of her daily life, by providing sufficient cues to the meanings of the graphic representations so that she would not need to comprehend spoken instructions. METHOD: An individual case study design was selected, including the use of written records, participant observation, and registration of the girl's graphic vocabulary and use of graphic signs and other communicative expressions. RESULTS: While the girl's comprehension (and hence use) of spoken language remained lacking over a 3-year period, she acquired an active use of over 80 photographs and pictograms. CONCLUSIONS: The girl was able to cope better with the cognitive and attentional requirements of graphic communication than those of spoken language and manual signs, which had been focused in earlier interventions. Her achievements demonstrate that it is possible for communication-impaired children to learn to use an augmentative and alternative communication system without speech comprehension, provided the intervention utilizes functional strategies and non-language cues to the meaning of the graphic representations that are taught.

Abnormalities, Multiple↗
PubMed2006

[Functional reorganization of language in a case of cortical development disorder with continuous spike and wave during sleep].

INTRODUCTION: Left cerebral hemisphere dominance for language is a well known fact. However, this typical lateralization may be affected when left hemisphere is damaged at an early age. In this case, neuronal plasticity allows language to be totally or partially transferred to another area within the ipsilateral or contralateral hemisphere. CASE REPORT: 21 year old woman. Congenital right hemiparesis. Focal epilepsy and continuous spike and wave during NREM sleep at 4 years old. Magnetic resonance imaging: extensive malformation of cortical development in the left hemisphere. From 7 years old, seizures were controlled and the continuous spike and wave discharges disappeared. TREATMENT: Valproic acid, 500 mg/day. Neuropsychological assessment showed that basic language functions were well preserved, while there were signs of non-dominant hemisphere dysfunction. These paradoxical results, together with the fact that the patient was left-handed, established the possibility of functional reorganization of language to the right hemisphere. A functional magnetic resonance imaging (fMRI) study for language lateralization demonstrated preferential right hemisphere activation on Broca's and Wernicke's areas. CONCLUSIONS: This case shows the brain's potential for functional reorganization, especially if, according to Kennard's principle, the injury is produced at an early age.

Adult↗