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The Colorado Newborn Hearing Screening Project: effects on speech and language development for children with hearing loss.

OBJECTIVE: To compare the speech and language development of children with bilateral hearing loss and normal cognition who were born in hospitals with universal newborn hearing screening to that of their peers who were born in hospitals without this screening program. STUDY DESIGN: Subjects for the major analyses are 50 Colorado children (25 matched pairs) from 9 to 61 months old who are participants in a study of the development of children birth to six with bilateral hearing loss. Analyses included parametric dependent t-tests and analysis of covariance, nonparametric chi-squared and Wilcoxon signed rank tests, descriptive statistics and odds ratios. RESULTS: Newborn screening programs for hearing loss are positively related to scores in expressive and receptive language (p < 0.001) and vocabulary production (p < 0.001) on standardized inventories; speech intelligibility (p = 0.015) from independent ratings; number of different simple consonants (p < 0.01) and consonant blends (p = 0.026) from phonological transcripts; and total number of intelligible words (p < 0.01) and number of different words produced (p < 0.01) from computer analysis of videotaped language samples. CONCLUSION: Hospital-based newborn hearing screening programs are positively related to language and speech performance for children in early intervention programs who are deaf and hard of hearing.

Child Development↗

Early-onset schizophrenia as a progressive-deteriorating developmental disorder: evidence from child psychiatry.

The developmental perspective as reflected by investigations of childhood and early-onset schizophrenia has become a major research area during recent years and contributed much to the understanding of schizophrenia at all ages. This paper reviews clinical features, neurobiological and neuropsychological findings in childhood and adolescent onset schizophrenia including some results of studies of the author on age at onset, premorbid symptoms, treatment and course. Childhood-onset schizophrenia is a rare disorder with a prevalence of one child in 10,000 before the age of 12 and a remarkable increase around puberty and early adolescence. Developmental events and precursors of schizophrenia cover a wide range of dysfunctions and disturbances including elevated rates of soft neurological signs and birth complications, slow habituation and high baseline autonomic activity, high rate of developmental disorders of speech and/or language and overall and specific cognitive deficits. Brain morphological studies and intelligence testing as well as investigations of the course provide evidence of deterioration. Therefore, early-onset schizophrenia can be understood as a progressive-deteriorating developmental disorder.

Adolescent↗

[Pseudomigraine with transient neurological deficits and cerebrospinal fluid lymphocytosis].

INTRODUCTION: Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis is a rare syndrome resolving within less than 3 months. CASE REPORT: A young 17-year-old woman without previous medical history was admitted to the hospital because of right motor weakness and language disturbances. The symptoms resolved in a few hours and were followed by severe left headaches with important vegetative signs. Several similar episodes were noted in the previous 10 days. Cranial MRI was normal. EEG showed important slowing of the cerebral electrogenesis. More than 250 lymphocytic cells were found at CSF analysis. Outcome was spontaneously favorable, without similar symptoms after 6-month follow-up. CONCLUSION: Pseudomigraine with lymphocytic pleocytosis seems to be a particular syndrome of unknown origin. This is an elimination diagnosis, generally with a benign course.

Acyclovir↗

Selected herbal hazards.

One of the most important considerations in treating herbal ingestions is product quality assurance. Although most herbal companies are reputable, there are numerous reports of adulterated products (addition of substances not noted on the label). This has been particularly true of Chinese herbal products, which frequently contain pharmaceutical agents. Plant identification errors occur, and entire batches of product have been mixed using the wrong herb. In some cases, labels are written in a foreign language or only contain directions for mixing, making interpretation difficult. In cases where a known ingestion produces unexpected clinical signs, the potential for adulteration or other errors should be considered. When a product is not standardized, a consumer cannot be sure what dose of active constituents has been used or how bioavailable the product may be. Standardization also provides assurance that the actual herb is in the product [26]. Clients who use herbal products should be advised to treat them as a medication and to keep them away from pets. Specifically, ask clients if they take or use any natural or herbal products. Many people do not consider these substances drugs or assume, "If natural, it is harmless." Clients should be encouraged to learn about the herbal and neutraceutical products they are taking or giving their pets. Owners need to discuss the proper use of herbal products in pets with their veterinarian. Clients can be encouraged to discuss alternative therapies by discussing a pet's diagnosis and suggested treatments thoroughly. Discuss the client's expectations and opinions of alternative and conventional medicine. Issues of safety and efficacy must be fully explained to clients. Clients should be encouraged to report potential adverse reactions or to discuss different routes of therapy if a pet's medical condition is not improving. Clients who want to use alternative medical treatments should obtain a thorough medical workup so as to make a correct diagnosis and be referred to a veterinarian trained in alternative medicine. In choosing an alternative medicine practitioner, the same criteria would be used as for any other specialist: education, training, and professionalism [14].

Animals↗

Validation and application of verbal autopsies in a rural area of South Africa.

OBJECTIVE: To validate the causes of death determined with a single verbal autopsy instrument covering all age groups in the Agincourt subdistrict of rural South Africa. METHODS: Verbal autopsies (VAs) were conducted on all deaths recorded during annual demographic and health surveillance over a 3-year period (1992-95) in a population of about 63 000 people. Trained fieldworkers elicited signs and symptoms of the terminal illness from a close caregiver, using a comprehensive questionnaire written in the local language. Questionnaires were assessed blind by three clinicians who assigned a probable cause of death using a stepwise consensus process. Validation involved comparison of VA diagnoses with hospital reference diagnoses obtained for those who died in a district hospital; and calculation of sensitivity, specificity and positive predictive value (PPV) for children under 5 years, and adults 15 years and older. RESULTS: A total of 127 hospital diagnoses satisfied the criteria for inclusion as reference diagnoses. For communicable diseases, sensitivity of VA diagnoses among children was 69%, specificity 96%, and PPV 90%; among adults the values were 89, 93 and 76%. Lower values were found for non-communicable diseases: 75, 91 and 86% among children; and 64, 50 and 80% among adults. Most misclassification occurred within the category itself. For deaths due to accidents or violence, sensitivity was 100%, specificity 97%, and PPV 80% among children; and 75, 98 and 60% among adults. Since causes of death were largely age-specific, few differences in sensitivity, specificity and PPV were found for adults and children. The frequency distribution of causes of death based on VAs closely approximated that of the hospital records used for validation. CONCLUSION: VA findings need to be validated before they can be applied to district health planning. In Agincourt, a single verbal autopsy instrument provided a reasonable estimate of the frequency of causes of death among adults and children. Findings can be reliably used to inform local health planning and evaluation.

Adolescent↗

A CLN2 gene nonsense mutation is associated with severe caudate atrophy and dystonia in LINCL.

Clinical features and results of the blood DNA analysis are reported of a child affected with a distinct phenotype of the late infantile form of neuronal ceroid-lipofuscinosis (LINCL). He was affected by microcephaly and hypotonia since the fourth month of life; acquisition of motor and language abilities was severely impaired, and a disorder of communication with stereotyped movements followed. By age four, he developed signs and symptoms of progressive myoclonic encephalopathy along with motor and cognitive deterioration. Extrapyramidal signs were associated with neuroradiological findings of marked atrophy of the caudate nucleus. Specific curvilinear bodies were observed in blood lymphocytes and skin biopsy. Homozygous, nonsense mutation in the CLN2 gene was found giving origin to an Arg208stop, which produces an early transcription termination with loss of translation of about 50% of the gene product. Any relationship between the severe clinical features of our patient and the homozygous mutation here reported must be investigated on a larger number of LINCL patients bearing the same mutation.

Atrophy↗

Clinical caveats on medical assessment and treatment of pain after TBI.

The diagnosis and management of pain in the patient with traumatic brain injury (TBI) can be difficult in light of the limitations imposed by the cognitive, language, and behavioral deficits. With patients in the acute rehabilitation setting, one must be vigilant for the often subtle signs and symptoms of pain. Causes more commonly seen in the population with TBI as a consequence of the injury itself include dysautonomia, neuropathic pain, spasticity, and heterotopic ossification. Headaches may be a consequence of TBI or associated with it for other reasons. Sources of pain associated with TBI include deep venous thrombosis and others. The reader is reminded that patients with TBI are subject to all the causes of pain that affect the general population.

Autonomic Nervous System Diseases↗

Diagnosis of patients with chronic heart failure in primary care: usefulness of history, examination, and investigations.

Chronic heart failure is a common clinical syndrome that may have different causes. Its incidence and prevalence are predicted to rise substantially over the next 10 years. There are therefore major consequences for resource provision, especially in primary care, where most patients are managed. Chronic heart failure is a serious condition with high morbidity and mortality. There is good evidence to show that treatment with angiotensin-converting enzyme (ACE) inhibitors in patients with left ventricular systolic dysfunction improves symptoms and signs, slows progression of heart failure, reduces hospitalisation rates, and improves survival. Despite this evidence, primary care studies show that patients with heart failure are incorrectly diagnosed and inadequately treated. Most patients present in general practice, and because effective treatment relies on a correct diagnosis, this is a key step in the appropriate management of heart failure. The aim of this paper is to review the evidence about the usefulness of signs, symptoms, and investigations in diagnosing heart failure in primary care. To identify relevant studies for this review, four strategies were used: a MEDLINE search from 1993 to January 1998 using the diagnosis search filter; a MEDLINE search from 1993 to January 1998 using the guideline search filter to locate published heart failure guidelines; a search for review articles in the Cochrane Library; and a check of references in the studies identified. The search terms included MeSH terms and the keywords 'heart failure' and 'diagnosis'. All searches were limited to humans and English language articles. Studies were included in this review on the basis of quality and relevance to primary care. The review shows that symptoms and signs are important because they alert clinicians to the possibility of heart failure as a diagnosis. However, they are not sufficiently specific for confirming left ventricular systolic dysfunction. From the evidence available, a patient with suspected heart failure must have objective tests to confirm the diagnosis. These should include an electrocardiogram and, ideally, an echocardiogram. Further research is also needed on the usefulness of signs and symptoms in primary care, as most studies of heart failure have been conducted in secondary care.

Electrocardiography↗

Propylthiouracil hepatotoxicity: two pediatric cases and review of the literature.

We have observed isolated hepatotoxicity in two children treated with propylthiouracil (PTU) for hyperthyroidism. Neither patient had risk factors for or clinical evidence of preexisting liver disease. In one patient the drug was promptly discontinued when signs of liver disease were noted. This patient quickly recovered. The second patient continued to receive PTU for several days after developing symptoms. Her illness progressed to fulminant hepatic failure with encephalopathy, and she died. These are the third and fourth pediatric cases reported, and there have been 10 cases reported in adults in the English language literature. Thirteen of the 14 patients are female. The literature regarding all these patients is reviewed. Propylthiouracil may cause lethal hepatic damage. This drug should be discontinued immediately if signs or symptoms of hepatic injury are detected.

Adolescent↗

Association between scalp hair-whorl direction and hemispheric language dominance.

Asymmetry is a common phenomenon in higher organisms. In humans, the cortical representation of language exhibits a high degree of asymmetry with a prevalence of about 90% of left hemispheric dominance, the underlying mechanisms of which are largely unknown. Another sign that exhibits a form of lateralization is the scalp hair-whorl direction, which is either clockwise or anti-clockwise. The scalp hair-whorl develops from the same germ layer as the nervous system, the ectoderm, between the 10th and 16th week in utero and has been shown to be associated with various neurodevelopmental disorders. Here, we use an established fMRI paradigm to examine the association of a solely biological marker of asymmetry, hair-whorl direction and language lateralization. We show that the mechanism that influences hair-whorl direction and handedness [Klar, A.J.S., 2003. Human handedness and scalp hair-whorl direction develop from a common genetic mechanism. Genetics 1651, 269-276.] also affects cerebral language dominance.

Adult↗

Scopolamine effects on memory, language, visuospatial praxis and psychomotor speed.

Scopolamine hydrobromide was administered by subcutaneous injection to 30 young subjects in a dose of 0.22 mg/70 kg, 0.43 mg/70 kg, or 0.65 mg/70 kg. Treatment effects were compared to placebo on an extensive cognitive assessment battery. Almost all tests in the battery had been previously administered to Alzheimer's disease patients and nondemented elderly subjects. Scopolamine produced deficits on tests of verbal recall, visuospatial recall, visual recognition memory, visuospatial praxis, visuoperceptual function, and psychomotor speed. Immediate memory, language function, object sorting, and frequency of intrusion errors were unaffected. The low dose of scopolamine produced some peripheral anticholinergic signs but did not affect the cognitive measures. The results support the conclusion reached in previous studies that the cognitive profile of scopolamine-injected young subjects is more similar to that of the nondemented elderly than to that of Alzheimer's disease patients.

Adolescent↗

Pure post-stroke cerebellar cognitive affective syndrome: a case report.

Cerebellar pathology commonly shows important motor signs and less evident cognitive dysfunction. The 'cerebellar cognitive affective syndrome' is characterised by impairment on executive function, spatial cognition, language and behaviour. We report the case of a man with acute onset of transitory motor features and severe mental disorders. Cranial CT and brain MRI revealed extended cerebellar lesions. Neuropsychological assessment disclosed deficits of attention, executive function and memory. Auditory event-related potentials showed abnormal P300. These data suggest a pure "cerebellar cognitive affective syndrome"and strengthen the hypothesis of cerebellar cognitive function modulation.

Aged↗

Splenic complications in malaria: report of two cases from Turkey.

Malaria is still a major health problem in Turkey, where Plasmodium vivax malaria is endemic. Spontaneous rupture of the spleen is an important and life-threatening complication and occurs in up to an estimated 2 % of cases. Hence the small number of case reports suggests under-reporting or underdiagnosis. Review articles have reported only 18 malaria cases with spontaneous splenic rupture in the English language literature since 1960. Two cases of P. vivax malaria with splenic complications are reported here. One of them showed signs and symptoms of acute abdominal pain, then splenic rupture occurred.

Adult↗

Status epilepticus manifesting as reversible Wernicke's aphasia.

Ictal aphasia in adults is a rare phenomenon, with the majority of reported cases showing a nonfluent Broca's or mixed aphasic speech pattern associated with disturbances of level of consciousness. There is usually only one prolonged episode of aphasia with evidence of lateralized neurological findings and structural pathology on CAT scan. We describe a patient with intermittent episodes over a 10-year period of a fluent Wernicke's aphasia associated with paroxysmal posterior temporoparietal spike-wave activity on the EEG. Interictally, the patient was clear of neurologic signs and symptoms. These episodes were repeatedly misdiagnosed as psychotic breaks and treated with antipsychotic medication and psychiatric hospitalization. Unless language performance is assessed, these cases of focal epilepsy may be diagnosed as schizophrenic "word-salad," leading to delay of appropriate treatment.

Aphasia↗

Familial presenile dementia with bitemporal atrophy.

This study describes the clinical, neuropsychological, neuroimaging and genetic characteristics in two generations of a Swedish family affected by presenile dementia. The pedigree includes 5 cases (mother and 4 of 5 children) of progressive dementia with onset between 54 and 62 years. The clinical picture is characterized by insidious onset and progressive decline in episodic memory without spatial impairment or dyspraxia, followed by changes in personality and behaviour, with signs of disinhibition, irritability, impulsivity and loss of social awareness. Three siblings, examined after 10 years of duration, showed moderate language deficits but preserved spatial function and praxis. CT and MRI showed progressive bilateral temporal atrophy and moderate frontal white matter changes. Regional cerebral blood flow measurements showed hypoperfusion in temporal areas bilaterally. Quantitative EEG was normal within 5 years after symptom onset and thereafter showed a moderate increase in relative theta power. Sequencing of the tau gene (chromosome 17) revealed the previously described R406W mutation in exon 13 as a likely cause of the disease. This mutation was identified in all affected cases. The clinical picture of this family shows striking similarities not only to frontotemporal dementia but also to Alzheimer's disease.

Alzheimer Disease↗

Common speech problems encountered in general practice.

The authors consider speech and communication in the light of whole patient care and point out that defects may be signs and symptoms of underlying organic disease. They describe the four classifications of speech disorders-articulation, rhythm, voice and language, with an indication of the speech therapy required and duration of treatment. Special emphasis has been given to those speech problems which are seen by the family physician; these are usually of the articulation group. A short discussion of stuttering and aphasia is given. Emphasis is put on the direction of treatment by the physician and the use of well-qualified personnel as members of the rehabilitation team.

Aphasia↗

Psychoanalysis--a phenomenology of language.

It is often said that there is an unbridgeable gap between phenomenology and psychoanalysis. But this is only half the truth. Starting from Merleau-Ponty's proposal of an objectivistic and an idealistic divergence of Freud's enterprise the paper tries to reconstruct two possible movements: the objectivistic divergence of Husserl's enterprise as a phenomenological shift towards psychoanalysis and the idealistic divergence of Freud's enterprise as a psychoanalytical shift towards phenomenology. It is shown that this approach is possible on the field of language and semiotics where psychic life, as the essence of our subjectivity, may be rendered as a uniform phenomenon of an articulation in the world of signs. This thesis is elucidated at the end of the paper by discussing first (to the phenomenological side) a certain interpretation of the use of Husserl's reduction as a method of investigation in psychiatry and second (to the psychoanalytic side) the importance of Lacan's difference between repression and foreclosure for a psychopathological understanding of psychosis.

Humans↗

A prospective cohort study of childhood behavioral deviance and language abnormalities as predictors of adult schizophrenia.

Language and behavioral deviance in early childhood in preschizophrenia individuals suggests that the pathologic processes predisposing to schizophrenia are present from early in life. However, the etiologic antecedents of such impairments, and the degree to which they predict adult schizophrenia, have not been conclusively demonstrated. To address this, we examined language and behavioral predictors of adult psychiatric outcome in a population cohort (72 individuals with schizophrenia or schizoaffective disorder, 63 of their unaffected siblings, and 7,941 with no diagnosis) evaluated prospectively with behavioral examinations and a speech and language evaluation at 8 months, 4 years, and 7 years of age. Psychiatric outcome was ascertained via adult treatment contacts, and diagnoses were made by chart review according to DSM-IV criteria. Social maladjustment at age 7 was found to predict adult schizophrenia, and focal deviant behaviors (e.g., echolalia, meaningless laughter) at ages 4 and 7 were significantly associated with both schizophrenia and sibling status. Unintelligible speech at age 7 was a highly significant predictor of adult schizophrenia (odds ratio = 12.7), and poor expressive language ability predicted both schizophrenia and unaffected sibling outcome. Early behavioral and language dysfunction did not differentially characterize preschizophrenia subjects with a history of fetal hypoxia or an early age of first treatment contact. Given that unaffected siblings show similar signs of deviance, such problems may indicate genotypic susceptibility to the disorder, or shared environmental influences, or both.

Adult↗