[Prespasm of accommodation].
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The authors present a review of the methods of surgical treatment of spasmodic torticollis, particularly the selective denervation of cervical muscles. Based on the literature and own experience cases treated by means of this method are described. Using selective ramisectomy it is possible to attain permanent improvement in most patients suffering from spasmodic torticollis, with very low complication rate. Our preliminary cases indicates that this method of treatment allows to acquire improvement of quality of life in patients with this particular type of dystonia.
64 cases with spasmodic torticollis were observed during 5 years and treated with botulinum toxin (BTX). BTX was injected into dystonic muscles mostly into sternocleidomastoid then--trapezius, and splenius capitis muscle. Improvement (excellent, good and fair) was achieved in 40 patients (62%). Lack of information about 6 patient (9%). Injections were repeated every 3-4 months and in several cases even 1-2 during the year. After several injections atrophy and denervation potentials in EMG were observed in the majority of injected muscles. Neurotic syndromes coexisting with dystonia had worsening influence on therapeutic effects. Adverse events were observed in 5 cases. Treatment with BTX is very simple, easy, harmless and can be administered in outpatients.
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To elucidate the role of the basal ganglia on neck movement control and thus clarify the side of targets for stereotactic surgery of spasmodic torticollis, effects of electrical stimulation to the globus pallidus-entopeduncular nucleus complex (GP-EP) upon the activities of the sternocleidomastoid (SCM) muscle motoneurons were studied in cats. Repetitive stimulation of the ipsilateral GP-EP increased discharge rate in the majority of the SCM motoneurons, whereas contralateral GP-EP stimulation caused a decrease in discharge rate. These effects were gradually enhanced in the course of repetitive stimulation and lasted even after its cessation. Bilateral ablation of the motor and premotor cortices did not influence the effects of GP-EP stimulation. Latency studies suggested that the pallidal control of the SCM motoneuron is mediated by polysynaptic pathways on both sides; however, these same studies produced evidence of an ipsilateral oligosynaptic connection, as well. Repetitive antidromic stimulation of the accessory nerve caused a gradual increase in discharge rate of the SCM motoneurons, similar to the augmentation induced by ipsilateral GP-EP stimulation. Features of the responses indicate that the pallidal control on the neck muscles involves reverberating circuits in the brain stem and spinal cord. These experimental findings suggest that stereotactic thalamotomy of the ventrolateral nucleus for spasmodic torticollis should be performed on the side ipsilateral to the contracting SCM muscle.
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Thirty-one patients who had transfer of the flexor digitorum superficialis tendons to the flexor digitorum profundus tendons en masse in thirty-four non-functional spastic hands were examined at an average of fifty months postoperatively. All of the patients had had a clenched-fist deformity preoperatively, with severe hygienic problems of the palmar skin and no active function of the hand. Postoperatively, all of the hands were in an open position, which allowed for good hygiene of the palmar surface. A minor wound infection developed in three patients. Neurectomy of the motor branch of the ulnar nerve distal to the Guyon canal was needed for control of spasticity of the intrinsic muscles in twenty-five hands. An intrinsic-minus deformity did not develop in any of the hands that had neurectomy of the ulnar nerve, although an intrinsic-plus deformity developed in seven of the nine hands that did not have a neurectomy.
The surgical management of functional esotropias is now very different from some years ago with Fadenoperation settled by Cüppers. Indeed this procedure is the only one which can act in a fitted manner on muscular spasms responsible of more than 60% of convergent squints. The simultaneous using of classical technics remains necessary, but in very different way from the custom formely. In the first part of this work, the authors begin to analyze the mechenism of medical rectus recession, and they show that operation doesn't act by a modification in arc of contact as it was always said, but only by a muscular slacking. On the contrary the change in arc of contact is the main factor which intervenes in Fadenoperation, but the muscle exclusion has also an important part. Whereas the effects of Fadenoperation on adduction incomitance are perfectly clear, those on the deviation in primary position still remain very abstruse. A statistical report on 143 cases of operated blocking esotropias shows that often Fadenoperation alone does not bring a complete cure of motor troubles. The authors explain the reasons of these failures and point out the other accurate muscular actions which must be associated to obtain a good result.
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Spasmodic torticollis is often a severely disabling disorder which can be extremely resistant to any form of treatment including newer methods employing electromyographic biofeedback. This case study describes the diagnostic and treatment methods utilized with a 63-year-old woman with a 6-month history of spasmodic torticollis. Several treatment approaches were attempted, including biofeedback, relaxation training, and a combination fading desensitization approach. All corrective measures were unsuccessful until serendipitous circumstances suggested a treatment employing both a particular movement pattern that relieved the problem in the total behavior chain associated with spasmodic torticollis.
A 7 1/2-month-old boy had an anterior inferior dislocation of the shoulder. Dislocations of the shoulder in infants have previously been reported, and were either congenital dislocations with associated anomalies of the glenohumeral joint with dislocations from Erb's palsy or septic arthritis. In the present case, dislocation of the shoulder was associated with spastic brain damage. The marked spasticity of the deltoid muscle may have been the deforming force. Capsular and subscapularis plication plus recession of the deltoid corrected the deformity and prevented further dislocations.
We present a consanguineous Pakistani family in which four patients (two males and two females) had a new Troyer-like phenotype. All four patients showed some marfanoid features (span more than height, arachnodactyly, high arched palate), and generalized hyper-reflexia. The two affected males and the younger female also had microcephaly and mental retardation. Features only present in the affected males included short stature, dysarthria, amyotrophy of the distal muscles, fasciculations and tremor. The distal muscle wasting in the two affected brothers reflected the presence of axonal neuropathy demonstrated both electrophysiologically and by nerve biopsy. Although neither of the sisters had any degree of distal muscle wasting, both had reduced M-wave amplitude of the tibial and peroneal nerves, bilaterally. The described phenotype does not fit any of the recognized forms of hereditary spastic paraparesis with distal muscle wasting. The specific axonal neuropathy differentiates it from familial motor neuron disease with mental retardation. The reported phenotype represents a possible new Troyer-like syndrome similar to that described by Neuhäuser (1976), but differs from it by the lack of major dysmorphic features.
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