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PubMed1988

Diurnal rhythms in performance tests of school children with and without language disorders.

Time-of-day related changes on four tests used by speech therapists and four other performance tests, in addition to oral temperature, were documented in 16 school children (7-9 years of age). Six of them had language disorders and were receiving speech therapy. Children were synchronized with diurnal activity from around 0730 to around 2100 and nocturnal rest. For each child, at each test time point (e.g. 0900, 1100, 1530 and 1930) tests were performed three times, with two different speech therapists, in a random order, with only one session per day. Conventional methods (t-tested mean differences; ANOVA; correlation tests) were used for statistical analyses. Among 29 parameters (items) which were analyzed, only nine exhibited time-of-day related changes, mainly in speed to-perform measures. In most detected rhythms best performance occurred either at 1100 or at 1530 with no difference in subgroups except for the fastest performance of the sentence repetition test. With regard to the daily mean M, controls performed better than children with language disorders for the word (syllabic) repetition test (P less than 0.0004) but this was reversed for both computing and colouring skill tests (P less than 0.04 and less than 0.002). A difference related to sex (but not to language disorders) was observed in the Ms of speed in sign reproduction (P less than 0.0000) and sorting cards (P less than 0.01), with boys being faster than girls. In children, as in adults, time-of-day effects should be considered when the quantification of performance is desired.

Body Temperature↗
PubMedDate not supplied

[Subacute sclerosing panencephalitis: fulminant form].

INTRODUCTION: Subacute sclerosing panencephalitis (SSPE) has become less frequent as a consequence of massive anti measles vaccination. Early infection or immunological factors could increase the risk of its appearing and of evolved forms of the disease. CASE REPORTS: We describe the cases of four patients with fulminating forms of SSPE: a girl who had measles at the age of eight months; a male who, without actually suffering the clinical disease, came into contact with measles in the family and, despite having been vaccinated, presented SSPE 18 months after the contact; a boy aged 4 years and 4 months who had measles at the age of 6 months, and a boy of a similar age who had the illness when he was one year old. They all developed ataxia, with focal and generalised neurological signs, myoclonic and atonic seizures with rapid deterioration of language and the cognitive functions. In the four cases, the computerised tomography scans were normal, the electroencephalograms showed bilateral paroxysms and periods of recurrent bioelectrical attenuation. The magnetic resonance images of the four patients revealed disseminated hyperintense lesions, and one of the patients presented hyperintense lesions in the cervical spinal cord. The anti measles IgG titres were high in the cerebrospinal fluid. Anti convulsive drugs were ineffective. In the third and fourth patients, intrathecal intraventricular treatment with interferon did not modify the course of the disease and neurological deterioration was seen in the subsequent follow up of all the cases. CONCLUSION: As a consequence of vaccination against measles, SSPE has become less frequent. Infection of infants, prior to the immunisation stage, can induce SSPE with periods of latency that are shorter than usual and with a fast progression of the disease.

Child, Preschool↗
PubMed1993

Infections of implantable cardioverter defibrillators: approach to management.

Implantable cardioverter defibrillators are being used with increasing frequency for the treatment of life-threatening ventricular arrhythmias. Nevertheless, no guidelines exist for the management of infections of these devices. We report our experience with infections of these devices and review the English-language literature. In all cases, patients presented with local signs of generator infection; systemic signs of infection and bacteremia were often absent. Most infections are due to staphylococcus. Risk factors for the development of infection include placement of the device via median sternotomy during another cardiac surgical procedure, reoperation, and intercurrent infection at another site. Infections are most reliably treated with full explantation of the device and antibiotics. In rare cases, patients may respond to a combination of intravenous antibiotics and removal and replacement of only the generator.

Adult↗
PubMed1991

Neuronal activity in frontal speech area 44 of the human cerebral cortex during word recognition.

The activity of neurons was recorded from cortical speech area 44 of human patients with diagnostic and/or therapeutical depth electrodes while they were performing word recognition and object naming tasks. Some cells responded selectively to either semantic or phonological aspects of word processing with the same latency, others responded consecutively to both semantic and phonological features. The present finding provides the first demonstration of neuronal mechanisms in frontal language cortex and evidences that both consecutive and parallel processing of these speech signs occur in neuronal activity of this area.

Frontal Lobe↗
PubMed1991

Augmentative communication systems taught to cerebral-palsied children--a longitudinal study. III. Teaching practices and exposure to sign and symbol use in schools and homes.

A longitudinal study of augmentative communication training with 40 language-impaired, cerebral-palsied children examined the teaching practices adopted in schools, and the extent to which teachers and parents used the augmentative systems with the children. The study found limited exposure to sign and symbol training in formal teaching sessions and in other school settings, and teachers made relatively few attempts to foster spontaneous use of the systems and generalisation outside of formal training settings. Exposure to augmentative communication in the children's homes was equally limited, and a significant number of parents expressed reservations about the value of the systems for their children. These findings may at least partly account for the children's limited progress in sign and symbol acquisition and use over an 18-month period, which was documented in two earlier reports.

Attitude↗
PubMed2006

[Language and neurodevelopmental disorders. A review of their clinical characteristics].

INTRODUCTION: Language is a keystone in the normal social and cognitive development of any group of children and early, fitting interventions can largely reduce the repercussions that the deficit has in this area. The article analyses the definition of language as 'the result of a complex nervous activity that allows individuals to communicate mental states by the production of multi-modal signs that symbolise these states in accordance with a linguistic community's own convention'. Other language-related terms are also dealt with. DEVELOPMENT: Different classifications of language disorders are also discussed and we analyse its characteristics in different neurological disorders, such as motor disorders with a central origin, autistic spectrum, learning disorders, mental retardation, and attention deficit and disruptive behaviour disorders. CONCLUSIONS: Disorders affecting language clearly display semiological heterogeneity, and therefore it is advisable to take into account the classifications and terms related to it. It is also necessary to be familiar with the specific features of each alteration in the different neurological disorders so as to be able to reach an accurate diagnosis that allows the implementation of suitable lines of behaviour and interventions. Additionally, this will also allow timely measures to be taken in order to avoid later complications.

Adolescent↗
PubMed2001

[Behavioral problems in children with specific and pervasive developmental disorders, evaluated with the psychopathology assessment scale (AMDP)].

Children with specific developmental disorders of speech and language have an increased rate of psychiatric disorder compared to children developing normally. Children with early infantile autism show psychopathological symptoms in addition to those that are required for the diagnosis. These are often important in daily dealings with the children. As part of a larger study 4 groups of school age children (21 with normal development, 18 with an expressive language disorder, 21 with a receptive language disorder and 11 children with early infantile autism) were rated according to the psychopathological documentation for children (AMDP) within the assessment situation and according to reports of the behaviour outside. Several items were added to include symptoms that are not rated in the present documentation. Significant differences in the means were found between the group of children with autism and the control group. The children with language disorder showed a high variance which was interpreted as a sign for a heterogeneous group. Several of the added items improve the documentation of psychopathological findings in young school children.

Age Factors↗
PubMed2005

Crossed right hemisphere syndrome following left thalamic stroke.

In most right-handed people, language and motor functions are more reliant on systems of the left hemisphere while several non-linguistic visuo-spatial and attentional processes depend more on the right hemisphere. The rare exceptions to these rules provide important clues as to what functions co-lateralise, and are thus crucial for models of cerebral specialization. Here we report on the case of a patient, who, after a lesion restricted to the left thalamic region, showed signs normally associated with right hemispheric injury including motor impersistence, visuo-spatial dysfunction and poor comprehension of facial expression. Language abilities were spared and no signs of apraxia were present, in spite of his right hand, foot and eye preference, a pattern normally associated with conventional cerebral dominance. In spite of his other right hemispheric signs, the patient showed no signs of hemi-spatial neglect. The patient's pattern of spared and impaired abilities is compared and contrasted with other rare cases of crossed right hemisphere syndrome.

Dominance, Cerebral↗
PubMed1997

Sarcoidosis manifesting as acute appendicitis: report of a case.

PURPOSE: This report presents a patient who developed signs and symptoms of acute appendicitis because of sarcoid involvement of the appendix. METHODS: This is a retrospective case review with review of the English language literature. RESULTS: The patient underwent appendectomy for suspected acute appendicitis. Histologically the appendix had no signs of acute inflammation but did have noncaseating granulomas. The patient's abdominal pain resolved following appendectomy, and she has had no further similar pain in the two years since the operation. CONCLUSION: Patients with sarcoidosis may develop signs and symptoms of acute appendicitis without inflammation. Operative exploration should not be delayed in equivocal cases of right lower quadrant abdominal pain in patients with sarcoidosis.

Adult↗
PubMed1990

[Course of recovery from motor aphasia in four patients with localized lesions in the vicinity of Broca's area].

The neuropsychological signs and prognosis of patients with localized lesions in the vicinity of Broca's area of the left inferior frontal gyrus have been controversial. We studied periodic changes in the neuropsychological signs of four patients who had relatively localized lesions in the vicinity of Broca's area and manifested aphasia of the non-fluent type. The ages of the four patients at the onset were 62, 66, 30 and 37 years. The localized lesions in their brains were the result of brain infarctions revealed by CT scans, and included so-called Broca's center in the left inferior frontal gyrus along with adjacent brain tissue. The neuropsychological signs of the four patients were periodically assessed by means of the standard language test of aphasia (SLTA). In the early stage of aphasia, they had moderate difficulties in speaking to explain test materials or recall words and mild difficulties in reading aloud, repeating and writing words. One year later, most of their neuropsychological signs disappeared except for mild difficulties in speaking fluently and recalling words. It should be noted, however, that all four patients were able to return to their previous occupations one or two years later, because they had recovered from aphasia. Based on the results of this study, we conclude that the neuropsychological signs of Broca's aphasia caused by relatively localized lesions in the left inferior frontal gyrus are mild and largely disappear within one or two years.

Adult↗
PubMed2002

Developmental language disorder associated with polymicrogyria.

BACKGROUND: Subtle disorders of neuronal migration occur in the brains of some dyslexic patients who presented developmental language disorder (DLD) during early childhood. OBJECTIVE: To investigate a possible neuroanatomical substrate based on neuroimaging evaluation in children with DLD. METHODS: The authors obtained psychological assessment, language evaluation, neurologic examination, and neuroimaging investigation. Inclusion criteria were as follows: children should be at least 4 years of age; primary complaint of language delay; normal hearing; IQ >70; and an informed consent form signed by parents or guardians. Exclusion criteria were severe motor and cognitive handicap. RESULTS: Fifteen children met all inclusion criteria. Ages ranged from 4 to 14 years and 11 were boys. Six patients presented diffuse polymicrogyria (PMG) around the entire extent of the sylvian fissure on MRI, and they had severe clinical manifestation of DLD: they did not speak at all or had mixed phonologic-syntactic deficit syndrome. Six children presented PMG restricted to the posterior aspects of the parietal regions, and they had a milder form of DLD: mainly phonologic programming deficit syndrome. The other three children had different imaging findings. CONCLUSIONS: Developmental language disorder can be associated with polymicrogyria and the clinical manifestation varies according to the extension of cortical abnormality. A subtle form of posterior parietal polymicrogyria presenting as developmental language disorder is a mild form of perisylvian syndrome.

Adolescent↗
PubMed1987

Anterior temporal focal abnormalities in EEG in normal aged subjects; correlations with psychopathological and CT brain scan findings.

Normal aged subjects (65-83 years) were examined by EEG and CT brain scan; also a Geriatric Mental Scale (GMS) and Neuropsychologic Assessment Battery were administered. Based on the EEG findings 2 subgroups could be distinguished: one with focal abnormalities in the left fronto-temporal region and one without these focal abnormalities. The focal delta subgroup proved to perform poorly on the Fluency Test, a simple quick bedside, but very sensitive, test for word association productivity. Also this subgroup showed more ventricular dilatation than the non-focal group. Therefore slight left-sided antero-temporal abnormalities in normal aged subjects are not irrelevant but an early (subclinical) sign of temporal lobe pathology as expressed in deterioration of language function.

Aged↗
PubMed1991

Oral manifestations of paracoccidioidomycosis (South American blastomycosis).

Paracoccidioidomycosis (South American blastomycosis) is an uncommon, progressive systemic mycosis, potentially fatal if untreated. It is virtually restricted to persons spending time in Latin America. Reports of oral lesions are extremely rare in the English-language literature. Three adults with oral lesions as the first sign of paracoccidioidomycosis are described; this appears to be the largest series in the dental literature. The oral lesions had a characteristic appearance with a granular purpuric surface. The upper gingiva was a typical site, but lesions were also seen in the palate, tongue, and buccal mucosa. Two of the patients proved to have detectable pulmonary involvement. Long-term systemic ketoconazole therapy produced resolution of oral lesions in all cases.

Adult↗
PubMed1995

Developmental dyscalculia and brain laterality.

The correlation between arithmetic dysfunction and brain laterality was studied in 25 children with developmental dyscalculia (DD). The children were tested on a standardized arithmetic battery and underwent a neurological and neuro-psychological evaluation. A diagnosis of left hemisphere dysfunction (n = 13) was based on right side soft neurological signs, performance IQ (PIQ) > verbal IQ (VIQ), dyslexia and intact visuo-spatial functions. The criteria for right hemisphere dysfunction (n = 12) were left body signs, VIQ > PIQ, impaired visuo-spatial functions and normal language skills. The groups were similar for age, gender, and socio-economic status. Our results showed that both groups scored more than 2 SD below the mean adjusted score on the arithmetic battery, but the left group was significantly worse in 3 areas: mastery of addition/subtraction, complex multiplication and division and visuo-spatial errors (p < 0.05). The data indicate that dysfunction of either hemisphere hampers arithmetic acquisition, but arithmetic impairment is more profound with left hemisphere dysfunction.

Adolescent↗
PubMed1997

[Fragile X syndrome is still unrecognized: efficacy of molecular diagnosis in mentally retarded probands].

BACKGROUND: The fragile X mental retardation syndrome is the most common cause of inherited mental retardation. Identification of the unstable mutation responsible for the disease has allowed the design of a fully reliable molecular test for the diagnosis of the disease and for genetic counselling (identification of clinically normal carriers and prenatal diagnosis). We started in July 1991 to search for the mutation in mentally retarded probands, with no known cause for their phenotype. We present the results of a 42-month experience. POPULATION AND METHODS: One thousand and one hundred fourty-nine probands were analysed. In case of a positive diagnosis, an extension of the molecular study to relatives was proposed. DNA samples were studied by Southern blot following EcoRI or EcoRI + EagI digestion. Clinical data were collected from referring clinicians. RESULTS: Seventy-three carriers of a full mutation were identified, belonging to 52 families. The mean age of the fragile X probands was 16 +/- 14 years, which is very surprising for a disease that causes significant manifestations by the age of 2 to 3 years. This indicates an insufficient knowledge about this disease in France. Most of the demands for the test were from clinical geneticists. This diagnosis is of major importance for genetic counselling, as illustrated by the following study of 108 women at risk in these families. CONCLUSIONS: The importance of an early diagnosis followed by an extended family study, for carrier screening and prevention of this severe disease, justifies molecular testing on any child with mental retardation or significant language delay of unknown cause, in the absence of clinical signs formally excluding a fragile X diagnosis.

Adolescent↗
PubMed1996

Dysphagia and post-polio syndrome: past, present, and future.

Swallowing difficulties, oral motor weakness, and oral pharyngeal paralysis have been reported in the historical literature on polio and post-polio. Attention to this finding was minimal in the original epidemics as many persons with bulbar polio who may have been dysphagic did not survive. Swallowing was often supplemented by artificial feeding for those who were placed in respirators. Although swallowing difficulty was often present in the acute stages of the initial attack, it usually subsided or disappeared after several weeks. In cases in which bulbar polio was diagnosed, swallowing impairment may have lingered, but most survivors learned to produce compensatory maneuvers to aid them to swallow and did not complain of difficulty. In the recent decades of the 20th century, many persons who had believed themselves fully recovered from the insult of polio began to experience new symptoms; among them were new or heightened complaints of swallowing difficulty. Studies of these patients with PPS have revealed that dysphagia is not only present but may be progressive, and suggest that there is a slowly progressive deterioration of the bulbar neurons of PPS patients underlying the finding of new or exacerbated swallowing signs. Patients with PPS should be referred to speech-language pathologists for evaluation of voice, phonatory-respiratory, and swallowing function and initiation of appropriate remediation strategies to avoid the potential negative consequences of dysphagia.

Deglutition Disorders↗
PubMed1976

Minimal brain dysfunction/specific learning disability: a clinical approach for the primary physician.

Minimal brain dysfunction is a neurodevelopmental disorder which can be found in nearly 20% of school children. It is characterized by evidences of immaturity involving control of activity, emotions, and behavior, and by specific learning disabilities involving the communicating skills needed in reading, writing, and mathematics. The prime deficits in the classroom are an inability to maintain attention and concentration and an inability to skillfully blend the auditory and visual functions essential in language performance. Medical evaluation will reveal many of the "soft signs" of neurologic involvement, and educational appraisal will indicate a wide scatter in testing scores with a marked discrepancy between evaluated potential and actual classroom achievement. Remedial efforts directed at early detection, relief from pressure and unjust punishment or ridicule from parents and teachers, and adjustment of the educational environment with consideration of the child's individual talents, combined with the judicious use of medications to prolong attention span and improve neurodevelopmental maturity, hold promise of improving the lot of most involved children. There are valid indications that expansion of such programs can do much to prevent these youngsters from developing severe personality maladjustment and delinquent behavior, as well as emotional illness in later life.

Attention Deficit Disorder with Hyperactivity↗