[Relationship between brain waves and psychological and behavioral abilities in mild dementia of the aged].
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OBJECTIVES: To define the cognitive characteristics of Pick disease (PcD), and to determine which features distinguish PcD from Alzheimer disease (AD), in a cross-sectional and longitudinal study. METHODS: The participants were 44 patients with PcD (10 pathologically verified), 121 patients with AD (14 pathologically verified), and 60 normal control subjects. We obtained information regarding the initial symptom of dementia from each patient's caregiver, estimated global dementia severity by the Blessed Dementia Scale and the Activities of Daily Living Scale, and assessed specific cognitive domains by administering 10 tests of memory, language, visuospatial, and reasoning abilities and selective attention. RESULTS: Among initial symptoms reported by caregivers, personality change and language impairment were significantly more common in PcD than AD; deficits in memory were common in both groups but more prevalent in AD (P<.001). At initial cognitive testing, the scores of patients with PcD were inferior to those of normal controls on all tests, except on a measure of visuospatial function; the scores of patients with AD were inferior to those of controls on all tests. Patients with PcD were superior to patients with AD on measures of explicit memory (P<.001) and visuospatial function (P = .001) but had greater impairments on the Activities of Daily Living Scale (P<.05). During the course of illness, patients with PcD declined significantly faster than those with AD on language tests and on global measures of dementia severity (P<.05), whereas measures of explicit memory and visuospatial and reasoning abilities worsened equally in both patient groups. CONCLUSIONS: There is a characteristic cognitive profile and course of dementia in PcD. Nonetheless, cognitive test performance does not clearly distinguish PcD from AD.
PURPOSE: Selective amobarbital tests with selective temporary inactivation of the left frontal operculum and/or the left parietotemporal cortex were performed in 5 patients with left-hemispheric epileptogenic lesions in or adjacent to classical Broca's and/or Wernicke's area. The aim was to assess language functions in these brain regions before surgery, to tailor the surgery according to the individual functional importance of these brain regions, and to predict postoperative outcome. METHODS: Amobarbital was injected by transfemoral selective catheterization of the arteries supplying the target areas. Along with neuropsychological and neurological testing during the amobarbital procedure, EEG recordings were performed in all patients, and [99mTc]HMPAO-single photon emission computed tomography (SPECT) in 2 patients. RESULTS: After the amobarbital injection into the left frontal opercular region, there was no recognizable language dysfunction in 3 patients. In these 3 patients, the lesions in or adjacent to the frontal operculum were completely resected without postoperative language impairment. In the remaining 2 patients, temporary language impairment after the amobarbital injection into the left frontoopercular and Wernicke's region, respectively, suggested language functions in these areas. Surgery was restricted to the left mesiotemporal lobe in 1 patient. In the other patient, the tumor infiltrating the frontal operculum was restrictively resected. Postoperatively, the first patient had no language impairment, but the latter had transient global aphasia, from which she recovered. CONCLUSIONS: Selective temporary amobarbital inactivation of brain regions that may be associated with language has clearly indicated the presence or absence of language functions in these regions. The test contributed substantially to planning of the surgical approach in each patient. The predictive value of the amobarbital test was demonstrated by the postoperative outcome.
Previous studies suggest disparities in use of preventive cancer services among U.S. Hispanics are partly explained by knowledge and access factors. One area of emerging interest is uptake of genetic counseling and testing services by underserved populations. This study aims to estimate the percentage of Hispanics in five ethnic subgroups who are aware of genetic testing for inherited cancer risk, and to assess the influence of acculturation factors primarily related to language on test awareness. Weighted data from 4,313 Hispanic respondents (age >25 years) in the year 2000 National Health Interview Survey were analyzed. Overall, 20.6% of Hispanics had heard of genetic testing for cancer risk, with percentages highest among Puerto Ricans (27.3%) and lowest among Mexicans (14.3%). Completing the interview in Spanish and English [odds ratio (OR), 0.52; 95% confidence interval (95% CI), 0.35-0.78], or only Spanish (OR, 0.60; 95% CI, 0.42-0.86), was inversely associated with test awareness (reference group, only English). Having an intermediate (OR, 0.66; 95% CI, 0.48-0.90) or low (OR, 0.63; 95% CI, 0.39-1.01) level of English language preference was also inversely associated (reference, high level) whereas being born outside the United States was weakly associated (OR, 0.80; 95% CI, 0.57-1.11). Estimates were adjusted for age, education, ethnicity, parents' cancer history, health care access, and selected health behaviors and beliefs. Results of this national survey indicate that acculturation factors related to language may affect cancer genetic test awareness in Hispanics. These factors must be taken into account when informing individuals about the role of genetics in cancer risk and providing cancer genetic health services.
For 42 children from rural north central Kansas school districts (27 boys, 15 girls) who were referred for Special Education testing, the WISC-R IQs (Full Scale, Verbal, and Performance), the means on the Expressive One Word Picture Vocabulary Test, the Peabody Picture Vocabulary Test--Revised, and the Test of Language Development were recorded from the children's files. No sex differences on any variable were noted; all variables correlated (Pearson) significantly but varied in magnitude. This group of tests, being intercorrelated, can be used to collect information on children's academic, speech, and language abilities. Valid judgments can be made regarding children's academic abilities for school when multiple tests are administered by professional staff.
The similarity between factors associated with child abuse/neglect and those associated with language disability suggests that maltreated children are a population at risk for language problems. This study investigated the performance of three groups of abused/neglected children and a matched group of nonmaltreated subjects on three tests of language comprehension. Results revealed significant differences among groups for all measures. Severely neglected children obtained the lowest scores on all tests; the abused children consistently obtained lower scores than the controls; and generally neglected children showed the smallest difference in performance from the control group. These findings suggest a model for understanding relationships between abuse/neglect and language disability.
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The course of development of a 5-year-old boy with a serious developmental retardation and massive behaviour problems who spoke only a few words is documented and described with psychometric tests and spontaneous language samples up to the age of 8-9. Inspite of this developmental retardation and poor care, the child reached until then an average to above-average intelligence in nonverbal functions. The symptoms were reduced only to a specific language impairment. At the age of 11, this disorder as well had practically disappeared. Such a course of development after the age of 5 is not common, whereas a specific language impairment normally remains a deficit. Possible causes for this favourable development are discussed.
This paper explores how the test-retest reliability is modulated by different groups of participants and experimental tasks. A group of 12 healthy participants and a group of nine stroke patients performed the same language imaging experiment twice, test and retest, on different days. The experiment consists of four conditions, one audio condition and three audiovisual conditions in which the hands are either resting, gesturing, or performing self-adaptive movements. Imaging data were analyzed using multiple linear regression and the results were further used to generate receiver operating characteristic (ROC) curves for each condition for each individual subject. By using area under the curve as a comparison index, we found that stroke patients have less reliability across time than healthy participants, and that when the participants gesture during speech, their imaging data are more reliable than when they are performing hand movements that are not speech-associated. Furthermore, inter-subject variability is less in the gesture task than in any of the other three conditions for healthy participants, but not for stroke patients.
OBJECTIVE: To adapt and validate a Spanish language medium test, of theoretical general knowledge of diabetes mellitus (questionnaire from the University of Michigan). To determine the validity of the concurrent and discriminatory content and establish reliability. DESIGN: The study was observational. Validity was verified prior to data collection. To analyse the concurrent and discriminatory validity, a questionnaire was used in personal interview with the patients, and the degree of knowledge evaluated on certain variables. SETTING: Hospital outpatient endocrinology consultations. PATIENTS: 167 diabetic patients were chosen at random, from the outpatient visits. 14 patients who had developed hearing, or language problems, or who had problems of a psychological nature, were excluded. Only 1 patient refused to answer the questionnaire. MAIN MEASUREMENTS AND RESULTS: Validity of the content was confirmed after careful analysis of the questions on the questionnaire by medical specialists in endocrinology. It was found that the test had adequate concurrence (p less than 0.01) when the average general knowledge levels of certain group of patients are compared. It also had acceptable discriminatory validity (r = 0.56: p less than 0.0001) and reliability (alpha: 0.84; p less than 0.45). CONCLUSIONS: Adaptation and validation has been obtained for a test of theoretical general knowledge on diabetes mellitus, and the test was found to be applicable to the population under study.
INTRODUCTION: Specific language disorder (SLD) is defined as a disorder in which language is slow and retarded with respect to the patient's chronological age, and which is not related to a sensory-auditory or motor deficit or to a pervasive development disorder; in other words, structural and functional neurological developmental disorders must be excluded. After many studies and attempts to reach an agreement, specific language disorders have been broadly classified into two groups: specific expressive language disorder and specific receptive-expressive language disorder. AIMS: Our objective was to study SLD using magnetoencephalography (MEG) in order to look for epileptiform manifestations in precise language areas. PATIENTS AND METHODS: We studied a sample made up of 11 patients with SLD, one of whom had Landau-Kleffner syndrome and one with cryptogenic-type SLD. The criteria used were age-dependent autism exclusion tests and specific language tests for SLD. The MSI technique was carried out on all the patients, by magnetic resonance and MEG. RESULTS: A characteristic pattern is obtained for SLD in the form of irregular spike and polyspike-wave discharges in bilateral frontal channels. The dipoles were situated mainly in the left perisylvian areas. CONCLUSIONS: The study of specific language disorders using MEG enables us to identify the SLD included in a proposed classification as primary-type specific language disorder, cryptogenic-type specific language disorder and secondary-type type specific language disorder, like Landau-Kleffner syndrome.
It is currently thought that fragile X syndrome (FraX; the most common inherited form of learning disability) results from having more than 200 cytosine-guanine-guanine (CGG) trinucleotide repeats, with consequent methylation of the fragile X mental retardation (FMR1) gene and loss of FMR1 protein (FMRP). It was also considered that premutation carriers (with 55-200 CGG repeats) are unaffected, although a tremor/ataxia syndrome has recently been described in older adult male carriers. We reported that premutation expansion of CGG trinucleotide repeats affects brain anatomy, which, together with other studies, indicates that the molecular model for FraX needs modification. However, there are few studies on the cognitive ability of adult male premutation carriers. Thus, we selected 20 male premutation carriers on the basis of their genetic phenotype, and compared them to 20 male controls matched on age, IQ and handedness. We investigated intellectual functioning, executive function, memory, attention, visual and spatial perception, and language and pragmatics. The premutation carriers had significant impairments on tests of executive function (Verbal Fluency, Trail Making Test and Tower of London) and memory (Names sub-test of the Doors and People, Verbal Paired Associates Immediate Recall and Visual Paired Associates Delayed Recall sub-tests of the WMS-R, and Category Fluency Test for natural kinds). We therefore suggest that CGG trinucleotide repeats in the premutation range affect specific neuronal circuits that are concordant with specific neuropsychological deficits; and that these deficits reflect an emerging neuropsychological phenotype of premutation FraX.
BACKGROUND: Very few assessment measures exist for evaluating progress in young deaf children with hearing aids and cochlear implants. OBJECTIVE: To introduce and describe an early assessment package that covers auditory perception, communication/language development, and speech production in very young deaf children. MAIN OUTCOME MEASURES: Seven of the assessment measures (Listening Progress Profile, Categories of Auditory Performance, Tait Video Analysis, Stories-Narratives Assessment Procedure, Profile of Actual Linguistic Skills, Speech Intelligibility Rating, and the Profile of Actual Speech Skills) have been specifically developed at the Nottingham Cochlear Implant Programme, and a further one (Meaningful Auditory Integration Scale) was modified for use within the package. Moreover, two commercially available tests (Pragmatics Profile of Everyday Communication Skills and Preschool Language Scale) are included to complete the package. METHODS: The present paper describes each measure, how to use it, and its time frame. In addition, two case studies demonstrate the usefulness of the package as a whole. RESULTS AND CONCLUSIONS: The Nottingham Early Assessment Package (NEAP) offers a framework with which to assess in young deaf children the use of audition and language and communication in real-life situations. Being simple, reliable, and time effective can be used in everyday clinical practice. NEAP is innovative in design and offers a structured approach to monitor very young deaf children, both in short and long term. In addition, it allows the identification of additional problems and areas of difficulty as well as specific abilities and skills. This enables the clinician to determine appropriate intervention strategies.