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Bilateral temporal lobe volume reduction parallels cognitive impairment in progressive aphasia.

BACKGROUND: Patients with isolated aphasia in the absence of other cognitive abnormalities have been the focus of several studies during the past decade. It has been called primary progressive aphasia (PPA), and the typical features of this syndrome are marked atrophy of the left temporal lobe according to the radiological examination and a language disorder as the initial symptom. In previous studies of PPA, the selection of the patients was based mainly on linguistic symptoms. Now, when computed tomography or magnetic resonance imaging scans are part of the routine investigation of cognitive impairment and suspected dementia, the patients with lobar atrophy will be found at an earlier stage. In the present study, we used a new approach and defined the study group by selecting patients with obvious left temporal lobe atrophy, assessed by MRI, and we referred to them as patients with temporal lobe atrophy (TLA). OBJECTIVE: To identify the features that distinguish TLA from other primary neurodegenerative disorders. PATIENTS: Six patients with TLA were compared with patients with Alzheimer disease (AD), patients with frontal lobe dementia (FLD), and healthy control subjects. METHODS: The investigations included magnetic resonance imaging volumetry, single photon emission computed tomography, and neuropsychologic and linguistic evaluations. RESULTS: In the TLA group, the mean volume of the left temporal lobe was 35% smaller than the right, while in the AD and FLD groups, the atrophy was symmetrical and bilateral. In the TLA group, the absolute volumes of the temporal lobes were significantly smaller on the left side compared with the AD and FLD groups, whereas there was no difference on the right side. The cerebral blood flow pattern in TLA was asymmetric and differed from that in the other study groups. All patients with TLA had a history of progressive Wernicke-type aphasia, ranging from 2 to 6 years. They showed primary verbal memory impairment but had preserved visuospatial functions. The clinical condition of all patients with TLA deteriorated during the study period; severe aphasia developed, and the patients exhibited signs of frontal lobe dysfunction. Serial volumetric measurements in 4 of 6 patients showed an annual 8% to 9% decrease of both left and right temporal lobes. CONCLUSIONS: The initial marked asymmetry in cognitive function found in patients with TLA contrasts with the general decline found in patients with AD. The bilateral degenerative process evident in patients with TLA paralleled the clinical deterioration, indicating TLA to be a non-AD lobar atrophy that develops into generalized cognitive dysfunction and dementia.

Aged↗

Familial aggregation in specific language impairment.

A case-control family study design, in which the current language-related abilities of all biological, primary relatives (mother, father, siblings) of probands with specific language impairment (SLI) and matched controls were assessed, was used to investigate familial aggregation for language disorders. Current test data from each family member showed the rate of language impairment for mothers, fathers, sisters, and brothers of the SLI probands to be significantly higher than for members of control families. Impairment rates for fathers and mothers were approximately equal, whereas rates for brothers were significantly higher than for sisters. In SLI proband families, Language Impairment (LI) occurred in 13.0% of offspring (excluding proband) with neither parent affected, 40% of offspring with one parent affected, and 71.4% of offspring in families in which both parents were language impaired. Rates of impairment as determined in current testing were compared directly to impairment rates estimated from family-history questionnaires collected from the same families. Group data showed impairment rates estimated from the family-history questionnaires to be similar to the rates based on actual testing. Furthermore, both appeared in line with rates based primarily on questionnaire data as reported previously in the literature. However, case-by-case analyses showed poor intrasubject agreement on classification as language impaired on the basis of current testing as compared to history information.

Adolescent↗

Executive functioning in high-functioning children with autism.

Executive functioning was investigated in 34 children (24 boys and 10 girls) with developmental language disorder (DLD) and 21 children (18 boys and 3 girls) with high-functioning autistic disorder (HAD) matched on Full Scale IQ, Nonverbal IQ, age (mean age 9 year, 1 month), and SES. The DLD group had a Verbal IQ that was 10 points higher than the HAD group. These children were given the Wisconsin Card Sorting Test (WCST), the Mazes subtest from the WISC-R, the Underlining test, and the Rapid Automatized Naming test. In addition, these children were given the Vineland Scales of Adaptive Functioning and the Wing Diagnostic Symptom Checklist in order to assess severity of autistic symptomatology. Results indicated that the only significant difference between the two groups on the cognitive tasks was perseverative errors on the WCST; there was no significant difference on total number of categories achieved or total number of errors on the WCST or on the other executive function measures. There was also significant overlap in the scores between the two groups and the difference in perseverative errors was no longer significant when Verbal IQ was partialled out. Executive functioning was strongly related to all IQ variables in the DLD group and particularly related to Verbal IQ in the HAD group. Although there was a relationship in the HAD group between executive functioning and adaptive functioning, as well as between executive functioning and autistic symptomatology, these relationships were generally no longer significant in the HAD group after the variance due to Verbal IQ was accounted for. The results are interpreted to indicate that although impaired executive functioning is a commonly associated feature of autism, it is not universal in autism and is unlikely to cause autistic behaviors or deficits in adaptive function.

Autistic Disorder↗

Neurophysiological evidence of auditory channel anomalies in developmental dysphasia.

Steady-state auditory evoked responses to frequency-modulated tones were obtained from normal children and two groups of children with developmental language disorders (developmental dysphasia). Children with predominantly expressive language impairment produced responses not different from normal children, while children with primary receptive language impairment produced responses that were markedly diminished, even absent. This occurred in recordings from either cerebral hemisphere and at mean frequency-modulation depths ranging from +/- 20 to +/- 100 Hz. Pathophysiology of auditory mechanisms concerned with frequency-modulation analysis are particularly associated with receptive developmental language impairment and may underlie associated difficulties in speech perception.

Acoustic Stimulation↗

Individual differences in the onset of tense marking: a growth-curve analysis.

The purpose of this study was to explore individual differences in children's tense onset growth trajectories and to determine whether any within- or between-child predictors could account for these differences. Twenty-two children with expressive vocabulary abilities in the low-average to below-average range participated. Sixteen children were at risk for specific language impairment (SLI), and 6 children had low-average language abilities. Spontaneous language samples, obtained at 3-month intervals between 2;0 and 3;0, were analyzed to examine change in a cumulative productivity score for 5 tense morphemes: third person singular present, past tense, copula BE, auxiliary BE, and auxiliary DO. Hierarchical linear modeling was used to model intercept and linear growth at 30 months and quadratic growth overall. A growth model that included mean length of utterance (MLU) and MLU growth better explained within-child productivity score growth trajectories than a parallel model with vocabulary and vocabulary growth. Significant linear growth in productivity scores remained even after a control for MLU was in place. When between-child predictors were added in the final conditional model, only positive family history approached statistical significance, improving the overall estimation of the model's growth parameters. The findings support theoretical models of language acquisition that claim relative independence of tense marking from other more general aspects of vocabulary development and sentence length. The trends for family history are also consistent with proposals implicating faulty genetic mechanisms underlying developmental language disorders. Systematic use of familial risk data is recommended in future investigations examining the relationship between late-talking children and children at risk for SLI.

Age Factors↗

Bias effects of socioeconomic status and sex in decision making in speech-language pathology.

Within the area of speech-language pathology there is a paucity of literature that specifically addresses the issue of bias in the referral, assessment, and placement of children in programs for the remediation of speech-language disorders. The purpose of this study was to examine the issue of bias [i.e., sex and socioeconomic status (SES)] as it related to decision making in speech-language pathology. Certified speech-language pathologists completed a questionnaire survey in which information regarding the referral and assessment of a hypothetical case study child was presented. Only the sex of the child and the parents' occupations were varied. Factor analysis and subsequent analyses of variance revealed a significant difference with regard to the SES main effect (p less than .006) for Factor 2 (Alternative Home and School Intervention Strategies). That is, it was found that regardless of sex, low SES children were viewed as more likely than high SES children to be recommended for alternative home and school intervention strategies for the remediation of speech-language delays.

Attitude of Health Personnel↗

Progressive language dysfunction and lobar atrophy.

The patterns of language disorder associated with 'progressive aphasia' due to lobar atrophy were compared with the language dysfunction of patients with dementia of frontal lobe type (DFT). The progressive aphasias were characterised primarily by impairment at the structural levels of language: phonology, grammar and semantics, whereas DFT was associated primarily with spontaneity and loss of generative capability. However, there was overlap in language symptomatology, particularly with progression of disease. The findings lend support to the argument that progressive aphasia and DFT represent different clinical manifestations of a common pathology, and form part of the spectrum of lobar atrophies.

Adult↗

Asymmetries in the superior temporal lobe in male and female first-episode schizophrenic patients: measures of the planum temporale and superior temporal gyrus by MRI.

Schizophrenia has been hypothesized to be associated with an underlying brain developmental anomaly, specifically affecting normal brain asymmetries. The most pronounced asymmetries are present on the superior surface of the temporal lobes, the left plane, as measured along the sylvian fissure (planum temporale) being longer than the right in the majority of normal individuals. These asymmetries encompass Wernicke's area, the anatomical substrate for language, and have been found to be less pronounced in individuals with developmental language problems, i.e. dyslexia. Since disordered language is one of the hallmarks of schizophrenia, the present study focuses on the planum temporale and related superior temporal gyrus. Eighty-five first-episode schizophrenic patients and 40 controls had measurements of the sylvian fissure taken from coronal slices. The pattern of asymmetry in controls was for the right length to be longer than the left in anterior slices, and for left to be longer than right in posterior slices (corresponding to the planum temporale). Schizophrenic patients as a group demonstrated less asymmetry (R > L) in anterior slices, and female patients showed a trend for less (L > R) asymmetry in posterior slices. In contrast to the report of Barta et al. (1990), the volume of the anterior superior temporal gyrus did not differ from controls in first-episode schizophrenic patients. Neither the presence of formal thought disorder nor auditory hallucinations defined a subgroup of patients with reduced size or lateralization of the planum temporal or superior temporal gyrus.

Adult↗

Thalamic thought disorder: on being "a bit addled".

Humans can generate and maintain relatively coherent trains of thought in natural discourse. The neural mediation of this ability and the phenomenology of its breakdown are not well understood. We report a case of a woman with paramedian thalamic strokes involving the mammillothalamic tract, intralaminar nuclei, parts of the dorsomedial and ventral lateral nuclei bilaterally. She presented with a dense amnesia and confusion typical of the syndrome of bilateral paramedian thalamic infarcts. Her Tc-99m HMPAO brain SPECT scan showed decreased thalamic and basal ganglia blood flow. General diminution of cerebral blood flow and areas of further diminution in the right frontal, left temporal and left temporoparietal regions were also observed. Although her amnesia was characteristic of diencephalic amnesia, her most striking clinical feature was a bizarre, disconnected and at times incoherent speech output. Analysis of her speech revealed relatively preserved lexical and morpho-syntactic linguistic production. By contrast, analysis of the macrostructure of her discourse revealed frequent unpredictable topic shifts that were completely unconstrained by contextual factors. Many of her shifts were intrusions from previous topics. We interpret her severely disordered speech output as representing the surface manifestations of a thought disorder (rather than as a language disorder per se) characterized by an inability to maintain and appropriately shift themes that normally guide discourse. Median and intralaminar thalamic nuclei appear to be critical for the neurophysiologic regulation of thalamocortical and striatocortical circuits, which in turn may be critical for the functional regulation of contextually appropriate transitions of thought.

Aged↗

Gabapentin and methylphenidate treatment of a preadolescent with attention deficit hyperactivity disorder and bipolar disorder.

Gabapentin is an anticonvulsant drug released in the United States in 1993 for use as adjunctive therapy in refractory partial epilepsy. The mechanism of action of gabapentin is unknown, but the drug has very favorable pharmacokinetics and a good safety profile, which allows its use in high-risk patients. Several reports have described the successful use of gabapentin for bipolar disorders in adults, but there are no controlled studies in the use of gabapentin in children and adolescents. We describe a 12-year-old boy with a history of attention deficient hyperactivity disorder (ADHD), reading disorder, mixed receptive and expressive language disorder, encopresis, and bipolar disorder II who was treated with gabapentin 200 mg/day added to methylphenidate 30 mg/day. Within 3 weeks the improvement and stabilization of mood symptoms was remarkable, as noted by mother, teacher, and clinician, and remained so for 6 months of follow-up. Comorbid bipolar disorder and ADHD is a hotly debated topic in the child and adolescent psychiatric literature, with rates of comorbid ADHD and bipolar disorder ranging from 22% to 90%. Controlled studies are needed to evaluate the possible antimanic mood stabilizing and/or antidepressant properties or gabapentin in youths.

Acetates↗

[Evaluation of children with developmental disorders by evoked potentials and event-related potentials].

INTRODUCTION AND DEVELOPMENT: Evoked potentials evaluation of children with central nervous system developmental disorders o dysfunctions is better than clinical and imaging assessment for determination of: 1) conceptional age, 2) auditory and visual threshold, 3) integrity of sensory pathways, 4) neurologic motor outcome, and 5) course and evaluation of treatment of neurometabolic disorders. CONCLUSIONS: However, it is not the same for predicting or making the diagnosis of the majority of primary developmental disorders (autism, receptive and expressive language disorders, learning disability, attention deficit disorders and Gilles de la Tourette syndrome.

Asphyxia Neonatorum↗

Prevalence of disabilities in a national sample of 7-year-old Israeli children.

The prevalence of chronic conditions and illnesses causing disability in Israeli Jewish children aged 7 years born in 1975 was studied on the basis of a national sample (n = 7,739). Eighty medical conditions causing disability were defined. The study showed a total disability rate of 17.5%, which is higher than that reported on a similar national sample of 3 year olds (prevalence of 6.9%). The percentage of disabilities among very low birthweight children and those with family problems was four times greater than in the total population. Mild retardation and undefined learning problems were more prevalent among children of mothers with low educational level and among children whose birth order was fourth or more. Asthma and spastic bronchitis were more prevalent among children whose mothers were of European/American origin (P less than 0.05). Behavior and mental disorders, learning problems, speech and language disorders were more prevalent among male children. Two-thirds of the children with a diagnosed problem also had at least one functional disability. There were relatively more children from lower social classes in the special education schools than in the national sample. Increased prevalence of disabilities among children of very low birthweight, low maternal educational level, high birth order, and those from families whose origin is Asian/African and from families with intrafamilial problems defines the children at risk for disabilities and placement in special education schools.

Birth Order↗

[Early diagnosis and early intervention in children with developmental disorders: introductory remarks].

Developmental disorders such as mental retardation, language disorders, autistic disorders, learning disorders, attention deficit/hyperactivity syndrome and conduct disorders are an important part of our daily practice in child neurology. Early diagnosis and early or timely intervention in these kinds of developmental disorders were stressed and family support in child-rearing was emphasized in this symposium. In addition to the above, sleep disorders in developmental disorders were discussed.

Child↗

The effects of psychoactive drugs and neuroleptics on language in normal subjects and schizophrenic patients: a review.

The aim of this survey is to present an overview of research into psychopharmacology as regards the effects of different psychoactive drugs and neuroleptics (NL) on language in normal subjects and schizophrenic patients. Eighteen studies that have investigated the effects of different drugs (alcohol, amphetamines, secobarbital, L-dopa, psilocybin, ketamine, fenfluramine) and neuroleptics (conventional and atypical) on language are reviewed. There are no studies concerning the effects of neuroleptics on language in healthy subjects. The results of the effects of other molecules indicate that language production can be increased (alcohol, amphetamine, secobarbital), rendered more complex (d-amphetamine), more focused (L-dopa) or more unfocused (psilocybin) and clearly impaired (ketamine). For schizophrenic patients, most studies show that conventional neuroleptic treatments, at a therapeutic dosage and in acute or chronic mode, reduce language disorders at all levels (clinic, linguistic, psycholinguistic). In conjunction with other molecules, the classical NL, when administered at a moderate dosage and in chronic mode, modify language in schizophrenia, either by improving the verbal flow and reducing pauses and positive thought disorder (NL + amphetamine) or by inducing an impairment in the language measurements (NL + fenfluramine). Clinical, methodological and theoretical considerations of results are debated in the framework of schizophrenic language disorders.

Adult↗

Prevalence, type, and correlates of psychiatric diagnoses in 200 children with communication disorder.

The type and prevalence of psychiatric disorders were assessed in 200 children with speech and language disorders referred to a suburban speech and hearing clinic. Possible correlates of psychiatric disorder, including demographic factors, medical and developmental problems, psychosocial stressors, and speech and language factors, were also assessed. Standardized psychiatric and speech and language evaluations were done for all subjects, as were IQ and academic testing. Approximately 50% of the sample had some definable psychiatric disorder. The most common diagnoses were behavior disorders, followed by emotional disorders. The presence of psychiatric disorder was strongly correlated with speech and language factors, as well as psychosocial stressors. These findings have implications for primary care practitioners and speech and language therapists involved in planning treatment programs for such children.

Adolescent↗

Dimensions of behavior in children with speech andlanguage disorders.

The behavioral symptoms of a population of children with speech and language disorders (with a mean age of approximately 6 years) were studied with a teacher and a parent questionnaire. A factor analysis produced similar factors for both questionnaires: Hyperactivity-Conduct, Affect, and Language. An Asocial factor was also found with the teacher questionnaire. The factors of this study were in good agreement with the general factors of "Aggression" and "Withdrawal" found in other quantitative studies. Mean factor score profiles were reported for this unique population of children.

Adolescent↗

Sampling reliability in elicited imitation.

This study examined the number of trials necessary to obtain sampling reliability in elicited imitation. An examiner-constructed elicited imitation test was administered to 15 language-disordered subjects, sampled from the age range of 5:6 to 6:6 years. All test sentences were controlled for length, syntactic construction, and semantic content. The test instrument contained multiple occurrences of 16 syntactic structures. For each of these forms, subject performance on 1, 3, 5, and 7 trials was compared with performance on 10 trials. It was observed that sampling reliability increased as the number of trials increased, but as few as three repetitions provided reliable data.

Child↗