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[Levodopa treatment of a case of torsion spasm with athetosis].

The result about Levodopa treatment in torsion dystonia and athetosis are quite contradictory. We report an interesting case, significant for the results that we obtained in a long treatment period. A girl was suffering from age of 8 years of torsion dystonia associated with athetosis. We made our first clinical examination when 12 years old: she was not able to stand-up and to walking, neither was she able to be sitting in a chair without arms. In two months of treatment with Levodopa 3.000 mg. by day, the symptoms completely disappeared. During the first two years of treatment, at every effort to decrease the therapy we observed an important increase of symptoms. Successively it was possible to decrease the therapy and now, after 8 years of treatment, with a minimal dose, the girl doesn't show any symptoms, so that she can manage a normal relative life. Probably these good results depend on the fact that was a sporadic recessive form of torsion dystonia case.

Athetosis

Bilateral cervical posterior rhizotomy: effects on dystonia and athetosis, on respiration and other autonomic functions.

Bilateral cervical posterior rhizotomy of C1 through C4, C5 or C6 was performed in 16 patients affected by dystonia and athetosis resulting from infantile cerebral palsy. The majority showed decreased muscle spasms and athetoid movements, with some improvements in their posture and voluntary mobility. 5 patients suffered from uneven and irregular breathing associated with lethargy immediately postoperatively, and 4 also showed reduced diaphragmatic activity; all 4 of these patients developed pneumonia, transitory in 3, but fatal to the other. Urinary retention lasting for a maximum of 3 months occurred in 4 of the 5 patients. The authors suggest that the lesion of ascending reticular fibers in the cervical posterior roots could have been responsible for the observations.

Adolescent

Glutaric aciduria in progressive choreo-athetosis.

The clinical symptoms in a 10-year-old girl with progressive dystonic cerebral palsy are described. The biochemical findings were dominated by large amounts of glutaric acid in the urine. The disorder is caused by impairment of the degradation of glutaryl-CoA. A survey is given of the clinical and biochemical symptoms, based on the five cases reported so far. It is concluded that patients with progressive dystonic palsy should be examined for disorders in the metabolism of organic acids.

Acyl Coenzyme A

Follow-up study of patients with cerebral palsy.

Of 319 patients with cerebral palsy recalled for reevaluation 15 years after the initial visit, 10 percent had died. Of the living, 55 percent had spasticity, 32 percent had athetosis, 4 percent had ataxia and 9 percent had mixed spasticity and athetosis; 38 percent had an intelligence quotient (IQ) less than 50, 24 percent between 50 and 79, and 38 percent had IQ above 80. There was a high correlation between overall functional outcome and intellectual level. Severity of physical disability, as measured by hand use, mobility and speech, also correlated with dependence, in part because increased severity of the disability was associated with decreased intellectual capacity generally.Twenty-five years after the initial visit, parental attitudes and personality intactness were evaluated (using the Minnesota Multiphasic Personality Inventory [MMPI]) and were correlated with satisfaction with status in life in 28 persons predicted to be independent on the 15-year study. Twenty (72 percent) of the 28 were satisfied with their status in life and of these, 16 were evaluated (with the MMPI) with 70 percent scoring in the normal range; 13 (65 percent) had parents with a positive attitude. Positive attitude was defined as parental feelings that the handicapped child was a worthy, valuable person, to be encouraged and assisted but not isolated from the world of nonhandicapped people. Careful serial assessment by professional teams combined with repeated long-term counseling of families can result in optimal outcome for the disability level involved, due to the primary role parents play in the development of a child's character and behavior.

Adult

[Statistical study of the relationships between the etiology and clinical picture of cerebral palsy].

846 children with cerebral palsy were examined and 642 were selected for a statistical study by correspondence analysis. The aim was to identify without any prior assumptions, the relationships between the aetiological factors and the clinical findings. The study was completed by conventional statistical analysis of 584 of the cases. Small birth weight and a history of abnormal pregnancies was associated with a cerebral palsy affecting both legs, and often with a squint. Resuscitation was associated with athetosis and abnormalities of posture and behaviour. If the resuscitation lasted for more than 15 minutes or there were certain problems during delivery, severe abnormalities of both arms and major speech difficulties were observed. Resuscitation for less than 15 minutes or for an unknown time or intractable vomiting during pregnancy was associated with quadraplegia. Hemiplegia was related to post natal events but the aetiology was not always known. Foeto-maternal incompatibility was associated with athetosis, deafness, severe speech problems and ophthalmoplegias.

Abortion, Threatened

Articulatory abnormalities in athetoid cerebral palsy.

This report presents cinefluorographic data on the articulation of isolated vowels, VCV nonsense utterances, and short sentences by five subjects with athetoid cerebral palsy. Articulatory abnormalities were identified from tracings of vocal tract shapes and from displacement-by-time plots of articulatory events. The most frequent abnormalities were large ranges of jaw movement, inappropriate positioning of the tongue for various phonetic segments (especially because of a reduced range of tongue movement in the anteroposterior dimension), intermittency of velopharyngeal closure caused by an instability of velar elevation, prolonged transition times for articulatory movements, and retrusion of the lower lip. The speech disorder associated with athetosis is considered with respect to a model of motor learning.

Adult

Long term results of stereotaxic surgery for infantile dystonia and dyskinesia.

Our experience is based on a series of 25 patients suffering from infantile dyskinesias and dystonias who underwent stereotaxic surgery of basal ganglia. We first note the immediate good results (77%) obtained by thalamo-sub-thalamotomy. Concerning dyskinesia this type of surgery enjoys in an opinion a place of choice. Secondly we have been able to estimate secondary deterioration on long term results in patients examined 2 to 15 years post-operatively. In contrast to various results reported in the literature good long term results are not superior to 50%. Some deterioration is noted in patients operated on for choreo-athetosis over the age of 20. A pallido-subthalamic lesion is efficient at the beginning of the disease course since it improves motor performance and thereby helps possibilities of intellectual acquirement. Bilateral lesions have after improved I.Q. Effects of this treatment specially on spasticity must be discussed among other types of surgery.

Adolescent

Natural history of the dislocated hip in spastic cerebral palsy.

A review was made of 88 adult institutionalized patients with spastic cerebral palsy and contractural deformity of the hips. 21 were untreated for dislocated hip, and 11 of these suffered from hip pain. The degree of pain was directly related to neurological maturity and to the coexistence of athetosis and spasticity. Decubitus ulcers and perineal care problems were more associated with contractures than with dislocation alone. It is concluded that dislocation and subluxation should be prevented by surgical means, but that surgical treatment of the already dislocated hip should be reserved for the neurologically mature and athetoid patient.

Adult

A physiological approach to motor disorders.

Spasticity in man is presented as a disinhibition of spinal cord mechanisms, the responses to stretch depending on the interaction of the reflex effects of group Ia with those of group II afferent fibres. The reflex responses to muscle stretch and shortening in Parkinson's disease do not depend on an abnormality of spinal reflex mechanisms. The superimposition of physiological tremor or alternating tremor in rigidity produces the classical cog-wheel sensation. The phase lead of the action tonic stretch reflex was found to be reduced in patients with athetosis and cerebellar disease, thus diminishing damping of unwanted movements. The more complex transmission characteristics of the action tonic stretch reflex of normal man are absent in patients with spasticity and cerebellar lesions, presumably due to interference with long-loop pathways. In normal subjects gain of the reflex loop increases with voluntary contraction but in spasticity gain remains high irrespective of contraction level.

Athetosis

[Motor goals of therapeutic horseback riding for cerebral palsied children (author's transl)].

The article reviews in a critical survey the current opinions on the possibilities of improving the motor functions of cerebral palsied children with the help of riding therapy. Furthermore, the essential motor difficulties with spasm, athetosis and ataxia are described. It is demonstrated that only a small number of these typical difficulties can be tackled by means of riding therapy and that some key problems, particularly encountered by the spastics, cannot be solved. If, despite these facts, the favourable effects of riding on the cerebral palsied cannot be denied, then this must be attributed, first of all, to the unique psychological motivation derived from riding. Credit is given to the effects on the autonomic nervous system, the psyche, the world of experience and the behaviour.

Animals

Multilocular lesions in the therapy of cerebral palsy.

The cerebral pareses occurring in early childhood or produced subsequently by trauma or spontaneous cerebral haemorrhages, present a heterogenous symptomatic picture. In the forefront we find the spastic pareses associated with extra-pyramidal motor hyperkinesia (athetosis, dystonia, ballism). 36 C.P.-patients were surveyed from 1--4 years after operative intervention. It is shown that better results can be obtained when multilocular lesions during a single session in correspondence with the dominant group of symptoms are performed (thalamic and subthalamic target points of the extrapyramidal motor nuclei and dentate nucleus). The results are presented. Continuous physical therapy after the operation and a reasonable intellectual level are essential for improvement.

Cerebral Palsy