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At least 775 records · Page 43Linked to original sources
PubMed1995

Selective injury of the globus pallidus in children with post-cardiac surgery choreic syndrome.

Occasionally children undergoing cardiac surgery using cardiopulmonary bypass with deep hypothermia and cardiac arrest develop a postoperative syndrome of acute chorea. The authors report the neuropathological findings in two such children surgically treated for congenital heart disease. Examination of the brain showed neuronal loss, reactive astrocytosis and degeneration of myelinated fibers (without frank necrosis) in the globus pallidus, primarily the outer segment, with sparing of other regions commonly susceptible to hypoxic-ischemic necrosis. The localization and relative mildness of the brain damage suggest a susceptibility of the globus pallidus to injury in this setting and implicate disruption of pallidal pathways in the pathogenesis of post-cardiac surgery choreic syndrome.

Acute Disease↗
PubMed1978

Ten year follow-up of paroxysmal choreoathetosis: a sporadic case becomes familial.

A 30-year-old woman is reported who was originally described in 1967 as an isolated instance of paroxysmal choreoathetosis. In the subsequent 10 years, her movement disorder has decreased in severity. However, she now has a 7-year-old daughter with a similar but more persistent and more serious condition. This family emphasizes both variability of manifestations of paroxysmal choreoathetosis and the importance of genetic factors.

Adult↗
PubMed1999

Familial paroxysmal kinesigenic choreoathetosis: an electrophysiologic and genotypic analysis.

PURPOSE: We report a pedigree of familial paroxysmal kinesigenic choreoathetosis (PKC) in which five of 18 members are affected. The pathophysiologic basis for PKC is still uncertain; reflex epilepsy versus dysfunction of basal ganglia. We examined (a) whether there were ictal discharges during the attacks, and (b) a linkage between PKC and possible DNA markers linked to several familial epileptic or movement disorders. METHODS: Video-monitoring EEG was performed in two patients with PKC during attacks elicited by movements of the lower extremities. Blood samples for DNA studies were obtained from 15 members of the pedigree. Fourteen polymorphic markers on chromosomes 1p, 2q, 6p, 10q, and 20q were genotyped, and two-point lod scores were calculated for each marker under a dominant model. RESULTS: No ictal discharges were found during the attacks in both patients. We could not obtain significant linkage of PKC with any marker examined. CONCLUSIONS: The video-monitoring EEG findings in our cases strongly suggested that the etiology of PKC should be considered distinct from that of reflex epilepsy. However, the patients in this pedigree had experienced generalized convulsions in their infancies; thus we could not deny the possibility of an epileptogenic basis for PKC. There was no strong evidence for a linkage of the gene for PKC with the candidate regions on 1p, 2q, 6p, 10q, or 20q.

Athetosis↗
PubMed1977

Observations on physiological and psychological functions of the ventral oral internal nucleus of the human thalamus.

Eight patients underwent VOI thalamotomy for spasmodic torticollis or choreoathetosis. The clinical results were disappointing: at follow-up examination 8 months postoperatively, on average, none of the seven patients with torticollis regarded the effect as good. In two there was some long-lasting improvement, but in five the results was nil. One patient with choreoathetosis did not benefit from surgery. Side-effects were frequent but not serious; they included excessive sleepiness, drowsiness, weight increase, deterioration of memory and loss of general activity. Electrical stimulation of the VOI nucleus caused subjective sensations in the neck and shoulder muscles without movements of the head, and autonomic or emotional reactions. An evoked scalp response was recorded in contralateral frontal area 8. As shown by psychological tests, in visuospatial performances there is a preoperative defect, which seems to be specific to spasmodic torticollis. After left VOI thalamotomy both receptive and expressive verbal efficiency were impaired. Visual perception and memory were almost unaffected by VOI thalamotomy.

Adult↗
PubMed1985

Progabide in the treatment of hyperkinetic extrapyramidal movement disorders.

The ability of the selective GABA-receptor agonist, progabide, to suppress abnormal involuntary movements was evaluated in a preliminary open pilot study. 17 patients, 10 males and 7 females, aged 10-78 years, with hyperkinetic movement disorders were included in the study. Daily doses of progabide ranged from 900 to 3600 mg (median 2400 mg) corresponding to 14-45 mg/kg (median 45 mg/kg), while the duration of treatment varied from 2 to 52 weeks. Improvement, with a reduction of involuntary movements exceeding 25%, occurred in two of four patients with Gilles de la Tourette's syndrome, and in two of three patients with postanoxic intention myoclonus, while no consistent beneficial effects were registered in ten patients with Huntington's chorea, postanoxic choreoathetosis, torsion dystonia, tardive dyskinesia, action tremor, essential myoclonus, or oro-branchio-respiratory myoclonus.

Adolescent↗
PubMed1994

Hand dystonia secondary to cervical demyelinating lesion.

Dystonia is generally considered a disease of basal ganglia. We report two patients with definite MS who acutely developed hand dystonia and athetoid movements. In both patients: 1) dystonia was electrophysiologically demonstrated by lack of reciprocal inhibition between antagonistic forearm muscles; 2) SEP from median nerve stimulation showed delayed cervical N13 and absent frontal and parietal components; 3) the long latency response after stimulation of median nerve of the affected hand was absent or delayed; 4) MRI showed lesions in the posterolateral cervical spine but no involvement of basal ganglia and thalami. We think that, in these cases, dystonia and athetoid movements could be ascribed respectively to the involvement of descending pathways regulating reciprocal inhibition of motoneurons and to the involvement of large diameter afferents due to the demyelinating lesion at the cervical level.

Adult↗
PubMed1993

Prospective data on the prevalence of abnormal involuntary movements among elderly people living in the community.

Trained raters from the Liverpool Continuing Health in the Community study interviewed 1070 people over the age of 65 in 1982-1983 using the Geriatric Mental State (GMS) examination. Three years later the cohort was re-interviewed, this time by psychiatrists trained in the GMS, who used the GMS and the History and Aetiology Schedule (HAS). The cohort had fallen in size to 875, because of mortality; 701 were re-interviewed. The cohort was followed up again 6 years after the first interview by trained nurse raters using the GMS (A3) and the Mini-Mental State Examination. Cases of mental disorder identified by the computer diagnostic program AGECAT were re-interviewed by psychiatrists along with a number of controls using the GMS and the HAS on the remaining 450 individuals. Observational behavioural ratings from the GMS and summary sheets were analysed along with AGECAT diagnoses and data on medication gained at the 3 assessments. The community prevalence of tardive dyskinesia and other movement disorders in elderly people over a 6-year follow-up appears to be very low (the community prevalence of tardive dyskinesia being 0.22% and akathisia 1.57%), is usually associated with organic mental disorder (and consequently higher mortality) and is furthermore not usually associated with antipsychotic medication.

Aged↗
PubMed1975

The changing panorama of cerebral palsy in Sweden 1954-1970. II. Analysis of the various syndromes.

From an unselected series of 560 Swedish cases of cerebral palsy, born 1954-1970, various data of etiologic and pathogenetic interest were analyzed in detail. Untraceable and prenatal factors were found to dominate within the group of spastic hemiplegia. Placental dysfunction in small-for-date babies and severe asphyxia were thought to be the two main pathogenetic factors among the patients with spastic tetraplegia. In spite of a significant decrease in the number of low birth weight children within the group of spastic diplegia, this syndrome was still very characteristic for the child born immature. Ataxic diplegic forms were found to have greater pathogenic similarities to spastic diplegia than to simple ataxia. In two-thirds of the children the latter syndrome was characterized by normal pregnancy, delivery and birth weight and an untraceable (genetic?) factor. Dyskinetic syndromes were mostly encountered after perinatal asphyxia.

Adolescent↗
PubMedDate not supplied

Magnetic resonance imaging in athetotic cerebral palsied children.

The magnetic resonance findings in 22 children with athetotic cerebral palsy were studied. Sixteen had perinatal asphyxia, two had neonatal jaundice, and four had no association with predisposing conditions. In six of the children, symmetrical high intensity areas were found in both the thalamus and putamen in T2-weighted images. In five children they were seen only in the thalamus, and in another one only in the putamen. In six children, symmetrical periventricular high intensity areas were seen. In seven of the subjects, no abnormal magnetic resonance findings were seen. Magnetic resonance lesions, possibly caused by asphyxia, were found in the basal ganglia, thalamus and/or cerebral white matter in 14 of 16 children. Three children with lesions only in the cerebral white matter had mild motor abnormality, and six children with lesions in both the thalamus and putamen did not have mild abnormality.

Athetosis↗