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[Conduction aphasia. Neuropsychologic study of a patient].

We report a patient with clinical features consistent with conduction aphasia. The patient had dilated cardiomyopathy, and as a complication, had cerebral infarction one year before neuropsychiatric evaluation. His language was fluid, paraphasic, with a "phonetic approximation behavior" in an attempt to pronounce the adequate word. Comprenssion was normal and repetition difficult, with numerous paraphasias. Number repetition was particularly affected. Reading and writing had the same features as spontaneous language. In association with the language disorder, he had "aphasic" acalculia, but other signs of left parietal involvement were absent. Magnetic resonance images showed a low parietal small chronic ischemic area, which also involved the left infrasilvian region, both in the cortex and in the white matter, presumably in the arcuate bundle. The differential diagnostic difficulties of this condition are discussed from a behavioral standpoint.

Aphasia↗

[Late declaration of pregnancy: an indicator of psychosocial vulnerability of the family].

Social, economical and family characteristics were studied in 90 women who notified their pregnancy beyond the French legal recommended limit of 3 months (LNP). The development of the children born from these pregnancies was also studied. A group of women notifying their pregnancy within the legal limit of 3 months served as a control. The data were collected at home by nurses from the Service de Protection Maternelle et Infantile visiting the family at the time of notification of the pregnancy and at ages 4, 12 and 36 months. The results showed frequent psychological, economical and social disabilities of women with LNP, presenting high rates of unemployment, low income, unusual ages of pregnancy (44% before 20 years and after 35 years), absence of father, consideration of voluntary interruption of pregnancy. Their offspring had a frequent unfavourable family environment, but their development did not show significant differences at 3 years of age as compared with children of the control group: however they presented signs suggesting a greater vulnerability, and especially frequent language and behaviour disturbances. From these data LNP appears as an indicator of psycho-social vulnerability of the family. Therefore LNP should lead to a systematic visit of the family at home by a social worker in order to assess its degree of psycho-social vulnerability and to provide, when necessary, preventive action directed towards the protection of the child development.

Child↗

[Ibn al-Nefis--discoverer of pulmonary blood circulation].

Medicine was developed to an astonishingly high degree by Muslims. During at least five centuries Muslim physicians translated medical books from Greek and other languages, and with their descriptions of the clinical signs of many illnesses, many of the ideas and concepts contained in medical encyclopedias (like al-Qanun written by Avicenna), passed it to the West and East. One of the greatest Muslim physician was undoubtedly Ibn al-Nefis (d.1288), who first accurately described the circulation of blood through the lung (in XVI century the credit went to Michel Servet), and body (in 1628 the credit went to W. Harvey), and author of the famous Excerpt of al-Qanun, written by Avicenna.

History, Medieval↗

Voice abnormalities in short stature syndromes.

Patients with short stature have a high incidence of voice and laryngeal abnormalities. In sixteen patients with short stature of various etiologies, these abnormalities appeared to be unrelated to hearing loss or other otolaryngologic problems. However, in many patients they were associated with typical symptoms and signs of voice abuse. Physicians and speech-language pathologists caring for patients with short stature should be alert for voice problems, and should consider instituting early voice education, diagnosis and treatment.

Adolescent↗

[Mass screening of deafness in neonates using otoacoustic emissions].

There is no clinical sign of hearing loss in the infant. Defective language acquisition and altered voice quality always occur late making early diagnosis essential for therapeutic management of deaf children. Subjective assessment of behavioral is highly dependent on the experience of the examinator and cannot be used as an effective routine method. Electrophysiological recordings of otoacoustic emissions have the advantage of allowing objective measurement. Otoacoustic emissions originate in the inner ear. A quantity of energy is transmitted via the tympanic membrane which acts as a microphone, allowing recordings in the external auditory canal. In certain infants these emissions are spontaneous and can be used to detect certain types of hearing loss. Stimulation is required however for screening tests. Recording provoked otoacoustic emissions is simple. A small probe in placed in the external auditory canal. This probe carries two microphones, one which emits clicks recorded by the other captor microphone. The examination lasts approximately 15 minutes, including the time needed to explain the procedure to the mother. There are two possible results. The presence of recorded otoacoustic emissions confirms that the inner ear is normal. If the absence of emissions is confirmed at a second examination, an evoked potential recording is required to identify the hearing loss. This safe, simple and reliable method for detecting hearing loss in infants is recommended for screening programmes during the neonatal period.

Deafness↗

Potential language and attentional networks revealed through factor analysis of rCBF data measured with SPECT.

We used changes in regional cerebral blood flow (rCBF) to disclose regions involved in central auditory and language processing in the normal brain. rCBF was quantified with a fast-rotating, single-photon emission computerized tomograph (SPECT) and inhalation of 133Xe. rCBF data were obtained simultaneously from parallel, transverse slices of the brain. The lower slice was positioned to include both Broca's and Wernicke's areas. The upper slice included regions generally regarded by neurobehaviorists as less related to primary auditory or linguistic functions. We presented three types of auditory stimuli to ten healthy, young volunteers: (a) diotically presented Danish speech, (b) dichotic word stimulation, and (c) white noise. Wilcoxon's signed ranks sum test revealed increased rCBF in language-related areas of cortex, viz., Wernicke's area and its right-sided homologous area as well as in Broca's area (left hemisphere), when subjects listened to narrative speech, compared to white noise (baseline). No significant rCBF differences were detected with this test during dichotic stimulation vs. white noise. A more sophisticated statistical method (factor analysis) disclosed patterns of functionally intercorrelated regions. The factor analysis reduced the highly intercorrelated rCBF measures from 28 regions of interest to a set of three independent factors. These factors accounted for 77% of the total variation in rCBF values. These three factors appeared to represent statistical analogues of independent brain networks involved in (I) auditory/linguistic, (II) attentional, and (III) visual imaging activity.

Acoustic Stimulation↗

Meaning matters: a clinician's/student's guide to general sign theory and its applicability in clinical settings.

UNLABELLED: The pragmatic mapping process and its variants have proven effective in second language learning and teaching. The goal of this paper is to show that the same process applies in teaching and intervention with disordered populations. A secondary goal, ultimately more important, is to give clinicians, teachers, and other educators a tool-kit, or a framework, from which they can evaluate and implement interventions. What is offered is an introduction to a general theory of signs and some examples of how it can be applied in treating communication disorders. LEARNING OUTCOMES: (1) Readers will be able to relate the three theoretical consistency requirements to language teaching and intervention. (2) Readers will be introduced to a general theory of signs that provides a basis for evaluating and implementing interventions.

Humans↗

Automating a severity score guideline for community-acquired pneumonia employing medical language processing of discharge summaries.

Obtaining encoded variables is often a key obstacle to automating clinical guidelines. Frequently the pertinent information occurs as text in patient reports, but text is inadequate for the task. This paper describes a retrospective study that automates determination of severity classes for patients with community-acquired pneumonia (i.e. classifies patients into risk classes 1-5), a common and costly clinical problem. Most of the variables for the automated application were obtained by writing queries based on output generated by MedLEE1, a natural language processor that encodes clinical information in text. Comorbidities, vital signs, and symptoms from discharge summaries as well as information from chest x-ray reports were used. The results were very good because when compared with a reference standard obtained manually by an independent expert, the automated application demonstrated an accuracy, sensitivity, and specificity of 93%, 92%, and 93% respectively for processing discharge summaries, and 96%, 87%, and 98% respectively for chest x-rays. The accuracy for vital sign values was 85%, and the accuracy for determining the exact risk class was 80%. The remaining 20% that did not match exactly differed by only one class.

Community-Acquired Infections↗

Acquired aphasia in childhood with seizure disorder: a heterogeneous syndrome.

The authors report six children with acquired aphasia of unknown etiology. The clinical picture was clearly different from that seen in the usual childhood aphasias and resemble other cases initially reported as "syndrome of acquired aphasia with convulsive disorder". All had associated paroxysmal EEG abnormalities, and 5 have had clinical seizures. The language disorder has improved or remained stationary and no other neurological signs have developed. Our review of the literature and the study of our personal cases show no uniform clinical picture in these children. Three different clinical patterns seem to emerge. The first group show rapid onset and recovery of aphasia, frequent fluctuations in the severity of the language deficit typical of so-called epileptic aphasia. These children appear to have a better prognosis. The second group show worsening of the aphasic deficit after repeated seizures or episodes of aphasia. In the third group progressive deficit in language comprehension (auditory agnosia) with a variable degree of recovery and rare or no clinical seizures. The possible significance of the EEG abnormalities has been discussed and the importance of the aphasia on general behavior and the problems of differential diagnosis have been stressed.

Adult↗

Role of registered dietitians in dysphagia screening.

OBJECTIVE: To examine the ability of registered dietitians to identify patients at risk for dysphagia and make appropriate diet/feeding recommendations in comparison with the speech-language pathologist, and to determine screening criteria for the registered dietitian to use for prediction of dysphagia risk. DESIGN: The dietitian and speech-language pathologist performed dysphagia screening on subjects independently through questioning and/or mealtime observation to identify signs and symptoms of dysphagia. Presence of dysphagia risk and diet/feeding recommendations were determined and results from the dietitian and speech-language pathologist were compared. SUBJECTS/SETTING: Thirty-four patients admitted during a 2-month period to a neuroscience unit at an urban teaching hospital were analyzed prospectively. STATISTICAL ANALYSES PERFORMED: kappa Statistics were used to assess agreement between the dietitian and speech-language pathologist. A kappa level of less than 0.4 indicated weak agreement, 0.4 to 0.7 indicated moderate agreement, and greater than 0.7 indicated strong agreement. Logistic regression methods were used to evaluate screening criteria as potential predictors of dysphagia risk. RESULTS: Moderate agreement (0.61) was found between the dietitian and speech-language pathologist in determination of dysphagia risk. The dietitian predicted the ability of the patient to consume an oral diet with strong agreement with the speech-language pathologist (1.0); various diet consistencies with moderate agreement (0.61); and the need for liquid restrictions with strong agreement (1.0). The most significant screening variables for prediction of dysphagia risk (P < .05) were age (P = .018), history of dysphagia (P = .042), difficulty swallowing solids (P = .0007), observed facial weakness (P < .0001), and a change in voice quality (P = .0007). Self-reported screening variables significantly related to dysphagia risk included drooling of liquids (P = .0009) and solids (P = .0080), facial weakness (P = .0006), change in voice quality (P = .0010), and prolonged eating time (P = .0157). APPLICATIONS/CONCLUSIONS: Dietitians can effectively identify patients with dysphagia. Screening for dysphagia can be implemented as part of standard nutrition assessments and may aid in decreasing dysphagia-related complications.

Age Factors↗

[Clinical phenotypes of classic Rett syndrome].

INTRODUCTION: Rett syndrome (RS) is a progressive neurological disorder that is diagnosed by essential, supportive and exclusion clinical criteria, and development takes place in four stages. It has been shown to be caused by de novo mutations of a gene located in the long arm of the dominant X chromosome that codes for the methyl CpG binding protein (MECP2). It has been observed that girls with classic RS (CRS) present distinguishing nuances with respect to the age of onset of the different criteria and as regards the progression of the disorder. Taking the ability or failure to walk as a reference, we have established three phenotypes. METHOD: Phenotype I. Ambulant CRS, which corresponds to a permanent stage III, or a stage III that lasts a long time before going into stage IV. The loss of the ability to use the hands in a purposeful way takes place at the age of 25.6 months, social withdrawal at 25.4 months, language impairment at 20 months, stereotypic hand movements at 22.8 months and signs of spasticity appear around the age of 8-10 years. Phenotype II. Ambulant CRS. Transitory, which corresponds to an early stage IV-A. The first signs of abnormality appear around the age of 9-10 months. This is followed by the loss of the purposeful use of the hands towards the age of 23.4 months, social withdrawal around 21.4 months, language impairment at 20 months, stereotypic hand movements at 25.2 months and scoliosis, neuromotor disorders and trophic and vasomotor disorders at the age of 4-5 years. Phenotype III. Non ambulant CRS, which corresponds to stage IV-B. It begins with hypotonia towards the age of 5-6 months, loss of voluntary grasping at 17.8 months, social withdrawal at 18 months, language impairment at the age of 12 months, stereotypic hand movements at 13 months and early onset of motor, trophic and vasomotor disorders. Genetic studies were conducted in 12 girls and MECP2 gene mutations were found in 10 of them, belonging to the three different phenotypes. CONCLUSIONS: We have established three phenotypes in RS according to the ability to walk. If walking is not achieved or the ability is lost early on, speech loss, social withdrawal and the onset of stereotypic movements, motor, trophic and vasomotor disorders all progress more quickly. Mutations in the MECP2 gene have been found in the three phenotypes. In 16.6% the genotype was normal. Greater accuracy is required in the definition of cases of CRS in order to establish phenotype genotype correlations.

Child↗

Clonic perseveration following thalamofrontal disconnection: a distinctive movement disorder.

We describe four patients who developed asymmetrical, rhythmic, stereotyped, and repetitive movements of the upper and lower limbs hours to days after infarction that involved the thalamus and/or basal ganglia. The movements appeared to occur spontaneously and were initially labeled as focal motor seizures, ballism, or tremor; they could however, be induced by passive movement of the limbs. The movements most commonly observed were scratching or rubbing movements of the hands that were of such persistence as to cause trauma to the skin; in the lower limbs, the heel was run up and down the bed sheet, often until it bled. The movements were part of a syndrome characterised initially by a reduced level of consciousness and followed by aspontaneity, usually with mutism and frontal release signs. One patient who had relatively preserved cognition and language repeated words or phrases again and again when encouraged to speak, but had no difficulty changing responses appropriately to different cues. In drawing, he overwrote each figure but could change the figure on command. The distinctive movement disorder in these patients was due to clonic perseveration. We suggest that clonic perseveration results from disconnection of prefrontal cortico-basal ganglia-thalamo-cortical loops that are important for the termination of motor plans. Clonic perseveration should be recognised as a movement disorder following thalamic lesions.

Aged↗

Primary progressive aphasia: description of a clinical case with nine years of follow-up.

We describe the case of a woman with primary progressive aphasia who, over a period of nine years, has shown no signs of clinical deterioration and has only a symptomatic language disturbance. Neuropsychological follow-up has revealed progressive language impairment, with the integrity of praxis, visuoperceptive gnosia and short term visuospatial memory remaining intact; the only impairment was that revealed by long-term memory tests.

Aged↗

Pure word deafness due to left hemisphere damage.

We report the case of a 55 year-old right-handed man who presented with a long lasting pure word deafness following left thalamic bleeding. There was no sign of aphasia. The auditory deficit was specific for language, while recognition of music and environmental sounds was normal. CT, MRI and PET examinations showed that the lesion was anatomically and functionally confined to the left cerebral hemisphere, mainly the white matter of the temporal and parietal lobes. Wernicke's area was largely preserved. It is proposed that pure word deafness was consequent to the isolation of Wernicke's area from incoming auditory information due to the interruption both of the association fibers from the right auditory area traveling across the corpus callosum and of the left auditory radiations.

Brain Injuries↗

Hemihydranencephaly: case report and literature review.

Hydranencephaly is a severe brain condition characterized by complete or almost complete absence of cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. It has been ascribed to different causes (infections, irradiations, fetal anoxia, medications, twin-twin transfusion), all leading to vascular disruption. Hemihydranencephaly is an extremely rare condition in which the vascular anomaly is unilateral. We report on a patient who was suspected to have hydrocephalus in utero; a brain magnetic resonance imaging scan showed left-sided hydranencephaly with preservation of basal ganglia. The patient developed signs of right hemiparesis but notably has only mild language delay. The available literature on hemihydranencephaly is reviewed.

Brain↗