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At least 163 records · Page 9Linked to original sources
PubMed1989

[A neurologic model of early infantile autism].

Based on the abnormalities in sleep-wakefulness cycle of early infantile autism, the author discussed its pathophysiology focusing on its main lesion in the raphe nuclei. These neurons, located in the midline portion of the brainstem send their axons to various neurons of the upper and lower nervous systems, including the locus coeruleus and the dopamine neurons of the tegmentum, the former having a broad innervation and the latter a restricted area in the central nervous system. These monoaminergic neurons modulate the functions of the involved neurons and regulate their functional and structural maturation in the early developmental course. The early lesion of the raphe nuclei causes poor adaptation to environment which develops as abnormal circadian oscillation and pervasive lack of responsiveness. Combined hypofunction of the locus ceruleus, particularly of its dorsal bundle, results in the failure of extinction of acquired memory in mice which relates clinically to the excellent memory and resistance to change peculiar interests and attachments in humans. From early childhood, the disturbance of dopaminergic neurons becomes apparent clinically, and causes hyperkinesia and stereotyped activities. With the other two monoaminergic neurons, dopaminergic neurons cause occasional aggressiveness or self-mutilation. The latter behaviors are like those of pampered children and are simulated to "muricide" and "friendliness" observed in rats with these monoaminergic lesions. The particular language disturbance with echolalia is due to the right hemispheric dominance, which might have been caused by a delayed functional lateralization of the hemisphere resulting also from the delayed development of the circadian oscillation in infancy. The motor disturbances consisting of hypotonia and impaired locomotion might be due to decreased tonic innervations of the locus ceruleus and the raphe nuclei to the spinal locomotion center. CT examination of symptomatic autism showed the amygdala as one of the causative nuclei for the autistic behavior.

Animals↗
PubMed1988

Autism in association with fragile X syndrome in females: implications for diagnosis and treatment in children.

Fragile X syndrome is the second most common chromosomal cause of mental retardation (MR). The calculated incidence is 1/1000, making accurate and early diagnosis important for specific preventive, pharmacologic, and cognitive treatment. The timely diagnosis in males is facilitated by the characteristic phenotype and an association with autism. In contrast, in females heterozygous for fragile X, the characteristic phenotype and infantile autism are rarely reported. We present two females with cytogenetic expression of the fragile X chromosome for whom the studies were performed because of the presence of autism or prominent autistic features and a behavioral and physical phenotype consistent with fragile X syndrome. The first female, age three years, has autism, hyperactivity, echolalia, language delay, hand stereotypies, and mild MR. The characteristic phenotype was not present nor was there a family history of X-linked MR. Fragile X expression was 6% in the proband, 3% in the mother and 1% (normal) in the father. The second child, seven years old, has prominent autistic features, hyperactivity, mild MR, mild language disorder, and a family history consistent with X-linked MR. Fragile X expression was 3% in the proband and 0% in the mother. These cases support the occurrence of fragile X in autistic females and emphasize the importance of cytogenetic screening for fragile X in this high risk population. Early diagnosis of fragile X allows precise genetic counseling and more specific cognitive and pharmacologic treatment.

Autistic Disorder↗
PubMedDate not supplied

[Immune status in infantile autism. Correlation between the immune status, autistic symptoms and levels of serotonin].

In sixteen autistic children high values of IgG and a high level of lymphocyte stimulation with PHA were observed. Principal component analysis showed: 1) a significant correlation between basic lymphocyte mitogenic activity and the clinical symptoms opposition and hyperactivity, 2) a significant correlation between high Ig levels, high PHA stimulation responses and the main autistic symptoms (withdrawal, inaffectivity, hypoactivity, mannerism, stereotypy and negatively echolalia), 3) a significant correlation with serotonin uptake by platelets and high immunological responses. Such correlations are strongly in favor of an immunologic component in autistic disease.

Adolescent↗
PubMed1987

A controlled study of Tourette syndrome. IV. Obsessions, compulsions, and schizoid behaviors.

To determine the frequency of obsessive, compulsive, and schizoid behaviors in Tourette syndrome (TS), we prospectively questioned 246 patients with TS, 17 with attention-deficit disorder (ADD), 15 with ADD due to a TS gene, and 47 random controls. The comparative frequency of obsessive, compulsive, and repetitive behaviors--such as obsessive unpleasant thoughts, obsessive silly thoughts, echolalia, palilalia, touching things excessively, touching things a specific number of times, touching others excessively, sexual touching, biting or hurting oneself, head banging, rocking, mimicking others, counting things, and occasional or frequent public exhibitionism--were significantly more common in TS patients than in controls. The frequency of each of these was much higher for grade 3 (severe) TS. Most of these behaviors also occurred significantly more often in individuals with ADD or in individuals with ADD secondary to TS (ADD 2(0) TS). When these features were combined into an obsessive-compulsive score, 45.4% of TS patients had a score of 4-15, whereas 8.5% of controls had a score of 4 or 5. These results indicate that obsessive-compulsive behaviors are an integral part of the expression of the TS gene and can be inherited as an autosomal dominant trait. Schizoid symptoms, such as thinking that people were watching them or plotting against them, were significantly more common in TS patients than in controls. Auditory hallucinations of hearing voices were present in 14.6% of TS patients, compared with 2.1% of controls (P = .02). These symptoms were absent in ADD patients but present in ADD 2(0) TS patients. These voices were often blamed for telling them to do bad things and were frequently identified with the devil. None of the controls had a total schizoid behavior score greater than 3, whereas 10.9% of the TS patients had scores of 4-10 (P = .02). This frequency increased to 20.6% in the grade 3 TS patients. These quantitative results confirm our clinical impression that some TS patients have paranoid ideations, often feel that people are out to get them, and hear voices.

Adolescent↗
PubMed1986

[Tics: from Itard to the neuroleptics].

When the six DSM-III (1980) diagnostic criteria are applied to the nine cases reported by Gilles de la Tourette in 1885, six of them are found to be in accordance with the diagnosis of Gilles de la Tourette's syndrome (cases nos 4, 5 and 7 do not involve vocal tics). Gilles de la Tourette deserves credit, not only for having regrouped fragmented observations into one remarkably well described clinical entity which held over time (such as Itard's observations nos 9 and 10 in 1825; the latter is the famous Marquise of D ... seen several times by Charcot and the only one which, along with no 1, appears in Gilles de la Tourette's paper), but also for having described the course of this chronic and fluctuating disease. Why Gilles de la Tourette did not use the term "tic", a term which had been in use for a long time in both veterinary and human medicine, to describe "the motor incoordination" of these patients? Did Charcot take some distance from his student's paper as early as 1885? He viewed tics as the basis of "the disease described by Gilles de la Tourette". In addition to coprolalia and echolalia, he alsa reported the existence of "mental tics". How have French neurologists and psychiatrists been able to perpetuate Brissaud's error who, contrary to Gilles de la Tourette, mentioned that the illness "can be associated with severe mental disorders which often lead to dementia"?(ABSTRACT TRUNCATED AT 250 WORDS)

France↗
PubMed1979

Remarks on the communication of psychotic children as seen in group therapy.

A group of 26 psychotic children, aged 5--8, in a day hospital, was treated in small groups of 4 or 5, by 2 therapists (a male and female). According to our observations the treatment set-up enhances a 'present therapeutic symbiosis' where the 'other half' of the symbiotic dyad is composed of all the children in the group, the therapists and the physical environment. The newly acquired imitation (echolalia and echopraxia) impraxia) improves the child's means of expression and is used for communication. It also helps the therapists in their task of decodifying the messages to the children and themselves.

Autistic Disorder↗
PubMed1995

[Auditory comprehension in transcortical motor aphasia due to a medial lesions of the left frontal lobe].

We assessed the anatomical findings and auditory comprehension of six patients with transcortical motor aphasia due to medical lesions of the left frontal lobe. All patients were right-handed and were initially mute for several hours after the onset, and they exhibited mild paresis of the right lower extremity. Their spontaneous speech was sparse and not fluent, and sometimes accompanied by echolalia, but their articulation was normal and repetition was excellent. They had difficulty in recalling words. A diagnosis of transcortical motor aphasia was made on the basis of their clinical symptoms. All patients were found to have an infarct in the left medial frontal region by MRI and/or CT. We administered the Western Aphasia Battery and 50 line drawing pointing task in order to evaluate auditory comprehension. Based on the results we concluded that there is no impairment of auditory comprehension of single words when lesions are limited to the superior frontal gyrus, but that lesions extending to the middle frontal gyrus interfere with auditory comprehension of single words. Our observations indicate that the middle frontal gyrus plays an important role in auditory comprehension of single words. All of the patients displayed impaired auditory comprehension of sentences even when their lesions were strictly limited to the medial frontal lobe. This suggests that the medial frontal lobe plays some role in the auditory comprehension of sentences.

Aged↗
PubMed1994

[Transcortical sensory aphasia in a patient with metastatic brain tumor in the left frontal lobe].

We reported a case of a 62-year-old right-handed woman who had transcortical sensory aphasia caused by a metastatic brain tumor in the left frontal lobe. She had mild right hemiparesis involving the face, without hyperactive tendon reflexes. She had neither sensory disturbance nor other cranial nerve deficits. Her spontaneous speech was fluent, and she sometimes had echolalia. Her object naming, word fluency, verbal comprehension and writing were severely disturbed. This contrasted with full preservation of repetition of phonemes and short sentences. Reading of words was preserved. CT scan revealed a subcortical lesion in the left superior frontal gyrus. Gd-enhanced MRI showed a ring-enhanced mass lesion in the frontal lobe outside of Broca's area. We thereby concluded that transcortical sensory aphasia may be caused by frontal lobe lesion independent of the perisylvian speech areas.

Adenocarcinoma, Papillary↗
PubMed1993

[From tics to the Gilles de la Tourette syndrome].

We report a case whose progressive course and forms of manifestation of generalized tic movements indicate relatively rare Gilles de la Tourette disease. It is characterized by the triad--non-coordinated mobility-dyskinesia, echolalia corporalia, epilepsy and according to the current data from the literature, compulsive behavior, phobias and sometimes episodes of insanity in the advanced stage. The clinical case we report as well as cases from the world literature is rather dubious, asks for prolonged follow-up and has polymorphic symptomatology and necessitates a multidisciplinary therapeutic approach. Beside the clinical features, laboratory findings has also been discussed.

Child↗
PubMed1993

[Two cases of primary progressive non-fluent aphasia].

Two patients were described with a five to seven-year history of primary progressive non-fluent aphasia. One patient developed atypical trascortical motor aphasia with marked anarthria, which has led to mutism. Magnetic resonance (MR) imaging showed lobar atrophy of the frontal lobe accentuated in the bilateral superior frontal gyri, the left middle frontal gyrus, the left anterior cingulate gyrus and the left operculum with some extension into the left temporal lobe. Single photon emission computed tomography (SPECT) scans demonstrated a decrease of regional cerebral blood flow (rCBF) in the atrophic site. The patient was clinically and neuroradiologically diagnosed as having Pick's disease. Another patient presented with atypical Broca's aphasia, which has worsened with slowly progressive right hemiparesis. Mitigated, sometimes complete, echolalia was also observed. MR imaging and SPECT scans showed mild atrophy and a decrease of rCBF in the left perisylvian region involving the frontal operculum, while a positron emission tomographic study disclosed diffuse hypometabolism in the left hemisphere. We pointed out that the features of primary progressive aphasia were frequently atypical in the light of classical classification of aphasia and that non-fluent aphasia might be observed even in the early stage of cortical degenerative processes.

Aphasia, Broca↗
PubMed1993

Autism with hyperlexia: a distinct syndrome?

Family and medical histories, autistic and dysmorphic features, and neurological status of 5 children with autism and hyperlexia and 5 sex and IQ-matched children with autism and no hyperlexia were compared. Results showed that the children with hyperlexia displayed more persistent echolalia, superior visual motor performance, and more favorable response to vestibular stimulation. These children performed better than did their matched controls in the physical and neurological assessment. Two of these children, one of whom had a sibling with hyperlexia, presented with macrocephaly. Both groups had a similar incidence of dysmorphic features, computerized tomographic and EEG abnormalities, and family morbidity. Results suggest that children with autism and hyperlexia probably represent part of the continuum of autism rather than a specific syndrome.

Adolescent↗
PubMed1997

The pattern of neurological sequelae of childhood cerebral malaria among survivors in Calabar, Nigeria.

OBJECTIVE: To determine the pattern and long term outcome of neurological complications following cerebral malaria (CM) in a group of Nigerian children treated in Calabar. DESIGN: Prospective, follow up study. SETTING: Children's emergency room (CHER) of the University of Calabar Teaching Hospital (UCTH) located in a malaria-holoendemic rainforest belt of south eastern Nigeria. SUBJECTS: Survivors among 45 children with CM treated between February and December, 1991. All received intravenous quinine infusion and supportive care. Survivors (39) were followed up until detected neurological sequelae had resolved. RESULTS: Case fatality rate was 13.3%, 95% CI. Eleven (28.2%) of the survivors developed neurological sequelae. Prolonged coma, focal seizures and abnormal posturing (decorticate/decerebrate) were associated with increased risk of sequelae. Commonest neurological sequelae were cortical blindness (3/11), speech disorders (3/11: aphasia or echolalia) and motor abnormalities (5/11: dyskinesia/hemiplegia). Eight cases recovered completely from the neurological deficits within a mean period of three (1.3) weeks. One persisted with hyperactivity and attention deficit, had a remarkable improvement at the sixth month of follow up but developed secondary dyslexia and other learning disabilities by the third year of follow up. CONCLUSION: Although short lived, neurological sequelae of CM appear common among these Nigerian children. This problem could significantly add to the burden of childhood disability in Nigeria. Early diagnosis, use of appropriate drugs and large scale malaria control programmes can prevent malady.

Central Nervous System Diseases↗