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Hemifacial spasm: importance of a complete investigation.

The authors report the experience of the Clinique d'O.R.L. de l'Université de Bordeaux II in the management of hemifacial spasm. The recent diagnostic and therapeutic progress in otoneurology has revealed an organic etiology for many cases of hemifacial spasm. The recent diagnostic and therapeutic progress in otoneurology has revealed an organic etiology for many cases of hemifacial spasm. From their experience, they propose a complete investigation for all cases of hemifacial spasm. The disorder is labelled as being idiopathic only if the complete investigation is negative.

Adult

Tardive dyskinesia and the long-term patient.

A psychiatric patient's long-term use of antipsychotic medication often results in the irreversible movement disorder, tardive dyskinesia. The author uses a composite case history as a basis for discussing the symptoms, diagnosis, epidemiology, and treatment of tardive dyskinesia. A number of drugs have been used to treat the disorder, but so far none have been effective. While tardive dyskinesia cannot be cured at this time, the author believes that it can be prevented by treating psychoses with the lowest possible dose of the least toxic drug for the shortest length of time.

Adult

Hemifacial spasm due to aneurysmal compression of the facial nerve.

Hemifacial spasm developed in a woman as the only symptom of an aneurysm of the posterior inferior cerebellar artery. Using microdissecting techniques, the aneurysm was clipped and moved from its distorting position at the brain stem exit zone of the seventh nerve. After surgery, she experienced immediate relief of her facial spasm of six years' duration. This case supports the finding that hemifacial spasm may be caused by vascular lesions of the seventh nerve at the brain stem junction. Recent surgical experience indicates that the majority of the hemifacial spasm cases may be due to normal but ectatic blood vessels that cross-compress the most proximal portion of the seventh nerve. Relief may be affected without facial paralysis by a retromastoid microvascular decompressive procedure.

Arteries

Significance of genetic factors in Gilles de la Tourette syndrome: a review.

Observations suggesting a genetic basis for Gilles de la Tourette syndrome are reviewed with particular emphasis on the finding of familial aggregation. Studies of both Tourette syndrome and simple tic have found that approximately 30% of patients have a positive family history of tic. The significance of this figure depends on a number of factors, in particular the prevalence of positive tic histories in the population. If the latter figure is 10%, which the best available evidence suggests is a reasonable estimate, approximately 30% of families in the general population would be expected to contain at least one present or former tiquer. It is argued, therefore, that the family aggregation findings in Tourette syndrome do not support the hypothesis that the condition has a significant genetic component. Methodological considerations for future research are discussed.

Female

Motor tics of the head and neck: surgical approaches and their complications.

Motor tics of the head and neck, especially hemifacial spasm and spastic torticollis, are the substance of this paper. Forty-six cases are presented, and surgical techniques are described. In hemifacial spasm the intracranial neurovascular lysis of Jannetta is a valid operation with the best results to date but has a 7 1/2% risk of unilateral deafness. The extracranial submastoid partial section of Scoville is completely safe and gives excellent results, but there is a probability of mild to moderate return of the spasm in one to two year's time. In spastic torticollis the accepted radical operation consists of bilateral anterior rhizotomy of the upper three roots plus bilateral spinal accessory nerve section in the neck. A tragic complication of this operation has recently been observed by ourselves, Sweet, and Hamlin. This complication is bilateral infarction of the medulla (bilateral Wallenberg's syndrome). This has also been reported as occurring following chiropractic manipulations. For this reason the writer does limited unilateral sectioning of the spinal accessory nerve in the neck and resection of the upper third of the sternomastoid muscle, as a first stage procedure, in those cases in which rotation of the neck is the principal symptom, before doing the radical operation. Safeguards to prevent this complication include preoperative vertebral arteriography and preservation of both motor and sensory radicular arteries under magnification and maintenance of adequate neck support during the early postoperative days.

Adult

Brief family therapy for childhood tic syndrome.

This paper reports the success of brief analytically oriented outpatient family therapy exclusively in treating an eight-year old girl presenting with a short history of multiple tics involving facial, thoracic, and upper limb musculature, with associated hoarse coughing and grunting. Diagnostic features are reviewed. No medicines were used, and the patient remained asymptomatic nine months after ceasing family therapy. Tentative indications for family therapy for tiqueurs are proposed.

Child

The theory and therapy of hyperkineses (torticollis).

The paper presents the interaction approach in psychosomatics, as developed by the author. In this context the physical proceedings, which are oversome by the categories of the structure of space, differ from the psychic ones which are not of space but of time. The psyche itself is understood as an intentional experience, wherein the affects and unconscious proceedings are essential. Language has an important function. The psychosomatic disease is understood as a presentative symbolic event and as compensation for the loss of the cognitive ability to symbolize in the field of language. The problem of symbolization is illustrated by a case of spasmodic torticollis. The results of an examination of 81 patients are given, 23 of whom were treated psychoanalytically. The object relation, the regression to the pregenital level, the ego-regression in the form of the omnipotence of motion, and the inability for developing an own self are presented. An alternative technique of treatment is pointed out. Finally, the results of an examination of 26 cases of tic, including a case of generalized tic, are described.

Adult

[Pathogenesis of hyperkinesis in children].

Pheylalanine metabolism was studied in 56 children with various forms of hyperkinesias. It was found that in the development of slow and fast hyperkinesias a certain role belongs to dihydroxyphenylalanine (DOPA). It is probable, that in patients with Turett's syndrome the synthesis of DOPA is increased while in patients the excretion of phenylacetylglutamine was found to be disturbed: it was decreased in the patients with the fast and increased in the children with the slow hyperkinesias. Phenylalanine load led to a lowering of the DOPA level in the patients with Turett's syndrome; an intensification of the synthesis of phenylacetylglutamine and diminution of the intensity of hyperkinesias. L-glutamine load resulted in detoxication of the toxic phenylalanine metabolites which inhibited the DOPA synthesis, as well as in a short-time increase in the phenylacetylglutamine excretion and a moderation of tonic hyperkinesias that manifested by athetosis and dystonia. All this points out that in the development of various forms of hyperkinesias a certain role belongs to amino acid metabolites.

Adolescent

[Facial myokomias in brain stem tumors].

Four cases of brain-stem tumor were described, in which myokymias were electromyographically derived from the region of several cerebral nerves. An attempt is made to interpret the effects of twitching by reference to the electromyogram. Myokymias are a positive indication of organic brain stem damage. The use subtle techniques of electromyographic examination shows that this muscular hyperkinesis is not so infrequent a symptom as is generally believed.

Abducens Nerve