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Hemifacial spasm. A vascular cause.

A review of the literature as well as recent surgical observations indicate that hemifacial spasm may be caused by normal or pathological vascular structures that cross-compress the facial nerve. The critical area of compression is invariably found at the brain stem exit zone of the seventh cranial nerve. In this area the central glial investment of the facial nerve changes to peripheral or schwannian myelin. It is suspected that this anatomical junction zone may be of pathophysiologic significance when directly compressed or irritated. A retromastoid craniectomy and vascular decompression operation is highly successful in relieving hemifacial spasm while at the same time preserving facial nerve function. This is in contrast to most commonly used destructive operations for hemifacial spasm. Microsurgical techniques, however, must be employed or high morbidity and mortality may occur from the retromastoid approach.

Cerebrovascular Disorders

[Nonaneurysmal vascular pressure lesions of the cranial nerves (author's transl)].

Four cases of facial spasm and 1 case of oculomotor paresis are described. The source of the disorder in all 5 cases is mostlikely not an aneurysm of the vessels of the base of the brain. The literature is discussed and thereby it is shown that mechanical disturbances of other cranial nerves (II, V, VI, IX, XII) can be caused by similar vascular (nonaneurysmal) abnormalities.

Aged

Diagnostic problems in extrapyramidal disorders.

The diagnostic problems of extrapyramidal disorders (ED) are reviewed. Many of the wide range of ED occur rarely, and clinical experience is difficult to obtain. Despite great advances in pathophysiology and pathological anatomy the diagnosis of ED is still mainly a clinical diagnosis, and the diagnostic problems are discussed principally in relation to the involuntary movements and partly with regard to muscle tone. Except in a few diseases, biochemical and microbiological analyses, EEG, EMG and X-ray examinations offer little contribution to the solution of diagnostic problems in these disorders.

Adult

Meiges disease: a clinical form of facial convulsion, bilateral and medial.

In 1910 the French neurologist Henry Meige described in detail a disorder characterized chiefly by symmetric dystonic spasms of the facial muscles, which he called "spasm facial median." Cases with this disorder are rare, and frequently misdiagnosed and inappropriately treated. We report here a translation of Meige's original description of "spasm facial median." We hope that this translation will make it easier to recognize and diagnose this disorder and that it will stimulate greater interest in this unusual syndrome.

Dystonia

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

Hypothenar Dimpling. A peripheral equivalent of Hemifacial Spasm?

In two patients, the skin over both hypothenar eminences underwent intermittent, spontaneous, irregular, dimpling contractions. The dimpling was benign, and was the result of spontaneous discharge of motor units in the palmaris brevis muscle. Electrophysiological investigations localized the site of origin of the discharge to the ulnar nerve, possibly at the wrist, but there was no clinical or physiological evidence of neuropathy or of nerve compression. In many respects, the clinical and electrophysiological features of hypothenar dimpling resemble hemifacial spasm.

Aged

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

Stuttering and tics in twins.

A total of 63 twin pairs (22 MZ and 41 DZ), with one or both members affected by not organic stuttering and/or tics, have been drawn out of the Mendel Institute's twin file. Concordance of stuttering was observed in 10:12 MZ, but only in 2:19 DZ twin pairs; concordance of tics in 6:10 MZ and in 2:22 DZ twin pairs. Stuttering did not come out significantly associated either with other disturbances of language or with an IQ under the average; almost always it was possible to find out a precipitating factor. Significant relations between stuttering and tics could not be stated. Sex ratio, obtained regarding any affected subject separately, clearly turned in favour of males both in stuttering (32:12) and in tics (27:12). Stutterers and left-handers incidence in the families of stuttering subjects appeared significant in comparison with families of subjects with tics.

Adolescent