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Botulinum toxin therapy has emerged as a treatment modality for a variety of spastic- or contracture-related muscle diseases. Its safety has been proven for long-term use in the treatment of benign essential blepharospasm, hemifacial spasm, and certain types of strabismus. Recent approval from the Federal Drug Administration should make botulinum toxin available for use in a greater number of patients.

Except in Wilson's disease, few secondary dystonias are susceptible te benefit from an etiological treatment. The somatic distribution of dystonia often determines the therapeutic strategy. Thus, stereotactic surgery may be the treatment of choice for hemidystonia while anticholinergic medication may alleviate generalized dystonia, particularly in childhood. Finally, local infiltrations of botulinum toxin are particularly useful for various forms of local and segmental dystonia. Certain subsyndromes as myoclonic dystonia, levodopa sensitive dystonia and paroxysmal choreoathetosis may benefit from relatively specific treatment strategies.

Thalamic stimulation for the treatment of tremor and other motor movement disorders is attractive in view of the fact that it is a non-destructive procedure. With the experience of 26 cases indications can be defined. The method is an alternative for treatment of tremor only when thalamotomy carries a high risk of complications. Not only is the cost a limiting factor but also repeated operations for replacing the neuro-pacemaker and thus the risk of infection have to be taken in consideration.

We have studied the effect of botulinum toxin in patients with focal dystonia, not responding sufficiently to medical therapy. Injection of 5-100 units to the muscles, selected by EMG and clinical examination, invariably resulted in reduction of muscle tonus, although to a different degree depending on the dosage. The outcome after the first trial was best when dealing with small muscles in patients with blephalospasm or Meige's syndrome. In contrast, several attempts were required for the treatment of a large muscle as in torticollis, or multiple muscles as in writers' cramps. The effect lasted 1-3 months. With precise selection of the affected muscles and careful regulation of necessary dosages, this type of therapy may contribute to the treatment of focal dystonia.

In the past five years, 477 patients with various focal dystonias and hemifacial spasm received 3,806 injections of botulinum A toxin for relief of involuntary spasms. A definite improvement with a global rating greater than or equal to 2 on a 0-4 scale, was obtained in all 13 patients with spasmodic dysphonia, 94% of 70 patients with blepharospasm, 92% of 13 patients with hemifacial spasm, 90% of 195 patients with cervical dystonia, 77% of 22 patients with hand dystonia, 73% of 45 patients with oromandibular dystonia, and in 90% of 21 patients with other focal dystonia who had adequate follow up. While the average duration of maximum improvement lasted about 11 weeks after an injection (range seven weeks in patients with hand dystonia to 15 weeks in patients with hemifacial spasm), some patients benefited for over a year. Only 16% of the 941 treatment visits with follow up were not successful. Except for transient focal weakness, there were very few complications or systemic effects attributed to the injections. This study supports the conclusion that botulinum toxin injections are a safe and effective therapy for patients with focal dystonia and hemifacial spasm.

Occupational cramps affecting the upper extremities have been described for centuries, though only recently is light being shed on their pathogenesis, pathophysiology, and improved strategies for diagnosis and treatment. These dystonias represent localized stereotyped painless movement disorders affecting certain occupational groups in characteristic ways. We retrospectively reviewed 65 patients given a diagnosis of focal dystonias of the upper extremity and describe painless manual incoordination syndromes, insidious in onset, without objective sensory abnormalities, precipitated by certain repetitive actions of the upper extremity. This confirms previous clinical reports. Pathogenesis and pathophysiology are still uncertain, but evidence for altered higher order (basal ganglia and cortical) influences on spinal interneurons leading to ill-directed motor output is hypothesized. Therapeutic options are reviewed stressing the inadequacy of any one mode discovered to date.

Results are reported of a study of 55 males with disorders of sexual potency with an objectively registered manifestation of vegetative dystonia. It was shown that sexual disorders in these patients have a psychogenic character. All of them showed neurosis and neurosis-like symptoms. With the purpose of finding effective treatment psychotherapy, phallic stimulation and physiotherapy were tried. Most effective was individual psychotherapy with elements of hypnotic suggestion and phallic vascular stimulation by local negative pressure.

It has been established that peripheral trauma can cause focal and generalized dystonias, which may be difficult to treat. The case presented of a 28-year-old with post whiplash focal dystonia shows that transcutaneous electrical nerve stimulation (TENS) may be a useful treatment in the early management of focal dystonias which fail to respond to conventional therapy.

An improvement in tardive dystonia in a patient who had received ECT for a schizophrenic psychosis is reported. The improvement suggests that the pathophysiology of tardive dystonia may involve neurotransmitter receptor changes similar to those seen in schizophrenia.

Injections of botulinum toxin into the main cervical muscles responsible for abnormal posture and movements in spasmodic torticollis reduced pain and attenuated dystonia for a period of 2 months on average. After several sessions 9 out of 36 patients (25 p. 100) felt they had improved by at least 50 percent, 16 (44 p.100) by 50 to 75 percent, and 6 (17 p. 100) by more than 75 percent. There were six failures.

The diagnosis of dystonic movements is based on the phenomenologic characteristics of the movements themselves. Therefore to evaluate and manage the patient, it is important to be cognizant of the varied clinical appearances; to classify the type of dystonia according to age at onset and distribution of the movements; to determine its etiology; to treat with specific therapy if available; and to reduce the symptoms, if therapy is not available. These steps are the focus of this article.

Marked, abrupt and disabling oscillations in motor performance--the "on-off" phenomenon--frequently occur in the course of long-term levodopa therapy for Parkinson's disease. Although these fluctuations are usually refractory to available medications it has recently been suggested that electroconvulsive therapy (ECT) may be beneficial Five Parkinsonian patients with incapacitating and unpredictable "on-off" changes who were free of significant depression were given conventional bilateral ECT while usual drug regimens were maintained. While ECT was well tolerated, after a total of six treatments no significant improvement in Parkinsonian disabilities or "on-off" changes were seen.

Four infants suffering from benign non-fluctuating, idiopathic dystonia with onset in the first year are presented. The discussion includes the relationship to childhood-onset dystonia, the significance of the tremor, the neurotransmitters in the CSF, spontaneous recovery, the risk of recurrence and the preference not to intervene orthopaedically.

Unlike tardive dyskinesia (TD) which is much better known by clinicians, tardive dystonia is a more recently recognised complication of neuroleptic use. It refers to chronic dystonia related to the use of neuroleptic drugs and may be an even more disabling condition than TD. This article reviews its epidemiology, clinical features and treatment aspects, and suggests that it should be separated from TD as a distinct entity.

The use of a transcutaneous nerve stimulator yielded positive results in a condition marked by involuntary movements (tremors and myoclonias) and dystonia. In the absence of clues to the pathophysiology of the myoclonus and dystonia, it is argued that transcutaneous stimulation induces an action on the neurotransmitters that inhibits neuromuscular function.

For more than 100 years there has been a discussion as to whether writer's cramp is caused by a disease of the central nervous system, or if it is to be considered as a disturbance of psychogenic origin. Whereas before 1982 there seemed to be a lot of evidence for the psychogenic theory many authors now tend to stress the opinion of Sheehy and Marsden who had explained that "writer's cramp" should be seen as a "focal dystonia". This article discusses the statements of Sheehy and Marsden in comparison with other scientific findings regarding the occupational cramps--above all "writer's cramp". It is concluded that there are different therapeutic approaches derived from the respective theory and which are considered to lessen the movement disorder. Scientific literature suggests that psychotherapy or some therapeutic approaches of behaviour therapy are more effectful in mastering this "mysterious" disorder than any pharmacological substance tested upto now.

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.

Musculoskeletal problems are common in instrumental musicians. Most of these problems can be classified as musculotendinous overuse, nerve entrapment/thoracic outlet syndrome, or motor dysfunction. Also seen in musicians are problems related to hypermobility and degenerative arthritis. Although these problems are seen in all instrumentalists, their prevalence is highest in professional musicians, with string players most commonly affected by musculotendinous overuse. Keyboard players are the performers most commonly affected by motor dysfunction. History and physical examination performed with an understanding of the problems of musicians are usually adequate to make the correct diagnosis. Electrophysiological studies are often helpful in confirming or excluding a diagnosis of nerve entrapment. With the exception of motor dysfunction, once these problems are recognized, they can be adequately treated. Treatment should begin conservatively with rest, evaluation of technique and practice habits, and possibly nonsteroidal antiinflammatory drugs. Depending on the type and severity of the problem, physical therapy, adaptive devices, steroid injection, or surgery may be indicated. A strong partnership with music educators is important in the management and prevention of these musculoskeletal problems.

We report a patient with torsion dystonia whose polysomnographic recordings revealed poor sleep and a pronounced and almost continuous type of spindle activity during non-rapid eye movement sleep. Rapid eye movement sleep was also reduced. These changes proved to be independent of medications. After a clinically successful unilateral thalamic operation, a normalization of sleep parameters and a reduction of the high amplitude sleep spindles was observed, implying that the regulation of sleep spindles and the advancement of dystonic symptoms are affected by a common mechanism.

A patient is described with myoclonic dystonia, a disorder characterized by a combination of dystonia and myoclonic contractions. A very good response to epidural cervical cord electrical stimulation was obtained. The findings in this case indicate that epidural cervical cord electrical stimulation could be a useful therapeutic approach in cases of myoclonic dystonia.

Torsion dystonia in 13-year-old girl progressed to severe opisthotonus and dystonic posturing of the limbs, accompanied by severe psychic changes, cachexia and calcification around joints. Drug therapy was ineffective. Ventrolateral thalamotomy and chlorpromazine brought about a delayed but remarkable amelioration of all symptoms and signs, still present 4 years later.

This report is a continuation of our previous report on chronic spinal cord stimulation in disorders of the motor system. Improved statistics were observed following changing from the early conventional two-electrode system to a newly developed four-electrode system. The success of the four-electrode system lies in the analysis of both the electrode combinations and the frequency of stimulation. These are carried out on each patient and remain the most critical aspect of the technique. The details of the multiple lead system--the technique of implantation and protocols for carrying out electrode and frequency analysis--are presented.

The position of the medial internal capsular border (lateral thalamic border) in relation to the width of the third ventricle is of considerable importance to stereotactic surgeons. Close estimation of its position from anteroposterior roentgenograms diminishes the possibility of multiple brain penetrations in an attempt to find it by electrophysiological methods. By correlating the position of the medial capsular border found by a stimulation technique, with the width of the third ventricle, in a series of 130 patients, reliable ranges of its position were obtained.

Craniocervical dystonias are a major therapeutic problem, since in the majority of cases various drug regimens as well as surgical interventions either fail or are accompanied by serious side effects. Botulinum toxin, well known as a biological toxin which blocks the cholinergic neuromuscular synapse, proved to be a new and successful concept in the treatment of these disorders. In the present study, mechanisms of action, clinical indications and practical use of botulinum toxin are described.

We report an unusual case of benztropine-induced acute dystonia and dyskinesia without findings of acute anticholinergic toxicity in a 20-month-old child. Laboratory analysis of blood, urine, and gastric contents demonstrated the presence of an atropinic compound and diphenhydramine only, suggesting the association of benztropine and acute dystonia. Effects of benztropine on neuronal uptake of dopamine may represent a possible mechanism for this unusual adverse effect.

Two patients suffering from dystonia and athetosis have been treated by means of chronic electrical stimulation of the paleocerebellum and were followed for over two and a half years. One of the patients showed signs of slight improvement, while no improvement at all was observed in the other patient. Some observations and problems are discussed.