11/22. Evidence of a developmental cerebello-cerebral disorder.The objective of this study was to examine the cognitive and neuroanatomical features of an adolescent with developmental hypoplastic left cerebellum who presented with executive and visuo-spatial deficits, nonverbal learning disabilities and interpersonal difficulties. He underwent a neuropsychological assessment, MRI and diffusion tensor imaging MRI. The neuropsychological impairments were primarily in executive functions, spatial and visual perception, graphomotor skills, arithmetic, social perception and comprehension. Fractional anisotropy, which is measured by diffusion tensor imaging and enables assessment of axonal integrity, was reduced in the right cerebral peduncle and right hemisphere white matter (p < 0.001). Based on the results, we hypothesize that disruption of neural circuits linking the hypoplastic left cerebellum to the right hemisphere may contribute to the evolution of a neurocognitive syndrome with characteristics of the developmental right hemisphere syndrome and suggestive of the cerebellar cognitive-affective syndrome.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
12/22. MRI findings in the parents and siblings of specifically language-impaired boys.Four families that include a specifically language-impaired (SLI) boy were studied to test the hypothesis that developmental language disorders are biologically transmittable. A majority of the parents of the SLI boys had experienced communication difficulty (i.e., difficulty with speech, language, or academic skills) as children. Evidence of communication difficulty was paired on an individual basis with neuroanatomical data obtained through quantitative analysis of magnetic resonance imaging scans. Atypical perisylvian asymmetries were documented in a majority of the parents and were frequently associated with a history of communication difficulty. Atypical perisylvian asymmetries and disordered language skills were also documented for siblings of SLI boys. These findings suggest that atypical perisylvian asymmetries reflect a transmittable, biological factor that places some families at risk for language impairment.- - - - - - - - - - ranking = 2.7633286550343keywords = speech (Clic here for more details about this article) |
13/22. Familial phonological disorders: four pedigrees.The pedigrees of 4 children with a severe phonological disorder demonstrating three generations of members with speech/language problems are presented. All 4 probands were female with two mothers, two fathers, and five out of six siblings affected. All pedigrees contained family members with dyslexia and learning disabilities as well as speech disorders. family members varied in the type of speech problems that they demonstrated and the severity of their disorder, thus suggesting variable expressivity and incomplete penetrance. An autosomal dominant mode, a multifactorial-polygenic model, and a sex-specific threshold model for expression are discussed.- - - - - - - - - - ranking = 8.2899859651028keywords = speech (Clic here for more details about this article) |
14/22. Metabolic correlates of learning disability.To summarize, the neuropsychologic findings in MSUD and MMA children in both groups demonstrated deficits in cognitive/language areas, but interesting individual differences existed. For example, a marked contrast in abilities existed between NT and GV, even taking into account the age difference between these children with MSUD. While NT's general intellectual functioning was within the low-average range, GV was severely impaired. In more specific areas of cognitive functioning, NT had an uneven performance profile with areas of strengths and weaknesses, while GV evidenced a flat profile with significant impairment in all areas. NT demonstrated mild delay in visual-spatial processing, articulation development, motor-speech abilities, and selective attention and concentration; however, his language and psychosocial development were within the broad range of normality. In contrast, GV exhibited severe dysfunction in speech, language, and perceptual/conceptual development. The three children with MMA also exhibited a range of disabilities in cognitive functioning. TJ and SH contrasted in the degree of developmental delay across language and cognitive domains. Both were distractible and exhibited very short attention spans but there was a measurable difference in the extent of their speech, language, visual-spatial development. CH was markedly delayed in all areas of cognitive development, which is consistent with global retardation. The severity of her disease and its probable impact on brain development were undoubtedly related to her intellectual impairment. These datasets illustrate the challenges we face, because within each group we see a range of impairment. Nevertheless, we are optimistic that our efforts to relate metabolic, anatomic and neuropsychologic findings for such children will lead to a better understanding of these disorders. We are hopeful that this will result in new advances in diagnosis and intervention, which will ultimately improve the prognosis for cognitive development. In summary, a multidisciplinary center has been established at UCSD to study the neurologic basis of disorders of language, learning and behavior in infants and children. The center draws together a group of researchers from many fields including neurology, biochemistry, pediatrics, cognitive and developmental neuropsychology, psycholinguistics, neurophysiology and communicative disorders. Because of the diverse interests and expertise of our group, it is hoped to forge a synthesis of the behavioral and neurosciences to study populations of children with a variety of neurologic, metabolic, and language/learning disorders. Disorders currently under study include Lesch-Nyhan disease, oculocutaneous tyrosinemia, propionic acidemia, carnitine palmityl transferase deficiency, Schwachman-diamond syndrome, histidinemia, hartnup disease, citrullinemia, galactosemia, maple syrup urine disease, and methylmalonic acidemia.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 8.2899859651028keywords = speech (Clic here for more details about this article) |
15/22. Distinguishing auditory and speech-specific perceptual deficits.The ability to discriminate speech and nonspeech auditory stimuli was tested in a learning disabled child. The perception of speech stimuli was normal when the stimuli were presented in quiet but below normal when the stimuli were presented in noise. Although the perception of pure tone stimuli and environmental sounds was normal both in quiet and noise, the perception of nonspeech stimuli with rapid changes in acoustic information was impaired in noise. These findings illustrate the importance of relating performance for speech and complex nonspeech stimuli in investigating the basis of speech perceptual deficits. Whereas abnormal performance for speech stimuli coupled with normal performance for complex nonspeech stimuli argues for the existence of specialized speech processing mechanisms, abnormal performance for both types of stimuli, as was found in the present subject, argues for the existence of more generalized auditory processing mechanisms.- - - - - - - - - - ranking = 40.18660117048keywords = speech, perception (Clic here for more details about this article) |
16/22. Abnormal carotid arteries in the velocardiofacial syndrome: a report of three cases.Internal carotid arteries of unusual size and tortuosity were found before or at the time of pharyngeal flap surgery in three children who had the velocardiofacial syndrome with velopharyngeal insufficiency. In two cases, medial displacement of the arteries prevented surgery, and in the other, hypernasality persisted because only a narrow, asymmetrical flap could be raised. Medial displacement of the internal carotid arteries inhibits surgical treatment of velopharyngeal insufficiency, necessitating treatment with a prosthetic speech device in such children. Since displacement and tortuosity may be associated findings in the velocardiofacial syndrome, the exact location of the internal carotids should be ascertained when pharyngeal flap surgery is planned.- - - - - - - - - - ranking = 2.7633286550343keywords = speech (Clic here for more details about this article) |
17/22. Developmental learning disabilities of the right hemisphere. Emotional, interpersonal, and cognitive components.In 14 patients, we encountered a behavioral syndrome that begins in early life and is characterized by emotional and interpersonal difficulties, shyness, visuospatial disturbances, and inadequate paralinguistic communicative abilities. All 14 patients had at least average intellectual capacity, but each had demonstrated some academic failure, particularly in arithmetic. Examination revealed neurologic and neuropsychological signs consistent with right-hemisphere dysfunction. Most of the patients avoided eye contact and lacked the gestures and prosody that normally accompany and accentuate speech. Although many of them could not convey their feelings, there was no evidence that they were unable to experience affect. Eight of them reported a family history of similar symptoms. Just as developmental involvement of the left hemisphere may produce dyslexia, damage to the right hemisphere suffered early in life, or inherited, may lead to chronic emotional difficulties, a disturbance of interpersonal skills, and poor visuospatial ability.- - - - - - - - - - ranking = 2.7633286550343keywords = speech (Clic here for more details about this article) |
18/22. Acquired learning disability for reading after left temporal lobe damage in childhood.A 6-year-old boy developed hemorrhage in the left temporal lobe and subsequently underwent left temporal lobectomy. Afterwards, he was completely unable to learn to read or write. psychological tests showed impairment in verbal learning and memory, with right ear and right visual field superiority in verbal perception. A Wada test showed no aphasia after right carotid injection. We conclude that damage to the speech-dominant temporal lobe resulted in the inability to learn to read or write. Despite the lesion, speech remained strongly lateralized to the left hemisphere, accounting for the right-sided advantage in verbal perception. Some patients with developmental dyslexia may have dysfunction of a strongly dominant left hemisphere rather than a delay or incompleteness of language lateralization.- - - - - - - - - - ranking = 6.5266573100685keywords = speech, perception (Clic here for more details about this article) |
19/22. Velocardiofacial syndrome: learning difficulties and intervention.Velocardiofacial syndrome, delineated in 1978 by Shprintzen, is a multiple genetic disorder, characterised primarily by cleft palate, cardiovascular anomalies, typical facies, and learning disorders. This syndrome with its recurrent pattern of anomalies has significant implications for education as it is linked to a variety of educationally relevant problems including learning difficulties, behaviour disorders, and speech and language deficits. While clinical research advances and new findings in cytogenetics enable early diagnosis and genetic counselling, ramifications of the syndrome in the field of education are still unknown. Learning difficulties should not be viewed solely as a pathological condition and with data on success of intervention, parents and teachers can be apprised of the information. This paper discusses academic characteristics and the effects of intervention with emphasis on interactive computer based instruction on the development of reading, language, spelling, and numeracy skills. Positive effects of computer based instruction on students' self-esteem, motivation, and competence in computer operational skills were also observed and noted. We are encouraged by the enthusiasms of the students for the system, the development of interest in reading, and the transfer of remedial instruction to classroom performance. Comparison of pretest and post-test results indicated significant improvement in reading ability as measured using Neale Analysis. This improvement is in accordance with results obtained using computer testing of specific subskills in reading/language and mathematics.- - - - - - - - - - ranking = 2.7633286550343keywords = speech (Clic here for more details about this article) |
20/22. Fetal valproate syndrome: clinical and neuro-developmental features in two sibling pairs.The clinical and neurodevelopmental features are presented of four children--two sibling pairs--who were exposed in utero to valproic acid. One of each pair of children presented for diagnosis and assessment of developmental delay; the other sibling was examined at a later date. Three of the children were globally developmentally delayed with marked speech disability, and had dysmorphic features consistent with fetal valproate syndrome. One also had features of infantile autism. The fourth child had some of the dysmorphic features connected with fetal valproate syndrome, but had normal intellect, with his verbal ability being significantly below his non-verbal ability. He currently attends a school for learning-disabled children.- - - - - - - - - - ranking = 2.7633286550343keywords = speech (Clic here for more details about this article) |
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