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At least 289 records · Page 16Linked to original sources
PubMed1996

Single-joint rapid arm movements in normal subjects and in patients with motor disorders.

In normal subjects the execution of single rapid one-joint movements is characterized by an electromyographic (EMG) pattern composed of three discrete bursts of activity; two bursts (first and second agonist bursts, or AG1 and AG2) are present in the agonist muscle separated by an almost complete period of electrical silence. During this pause, another burst (antagonist burst, or ANT) occurs in the antagonist muscle. If a rapid movement is executed during tonic activation of the agonist muscle, tonic activity is inhibited just prior to AG1 onset (agonist inhibition). Similarly, if the movement is performed during tonic activation of the antagonist muscle, such activity is also inhibited prior to AG1 onset (antagonist inhibition). Antagonist inhibition also starts prior to AG1 onset and lasts until ANT onset. A general descriptor of the kinematic features related to the EMG pattern described above is a symmetrical and unimodal velocity profile that is bell-shaped and shows an acceleration time roughly equal to the deceleration time. This holds true for movements performed under low accuracy constraints; as accuracy demands become stricter and stricter, the peak velocity decreases but, as long as the movement is made with one continuous trajectory, the velocity profile remains roughly symmetrical. In general terms, the function of AG1 is to provide the impulsive force to start the movement; the function of ANT is to halt the movement at the desired end-point; and the function of AG2 is to dampen out the oscillations which might occur at the end of the movement. The timing and size of the bursts vary according to the speed and amplitude of the movement. The origin of the EMG pattern is a central programme, but afferent inputs can modulate the voluntary activity. In this paper, we also review the EMG and kinematic abnormalities that are present during the execution of single-joint, rapid arm movements in patients with Parkinson's disease, Huntington's disease, Sydenham's chorea, dystonia, athetosis, cerebellar deficits, upper motor neuron syndrome, essential tremor and large-fibre sensory neuropathy. The data from these studies lead us to the following conclusions: (i) the basal ganglia have a role in scaling the size of AG1, reinforcing the voluntary command and inhibiting inappropriate EMG activity; (ii) the cerebellum has a role in timing the voluntary bursts and probably in implementing muscle force phasically; (iii) the corticospinal tract has a role in determining spatial and temporal recruitment of motor units; (iv) proprioceptive feedback is not necessary to produce the triphasic pattern but it contributes to the accuracy of both the trajectory and the end-point of rapid movements.

Arm↗
PubMed1987

Seating orientations and upper extremity function in children with cerebral palsy.

This study was conducted to determine the effect of body orientation on upper extremity function in children and adolescents with cerebral palsy. Thirteen children between ages 8 and 16 and diagnosed as having spastic or athetoid cerebral palsy were placed randomly in different seating orientations (30 degrees, 15 degrees, and 0 degrees of posterior inclination and 15 degrees of anterior inclination). In each seating position, the subject performed an upper extremity activity on cue. The tests were repeated in reverse sequence. Mean performance times were different at all seating orientations for both types of subjects. Performance time was lowest at the 0-degree orientation during the retest for the subjects with spasticity and highest at 15-degree anterior inclination during the retest for the subjects with athetosis. The results of this study show that orientation of the body in space affects upper extremity function and emphasizes the importance of positioning for maximizing upper extremity function.

Activities of Daily Living↗
PubMed2006

Postoperative cervical kyphosis after atlantoaxial fixation and cervical expansive laminoplasty at one time.

Cervical kyphotic deformity is one of the well-known complications after atlantoaxial fixation or cervical expansive laminoplasty, but to our knowledge there is no information about postoperative cervical kyphosis after performing these operations simultaneously. The aims of this study were to evaluate the changes in the cervical alignment after simultaneous atlantoaxial fixation and cervical expansive laminoplasty, and to discuss the risk factors of this deformity. Five patients (1 man and 4 women) who underwent the simultaneous procedure were examined. Their mean age at surgery was 68.4+/-3.4 years, and the mean follow-up period was 30.8+/-9.5 months. Their underlying diseases were rheumatoid arthritis, and one of them had athetosis, too. On neutral lateral cervical radiographs, tangents were drawn to the inferior endplates of C2 to C5, respectively, and we measured the angles between C2 and C3, C3 and C4, C4 and C5, and C2 and C5 just after surgery and at the latest follow-up. The mean angles were 2.1, -1.9, 1.8, and 1.2 degrees, respectively, just after surgery; and 2.1, -3.5, -2.6, and -5.8 degrees, respectively, at the latest follow-up. Although the mean angle between C2 and 3 hardly changed postoperatively, the mean angle between C2 and C5 significantly decreased postoperatively. In summary, atlantoaxial fixation and cervical expansive laminoplasty may be an unsuitable combination because cervical kyphosis after the simultaneous procedure developed as a complication during the short-term follow-up.

Aged↗
PubMed2006

The effects of therapeutic taping on gross motor function in children with cerebral palsy.

PURPOSE: Therapeutic taping to address dysfunctional sitting control in children with cerebral palsy (CP) was investigated in this study. METHODS: Eighteen children with quadriplegic CP, Gross Motor Function Classification System for Cerebral Palsy levels IV (n = 9) and V (n = 9) participated in the 12-week program. Subjects were assigned randomly to one of two groups: therapeutic taping + physical therapy or physical therapy only. Therapeutic taping was applied for periods of up to 72 hours over the paraspinal region. The effects were assessed with the Gross Motor Function Measure (GMFM-88) at baseline, six weeks, and 12 weeks. A factorial analysis of variance was used to examine group differences over time. RESULTS: No significant differences were found for the GMFM-88 scores between groups over time. CONCLUSION: Therapeutic taping does not evoke a positive functional change in the seated postural control of children with quadriplegic cerebral palsy. Subjective observation, however, suggested that one child with athetosis benefited from therapeutic taping over the paraspinal region.

Back↗
PubMedDate not supplied

Use of the Green transfer in treatment of patients with spastic cerebral palsy: 17-year experience.

We retrospectively reviewed Green procedures and transfer of the flexor carpi ulnaris (FCU) in treatment of spastic forearm pronation, wrist volarflexion, and ulnar deviation deformities. Patient's ages ranged from 3 years 5 months to 16 years 5 months. Surgically, a single volar incision was made and the extensor carpi radialis brevis and/or longus (ECRB, ECRL) were used for insertion. The FCU was tensioned at neutral against gravity and immobilized in 5 degrees of dorsiflexion and 45 degrees of supination. Arc of flexion (47 degrees) did not change but was centered about neutral. Supination improved markedly when operation included a pronator procedure. Quadraplegia athetosis and intellectual impairment did not affect the operative result adversely. Patients aged greater than 12 years showed less functional improvement. Eighty-eight percent had cosmetic improvement, and 79% improved functionally. None had a decreased functional rating.

Adolescent↗
PubMed1991

Drug-induced and tardive movement disorders.

Drug-induced and tardive movement disorders represent a large number of extrapyramidal disorders seen in neurologic practice. Iatrogenically induced, most commonly by neuroleptics, these disorders can be characterized by any abnormal body movement including tremor, chorea, athetosis, dyskinesias, dystonia, myoclonus, tics, ballismus or akathisia. Parkinsonism, dyskinesias and dystonia tend to be the most common. Management of patients with drug-induced or tardive syndromes is complex. Prognosis is frequently poor as patients usually need the offending agent to manage their underlying psychiatric or medical problem. Neuroleptics and other drugs known commonly to cause movement disorders should be used cautiously and significant consideration of all risks and benefits measured before initiating therapy.

Dyskinesia, Drug-Induced↗
PubMed1991

Propionic acidaemia: a neuropathological study of two patients presenting in infancy.

The clinical and neuropathological findings are described in two children with propionic acidaemia presenting in infancy; they survived for 4 and 16 years respectively. Abnormalities were noted in the basal ganglia of both patients. In one child who clinically had severe athetosis, there was marbling of the corpus striatum. Clinical and morphological evidence suggests that the basal ganglia are especially vulnerable in propionic acidaemia.

Acidosis↗
PubMed1992

Oculocerebral syndrome with hypopigmentation (Cross syndrome): report of a new case.

A syndrome of ocular and cutaneous hypopigmentation, severe mental retardation with spastic tetraplegia and athetosis was first observed by Cross in three siblings of an inbred Amish family. Since then, seven other patients, three sporadic and four with familial recurrence, have been reported in the literature, confirming the autosomal recessive inheritance. The clinical spectrum of the syndrome has been expanded to include true developmental defects of the CNS such as cystic malformation of the posterior fossa of the Dandy-Walker type. We report a new case of Cross syndrome.

Brain↗
PubMed1975

Stereotaxic neurosurgery in the management of cerebral palsy.

Ten children with cerebral palsy are presented on whom stereotaxic operations on the central nervous system were performed with the aim of ameliorating athetosis and spasticity. Tere were seven alert and co-operative children with spastic hemiplegia or diplegia, of whom six received benefit from thalamotomy or dentatotomy. The seventh, a child with diplegia, had improvement of his left lower limb, but the right became worse. One child with spastic diplegia, in whom a thoracic meningocoele had been closed at birth, was not improved by bilateral dentatotomy. Two severely quadriplegic children each had bilateral dentatotomy; one was a child with dystonic and spastic quadriplegia. In both cases the resulting reduction in tone and extensor spasm rendered the nursing of these patients much easier. The place of stereotaxic surgery in the central nervous system in the management of children with cerebral palsy is discussed. We suggest that in selected cases the stereotaxic operation should be performed early in order to gain the greatest benefit. Stereotaxic surgery should be regarded as an integral part of the management which involves close co-operation of paediatrician, physiotherapist, neurosurgeon and orthopaedic surgeon.

Cerebellar Nuclei↗
PubMed1981

Etiological factors in cerebral palsy: an historical review.

The etiological factors in 1503 patients with cerebral palsy seen since 1947 are listed. Prematurity was the most prominent factor, being present in 27.8 per cent of cases. 38.5 per cent of the causes were prenatal in origin, 46.3 per cent natal, and 15.2 per cent postnatal. Since 1947, the number of patients seen rose to a maximum in the 1950s and has fallen since. A steadily increasing percentage of the patient population has been children with spasticity, especially paraplegia and quadriplegia. Although small, the atonic group has shown a marked increase, from 0.8 per cent in the 1950s to 3.4 per cent in the 1970s. There has been a dramatic decrease in the incidence of athetosis, from 38 per cent to about 3 per cent, and a slight decrease in the rigidities. The incidence of some of the etiological factors has increased, such as hydrocephalus, prematurity, trauma and multiple birth. There has been a striking decrease in the incidence of erythroblastosis as a factor, which dropped to nil in the 1970s. There have been lesser decreases in encephalitis, dystocia and idiopathic factors.

Birth Injuries↗
PubMed1996

Magnetic resonance imaging in children with neonatal asphyxia: correlation with developmental sequelae.

Magnetic resonance (MR) findings and developmental sequelae were examined in 60 patients with neonatal asphyxia. Nine patients with marked destruction of the cerebrum had the severest motor and mental disability. Six had lesions of the periventricular white matter and thalamus. Seven had border zone infarction with lesions of the white matter and thalamus. Nine had lesions of the middle-lateral part of the thalamus, putamen and peri-Rolandic area. Five with lesions of the thalamus, putamen and cerebral white matter had the severest motor and mental disability. Twenty with lesions of the thalamus and/or putamen had athetosis or clumsiness. Four with lesions of the opercular region, white matter and thalamus had feeding difficulty. Some correlation between MR findings and developmental sequelae was recognized.

Adolescent↗
PubMed1981

Pathophysiology of dysarthria in cerebral palsy.

Electromyograms were recorded with hooked-wire electrodes from sixteen lip, tongue and jaw muscles in six normal and seven cerebral palsied adult subjects during a variety of speech and non-speech tasks. The recorded patterns of muscle activity fail to support a number of theories concerning the pathophysiology of dysarthria in cerebral palsy. There was no indication of weakness in individual articulator muscles. There was no evidence of uncontrolled sustained background activity or of abnormal tonic stretch reflex responses in lip or tongue muscles. Primitive or pathological reflexes could not be elicited by orofacial stimulation. No imbalance between positive and negative oral responses was observed. The view that random involuntary movement disrupts essentially normal voluntary control in athetosis was not supported. Each cerebral palsied subject displayed an idiosyncratic pattern of abnormal muscle activity which was reproduced across repetitions of the same phrase, indicating a consistent defect in motor programming.

Adult↗
PubMed1982

Self-regulation of spasm and spasticity in cerebral palsy.

Four young adult cerebral palsied subjects with a mixture of spasticity and athetosis attended an experimental reflex training program for three one-hour sessions each week over an 18 month period. During each session on-line measures of contraction level and tonic stretch reflex sensitivity from the biceps brachii muscle were shown to the subject on meter displays. Subjects were asked to attempt to control the displays. They were given goals such as: (1) reduce both contraction level and reflex sensitivity displays to zero and (2) increase the contraction level display to 10% of maximum while keeping the reflex sensitivity display at a minimum. Achievement of goals was automatically sensed and used to activate a cassette tape of the subject's favourite music. Contraction level and reflex sensitivity scores were averaged over one-minute intervals to provide a record of long term progress. Elbow-angle and IEMG data were recorded on FM tape for off-line analysis. All four subjects learned to suppress involuntary muscle activity and resting tonic stretch reflex responses. They also learned to produce a two or three-fold variation in action tonic stretch reflex sensitivity while sustaining 10% maximum voluntary contraction. In other words, subjects learned to self-regulate spasm and spasticity at the elbow and to regulate tonic stretch reflex sensitivity independently of contraction level. A visual tracking task requiring voluntary movement about the elbow was employed to assess improvement in functional control of elbow movement. One athetotic subject improved tracking accuracy as a consequence of reducing the amount of involuntary arm movement while the other three subjects showed negligible improvement in functional control.

Biofeedback, Psychology↗
PubMed2001

A new neurological entity manifesting as involuntary movements and dysarthria with possible abnormal copper metabolism.

A few patients with an affected CNS involving abnormalities in copper metabolism have been described that do not fit any known nosological entities such as Wilson's disease or Menkes' disease. Three sporadic patients (two men and one woman) were examined with involuntary movements and dysarthria associated with abnormal concentrations of serum copper, serum ceruloplasmin, and urinary copper excretion. The onset of neurological symptoms occurred at the age of 15 to 17 years. The common clinical symptoms were involuntary movements and dysarthria. The involuntary movements included dystonia in the neck, myoclonus in the shoulder, athetosis in the neck, and rapid orobuccal movements. The dysarthria consisted of unclear, slow, and stuttering speech. Two of the three patients did not have dementia. A cousin of the female patient had been diagnosed as having Wilson's disease and had died of liver cirrhosis. Laboratory findings showed a mild reduction in serum copper and ceruloplasmin concentrations, whereas urinary copper excretion was significantly reduced in all three patients. Two of the three patients showed a high signal intensity in the basal ganglia on T2 weighted brain MRI. In conclusion, the unique findings of involuntary movements, dysarthria, and abnormal serum copper and urinary copper concentrations suggest that the three patients may constitute a new clinical entity that is distinct from either Wilson's or Menkes disease.

Adult↗
PubMed1990

Stereotactic surgery for cerebral palsy.

The results of stereotactic surgery in 38 patients with cerebral palsy are reviewed. A total of 67 nuclei were approached and 109 stereotactic lesions performed. The motor disorders were spasticity, athetosis, dystonia and tremor. The targets were chosen according to the motor disorder, and included the pulvinar, ventrolateral (VL), sub-VL and cerebellar dentate, and a combination of 2 or 3 of these. The results of the surgery were evaluated on the basis of objective functional improvement using a scale for task achievement. Varying degrees of overall improvement were found in over 80% of the patients; these are discussed in detail.

Adolescent↗
PubMedDate not supplied

Thalamic lesions during the development period and their clinical correlation.

28 autopsy cases were investigated in which the thalamus was impaired in early life, i.e. prenatal, perinatal, and postnatal (under 1 year of age). The thalamus is vulnerable to brain damage in the perinatal and early postnatal period. Lesions in the thalamus are severe in the DM, PL and VPL, and moderate in the VL and pulvinar thalamus. The anterior nuclei and center median show little change. Many of the cases with athetoid movements have complicated thalamic lesions in addition to those of the basal ganglia. In one patient who showed athetosis as a sequela of encephalitis japonica, the VL and PL in the thalamus were involved severely; in contrast the basal ganglia revealed very slight changes.

Adolescent↗
PubMed1987

Cerebral palsy spasticity. Selective posterior rhizotomy.

We have performed selective posterior rhizotomies on 60 children with cerebral palsy. The procedure involves lumbar laminectomy with stimulation of the rootlets (fascicles) of the second lumbar to the first sacral posterior roots bilaterally; those rootlets associated with an abnormal motor response, as evidenced by sustained or diffused muscular contraction, are divided leaving intact rootlets associated with a brief localized contraction. The patients were between 20 months and 19 years of age representing all degrees of physical handicap and ranging from profoundly retarded to normal intelligence. Follow-up has been from 1 to 5 years. Each child was assessed pre- and postoperatively and graded in the following categories: muscle tone, power, sitting, standing, walking, upper limb function, and speech. The patients whose function improved most dramatically following rhizotomy were purely spastic and intelligent, were significantly more affected by spasticity in the lower than the upper limbs, had some degree of forward locomotion, and could side-sit independently. Patients with severe athetosis or marked contractures improved least.

Adolescent↗
PubMed1967

The effects of azathioprine (imuran) on purine synthesis in clinical disorders of purine metabolism.

Azathioprine, a purine analogue, significantly suppressed the purine synthesis de novo of two gouty patients manifesting overproduction of uric acid, as well as three of four gouty patients who showed normal uric acid production. This suppression is taken as evidence that phosphoribosyl-pyrophosphate amidotransferase, the rate-controlling step in purine synthesis de novo, has a normal sensitivity to feedback inhibitors in the patients who responded to the drug.Two children afflicted with the familial disorder of hyperuricemia, choreo-athetosis, and self-mutilation described by Lesch and Nyhan showed no reduction in the activity of the biosynthetic pathway in response to azathioprine. This inability to respond to azathioprine can be directly related to the absence in these patients of the enzyme hypoxanthine-guanine phosphoribosyltransferase which is required for conversion of the drug or its metabolites to the biochemically active ribonucleotide form.

Journal Article↗