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At least 559 records · Page 31Linked to original sources
PubMed1985

Hemichorea associated with varicella-zoster reinfection and endocarditis. A case report.

A 20-year-old woman developed transient right-sided hemichoreatic movements after household exposure to varicella-zoster. Some days before the appearance of involuntary movements a vesicular rash had occurred. About 6 months later an elevated IgG serum titer against varicella virus was found and two-dimensional echocardiography showed signs of an endocarditis. During the following 2 months the IgG value returned to within the normal range and the choreatic movements disappeared almost totally. The possibility is discussed that endocarditis had been caused and maintained by serum antibodies to varicella-zoster virus which cross-reacted with valvular tissue. Embolization to the region of the left striatum and/or postinfectious encephalitis in this region are assumed to be the most plausible causes of the transient hemichorea.

Adult↗
PubMed1978

3-methylglutaconic aciduria: report on a sibship with infantile progressive encephalopathy.

Choreoathetosis, spastic parapareses, dementia and optic atrophy were the main clinical features in a sibship with progressive encephalopathy of late onset. The urine contained constantly elevated amounts of 3-methylglutaric and 3-methylglutaconic acids. The identity of these metabolites was confirmed by synthesis and mass spectrometry. On leucine loading, the excretion of the metabolites was elevated.

Amino Acid Metabolism, Inborn Errors↗
PubMed1987

Paroxysmal kinesigenic choreoathetosis.

A healthy intelligent 13.5-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. He had had unilateral tonic spasms for 2 months. In a stressful situation, especially after a period of immobility, a sudden voluntary movement provoked a dystonic-choreoathetoic posturing of the left arm and leg. Attacks were brought on, for instance, by standing up quickly from a sitting position. They lasted only 5-10 s, without loss of consciousness and occurred up to ten times daily. Between the attacks there were no abnormalities. Family history was non-contributory. Neurological examination and laboratory findings, including repeated EEGs, were all negative. The child responded very well to phenytoin treatment and has now been completely free of dystonic attacks for a period of 9 months. The importance of early recognition of this syndrome and the differential diagnosis with reflex epilepsy and other forms of dystonic choreoathetosis is discussed.

Adolescent↗
PubMed1976

Frontal EMG-biofeedback training of athetoid cerebral palsy patients: a report of six cases.

Six athetoid cerebral palsy patients participated in the following: speech and motor prebiofeedback training evaluation; frontal EMG biofeedback training, 6 wk; speech and motor postbiofeedback training evaluation. Frontal pretraining levels for the subjects averaged 28.9 muV p-p. Subjects' feedback consisted of an auditory signal (clicks) varying proportionately with frontal EMG activity. A visual meter display of the integrated EMG was also provided. Self-regulation of frontal EMG was evident for all subjects within session 1. Throughout all sessions, EMG levels of 2-4 mu V were often attained. Trend analysis of EMG acquisition curves showed significant reduction in frontal tension across sessions for all but one subject. Frontal posttraining levels averaged 13.0 muV p-p. Parents or subjects, or both, reported subtle improvements in various speech and motor functions, a finding confirmed by objective postbiofeedback training evaluation. Only the 2 most severely impaired subjects, JA and DS, failed to improve significantly on the speech measures. All subjects improved significantly on those measures that tapped fine and gross motor skills. Collectively, these results indicate that EMG biofeedback training shows promise as an additional treatment modality in the habilitation of cerebral palsy patients.

Athetosis↗
PubMed1993

Therapeutic stereotactic procedures on the thalamus for motor movement disorders.

The value of functional neurosurgery in the treatment of motor movement disorders is emphasized. The two methods of stereotactic procedures, namely a destructive one with small lesions centered on specific targets, and a non-destructive one with chronically inserted electrodes connected with an also implanted programmable neuropacemaker are described in detail. The results in Parkinsonian tremor, essential tremor, tremor of multiple sclerosis, post-traumatic tremor and in other involuntary movement disorders are reported and demonstrate that stereotactic neurosurgical treatment of these conditions is a safe and efficacious method.

Adult↗
PubMed1988

[No Parkinsonian syndrome following acute paraquat poisoning].

Paraquat structurally resembles N-methyl-4-phenyltetrahydropyridine (MPTP) and its metabolite 1-methyl-4-phenylpyridine (MPP+). MPTP and MPP+ are neurotoxic chemicals, which induce in exposed humans and in animal-models a Parkinson's disease. A high correlation between the incidence of Parkinson's disease and herbicide use in Canada led to the assumption that paraquat could give rise to parkinsonism. We have therefore carried out a follow-up study with patients having had dermal contact with paraquat or having swallowed paraquat accidentally or in a suicidal attempt. 7 patients took part in the study. Three of them had dermal contact. One had ingested paraquat by accident and three were survivors from suicidal paraquat intake. It was possible to exclude parkinsonism in all patients. One patient exhibited tardive dykinesia most likely due to a long term therapy with neuroleptic drugs.

Accidents↗
PubMed1991

Low arylsulphatase A activity and choreoathetotic syndrome in three siblings: differentiation of pseudodeficiency from metachromatic leukodystrophy.

We report on a family with a sibship of three children for whom the diagnosis of "an unusual form of metachromatic leukodystrophy (MLD)" had been suggested earlier. The patients had choreiform movements and dystonic posturing accompanied by dysarthria since childhood. The availability of the polymerase chain reaction enabled us to show that the three siblings have a pseudodeficiency genotype (ASAp/ASAp). There was no abnormal sulphatiduria, and we propose that the neurological disease and low arylsulphatase A activity are unrelated to one another in this family. A diagnosis of MLD carries very serious implications, and we recommend that gene amplification by polymerase chain reaction and hybridization with allele-specific oligonucleotide probes should be used to corroborate the diagnosis, especially when there is no abnormal sulphatiduria and when metachromatic material cannot be demonstrated in a sural nerve biopsy.

Adolescent↗
PubMed1983

Paroxysmal kinesigenic choreoathetosis. Case report.

A case of sporadic, idiopathic, paroxysmal kinesigenic choreoathetosis is described. The frequent attacks triggered by the initial phase of voluntary movement responded well to treatment with diphenylhydantoin. The nosological, etiopathogenetic, clinical and therapeutic aspects of paroxysmal choreoathetosis are discussed and the literature reviewed.

Adult↗