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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Follow-up study of patients with cerebral palsy. 15 7

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.
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PMID:[Motor goals of therapeutic horseback riding for cerebral palsied children (author's transl)]. 46 42

The dentitions of 146 children with cerebral palsy showed a high incidence of gnathic anomalies. There was, however, no relation between these anomalies and the severity of the neuropathological findings. Also the total number of anomalies was almost evenly distributed among the various clinical pictures such as spasticity, athetosis, ataxia. Although combinations of the individual anomalies were found most frequently, marked protrusions of the upper jaw were particularly impressive with 55%, and were followed by class II malformations (47%). In addition, almost every third tested child had an open bite.
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PMID:[Jaw and dental abnormalities in children with cerebral movement disorders]. 105 45

We describe the histomorphometric changes in the superficial peroneal nerve biopsy from 13 patients with xeroderma pigmentosum (XP). The mean age at time of biopsy was 15 yr (range 2.5-24 yr). The clinical examination was normal in the two youngest patients and showed absence of the deep tendon reflexes, with choreo-athetosis in ten patients. In addition, in the three oldest patients there were cerebellar signs, ataxia and Babinski signs. The nerve biopsy showed an age-dependent decrease of myelinated fibres, which was mild in the youngest and severe in the oldest patients. This was associated with rare acute axonal degeneration, sparse axonal regeneration, rare axonal atrophy and few onion bulb formations. These findings suggest a neuropathic process. This neuronal degeneration seems to be a progressive and stereotyped phenomenon in XP.
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PMID:Age-dependent axonal loss in nerve biopsy of patients with xeroderma pigmentosum. 130 Jan 84

Disease and trauma to the central and peripheral nervous system can result in various disturbances to the stability and mobility of the knee joint. The aims of orthopaedic treatment in flaccid paralysis, spasticity, rigidity, athetosis and ataxia are discussed. The effect on the musculo-skeletal system is most severe when the damage occurs prenatally. The tendency to deformity in perinatal cerebral spastic disorders of movement can be considerably reduced by early systematic functional treatment. Active and passive exercise, corrective and reflex inhibiting casts and orthoses have proved their value in the prevention and treatment of muscle contractures in flaccid paralysis and spasticity. The dangers of treatment, such as subluxation of the knee and hip joint, osteoporotic fractures, and traction paralysis, must be taken into account. Muscle contractures often hinder function, both in spasticity and in flaccid paralysis, more than the underlying disease. The operative treatment of flexion contractures and impaired knee flexion in the swing phase of the step is discussed. Gait analysis facilitates the planning of treatment. The hip, knee and ankle joints must be treated as a single functional unit.
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PMID:[Treatment concepts in knee malposition within the scope of primary neurologic diseases]. 140 26

Between 1986 and 1988 a door-to-door survey was conducted on a stable rural population of 60,820 in central Ethiopia. Trained lay health workers made a complete census and identified cases with symptoms and signs of neurological disorders, using specially designed questionnaires which, in a previous pilot study, were found to have a sensitivity of 91% and specificity of 85%. Neurological disorders in the rural population were epilepsy, postpoliomyelitis paralysis, mental retardation, peripheral neuropathy (mainly due to leprosy), and deaf-mutism with prevalence rates (cases/100,000 population) of 520, 240, 170, 150 and 130, respectively. The prevalence rates of the other less common neurological disorders were 62 for hemiparesis (15 of which were for cerebrovascular accidents), 20 for cerebral palsy, 16 for optic atrophy, 12 for perceptive deafness, 10 for tropical spastic paraparesis, 7 for Parkinson's disease and 5 for motor neuron disease, ataxia and chorea/athetosis. Among related non-neurological conditions, blindness, locomotor disability and deafness were predominant. The significance and role of such a neuroepidemiological study in laying the strategies for the prevention of neurological disorders and rehabilitation of patients are discussed in the context of a developing country.
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PMID:Community-based study of neurological disorders in rural central Ethiopia. 208 51

The author describes symptoms typical for children with bilirubin encephalopathy (the hyperkinetic patterns of infantile cerebral paralysis, audition deficiency, mental deficiency). During follow up studies (from 1 month or 1-5 years and up to 10-25 years) of children with bilirubin encephalopathy uncomplicated by prolonged asphyxia and derangement of cerebral circulation, the majority of them demonstrated the evolutional time-course of changes in motor disorders (from rigidity to athetosis and then to ataxia). Variants of neurosensory hypoacusis and peculiarities of secondary retardation of the mental development determined by audition and speech distress in children trained at the boarding++ schools are described. Preventive and treatment measures for these abnormalities are provided.
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PMID:[Bilirubin encephalopathy]. 261 30

The H-reflex was studied in 53 children with cerebral palsy, and the results were compared with those obtained for 56 normal control subjects. Pairs of identical stimuli were delivered and the time course of recovery of the amplitude of the H-reflex was determined. Recovery of the H-reflex was increased in the normal control group aged 0 to 12 mos compared to that in the normal control group aged 1 to 9 yrs, especially at interstimulus intervals from 100 to 800 msec. In children with spasticity, marked recoveries of the H-reflex were observed in both age groups, 0 to 12 mos and 1 to 9 yrs, compared to those in normal controls. In the cases of athetosis, recovery of the H-reflex was also marked. In children with ataxia, it was not pronounced and similar to that in normal controls. From these findings, an H-reflex study was considered to be useful for evaluation of central nervous system function in childhood.
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PMID:H-reflex study in normal children and patients with cerebral palsy. 406 78

Salla disease is a lysosomal storage disorder associated with increased urinary excretion of free sialic acid. The main clinical features in 34 patients were severe psychomotor retardation of early onset, ataxia, athetosis, rigidity, spasticity, and impaired speech. Growth retardation, thick calvarium, and exotropia were present in about half the patients. The amplitude of EEG decreased progressively with increasing age. Life span appears to be normal; the age range of the patients was 3 to 63 years. Genealogic studies suggest an autosomal mode of inheritance. A thin-layer method is described for the detection of increased urinary free sialic acid excretion. The basic defect is so far unknown.
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PMID:Salla disease: a new lysosomal storage disorder with disturbed sialic acid metabolism. 668 60

In cerebral palsy, principles involved in proper selection of orthopedic management of the lower limbs include diagnosis, timing, goals, degree of involvement, linkage, kinetic electromyography, retention of neonatal automatisms, and dynamic versus fixed deformity, as well as the physical plant and professional skills. Specific lower extremity problems in the hip, knee, ankle, and foot must be analyzed and management for each should be approached on a rational basis. Unsolved enigmatic problems of etiology, pathways of neonatal automatisms that impose abnormal posturing, neurophysiologic mechanisms of spasticity, athetosis, rigidity and ataxia, pathogenesis of contracture, linkage biomechanics, and the neurologic, including the biomechanical effects of surgical intervention, require special research programs.
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PMID:Current concepts of surgical management of deformities of the lower extremities in cerebral palsy. 727 32


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