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Two patients with paroxysmal attacks of "no-no" direction head tremor and mild torticollis are described. One of them had the characteristic features of paroxysmal dystonic choreoathetosis and responded well to clonazepam.
We examined 56 members of a large Norwegian family with hereditary essential myoclonus, affecting mainly the neck and upper parts of the body, and inherited in an autosomal-dominant pattern. We observed definite myoclonus in nine individuals, probable myoclonus in one, and possible myoclonus in one. There were two other living members who had a history compatible with myoclonus but who had developed a permanent remission, so we did not observe the movements, and two who had involuntary movements only with stress. Writing usually increased the myoclonus in the neck and trunk, but did not produce myoclonus in the arm used for writing. Having a conversation with an individual who was aware of being watched would also usually increase the myoclonus. Alcohol ameliorated the myoclonus in many, but not all, affected members. Activities such as walking and concentrating during reading would usually reduce the myoclonus. Three living members with definite myoclonus also had features of mild focal dystonia, either spasmodic torticollis or blepharospasm, indicating that focal dystonia may exist as part of the clinical spectrum in hereditary essential myoclonus. In addition to examining the members of the family, we videotaped them and obtained blood samples for molecular genetic analysis.
Buspirone, an azospirone compound, is a nonsedative anxiolytic that has achieved wide usage since its introduction in 1987. Although relatively free of side-effects, there have been several instances of dyskinesia and dystonia associated with the use of buspirone. We report two patients with persistent movement disorders that developed after prolonged treatment with the drug. One patient developed a lasting problem of cervical-cranial dystonia and tremors after treatment with buspirone at a dosage of 40 mg/day for several weeks. Another, receiving 30 mg/day for 6 weeks, experienced an exacerbation of preexisting spasmodic torticollis and tardive dyskinesia as well as the onset of involuntary phonations. As shown by these and other examples, buspirone poses the risk for inducing or exacerbating several types of movement disorders.
We evaluated the prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. The population of the area was 244,935 on October 1, 1992. Because four patients with blepharospasm and three patients with writer's cramp did not visit any hospitals or clinics in 1993 and did not reply to our question letter, we could not confirm their present condition: with or without focal dystonia in 1993. Four patients with facial dystonia including blepharospasm and oromandibular dystonia, seven with spasmodic torticollis, and four with writer's cramp were observed. The prevalence of focal dystonias was 6.12 per 100,000 persons, which may be lower than that in western countries. Although the reasons for this difference are still unclear, a genetic factor may be one implication.
We report a family with autosomal dominant type hereditary juvenile dystonia-parkinsonism in which eight members in three generations exhibited parkinsonism, sleep benefit, marked efficacy of levodopa, wearing-off phenomenon, and dopa-induced choreic dyskinesia. However, one case showed mainly dystonic movement that worsened after administration of levodopa. The patients in this family showed neck dystonia, such as torticollis and retrocollis, in addition to foot dystonia and other dystonic movement, such as frequently lifting the thigh. From the family history and clinical findings, these patients are considered to have a specific form of hereditary dystonia-parkinsonism.
Remote adverse effects of local intramuscular botulinum toxin were investigated in a prospective follow-up study. Twenty-six patients with spasmodic torticollis were examined (18 women, eight men, 45 +/- 13 years). Respiratory heart rate variation (HRV) was investigated by a computerized method. Different parameters were recorded (beats per minute, coefficient of variation, root mean square successive difference (RMSSD), spectral analysis, difference and quotient between maximum and minimum RR intervals, mean circular resultant). After one intramuscular injection of 12.5 ng botulinum toxin (Porton Products Ltd., England), no significant influence on HRV was seen. After the second injection, a significant attenuation was seen of four parameters (coefficient of variation, Rmax - Rmin, Rmax divided by Rmin, mean circular resultant) that lasted up to several months. No clinically manifest remote side effects and no cardiac arrhythmia were seen for several months of botulinum toxin treatment. Our investigation proves an effect of local intramuscular botulinum toxin on autonomic cardiac innervation.
Patients with cervical dystonia or tics of the nuchal muscles can develop serious cervical spine disease. We report a series of four patients who received botulinum toxin injections to control their movement disorders prior to their required surgery. One patient with cervical tic-induced radiculomyelopathy required botulinum toxin injection postoperatively to facilitate stabilization of the cervical fusion. Two patients with torticollis-induced cervical radiculomyelopathy, and one patient with dystonia-induced C5 fracture, had botulinum toxin injected preoperatively to facilitate postoperative recovery. Botulinum toxin appears to be a useful adjunct in the treatment of cervical movement disorders prior to or following surgery for associated cervical spine disease.
Seventy-six consecutive patients with cervical dystonia. (CD) treated with botulinum toxin were assessed with the Tsui rating scale, the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), and a global scale of improvement. Tsui, TWSTRS, and TWSTRS subscale scores all showed significant improvement. Tsui and TWSTRS score reduction rates correlated significantly with each other, and each correlated with the global scale. Analysis of these scales and TWSTRS subscales indicates that an objective scale of severity such as either the Tsui rating scale or TWSTRS severity subscale used in conjunction with the TWSTRS pain subscale adequately assesses improvement of CD following treatment with botulinum toxin.
It has been suggested that tardive cervical dystonia may be clinically indistinguishable from the idiopathic form and that the diagnosis rests solely on documenting an exposure to dopamine antagonist medications. To investigate this, we performed a retrospective evaluation of patient records on 102 patients with idiopathic and 20 patients with tardive cervical dystonia seen in our Movement Disorder Clinic over the past 8 years. Several clinical and demographic variables were compared and a number of differences were observed. The presence of extracervical involvement, retrocollis, and spasmodic head movements were individually found to be predictive of tardive cervical dystonia. Torticollis, laterocollis, and trick maneuvers were predictive of idiopathic cervical dystonia. Head tremor (42.2%) and family history of dystonia (9.8%) were present only in the idiopathic group. Cervical muscle hypertrophy was significantly more common in the idiopathic group (100% versus 75%). No difference was found between the two groups in their response to treatment with botulinum toxin A. These results indicate that clinical differences between idiopathic and tardive cervical dystonia exist. These differences may help to distinguish them in the clinical setting, improve diagnostic accuracy, and support the existence of a causal relationship between exposure to dopamine antagonist medications and chronic dystonia.
We used single fiber electromyography (SFEMG) to study 42 patients who had enrolled in a double-blind, placebo-controlled trial undertaken to assess the efficacy of botulinum toxin (BTX) injection of neck muscles to treat torticollis. SFEMG in a limb muscle was performed before treatment, 2, and 12 weeks after injection of placebo or BTX. Before treatment, the mean jitter was 26.8 microsec in patients who were to receive BTX, and 25.7 microsec in the placebo group. Two weeks after injection, mean jitter in the group receiving BTX was 43.6 microsec. In the placebo group, it was 26.5 microsec (P = less than .05). Twelve weeks after injection, mean jitter in the BTX group was 35.5; for the placebo group it was 24.5. Fiber density did not change in any patient during the study. There were no remote clinical effects of BTX. Injection of BTX into muscles affected with focal dystonia is a promising and safe treatment, but there are subclinical effects on uninjected muscles.
Technical-grade dinocap, a complex-mixture fungicide, is teratogenic in the CD-1 mouse, causing cleft palate and otolith defects. In this study we compared the developmental toxicities of 2,4-dinitro-6-(1-methylheptyl)phenyl crotonate and 2,6-dinitro-4-(1-methylheptyl)phenyl crotonate, model isomers of the major active ingredients of technical dinocap, to the known teratogenicity of the technical compound. Individual isomers, both isomers combined, or technical dinocap were administered to pregnant mice on days 7-16 of gestation. Some dams were killed at term and litters were removed, dead fetuses and resorptions were counted, and live fetuses were weighed and preserved in Bodian's fixative for examination for cleft palate. Other treated dams were allowed to give birth: postnatal viability and growth, development of swimming behavior, and otolith formation were evaluated. As in previous studies, technical-grade dinocap caused cleft palate and weight deficits in fetuses at term and increased neonatal mortality and abnormal swimming behavior, torticollis, and deficient otolith formation in surviving pups. Neither of the purified isomers exhibited any developmental toxicity when administered under identical conditions. Thus, it is concluded that these isomers are not the active teratogenic component(s) in technical-grade dinocap.
A unique case of a 3-year-old child who presented with neck pain and torticollis following a streptococcal pharyngitis is described. Radiological evaluation revealed dislocation of the synchondrosis between the odontoid and the body of the axis with resultant atlantoaxial instability. This dislocation healed spontaneously over the course of 2 months without any orthotic or surgical treatment.
Six members of a family suffered from benign myopathy over four generations. The clinical, laboratory, electromyographic, histological and genetic data were consistent with benign myopathy with autosomal dominant inheritance. Congenital torticollis was a feature in one patient. Linkage studies revealed no linkage between the locus of this myopathy and the locus of any of 17 genetic markers investigated. This family was of Polish descent, which indicates a widespread occurrence of this benign hereditary myopathy. The data presented are a strong argument in favor of a specific new disease entity.
Eight cases of spastic torticollis were examined during the course of stereotactic operations with the writing pressure apparatus of Steinwachs while the ventrolateral thalamus was stimulated. When 50 stimuli per sec are given, the significant changes of motor function in writing are the following: slowing of writing speed, an increase in writing pressure, greater changes of pressure amplitude with tendences to parallel course. With 25 stimuli per sec, simular results may appear, but smaller amplitude changes and lowering of writing pressure may also occur. When 8 stimuli per sec are given, no changes of pressure patterns in writing were found. Three typical cases are described. It is concluded that the recording of fine pressure changes in writing may indicate alterations of cerebral motor regulations although specific changes for certain thalamic stimulus locations were lacking.
Two patients with fibromatosis colli (congenital torticollis) presented with lytic lesions in the clavicle at the insertion of the fibrosed clavicular head of the sternocleidomastoid muscle. Biopsy of one lesion showed intraosseous fibrosis. These lesions are probably not uncommon but radiographs are rarely performed in uncomplicated cases.
One hundred and twenty-six patients with different forms of focal dystonia (89 with cervical dystonia, 12 with hand cramps and 25 with laryngeal dystonia) were treated with localised injections of botulinum toxin. Mean doses per muscle were 200 mouse units (m.u.) for treating cervical dystonia, 40-120 m.u. for forearm muscles in writers' cramp and 3.7 m.u. for the thyroarytenoid muscle in laryngeal dystonia. Responder rates have been above 80% in all patient groups and beneficial effects could be reproduced over follow-up periods of up to 4 years. The commonest side-effects were dysphagia after treatment of spasmodic torticollis, weakness of neighbouring muscles after injections for hand cramps and breathiness and hypophonia following laryngeal injections. All these were transient and generally well tolerated. It is concluded that botulinum toxin injections are a safe and effective treatment in all three types of focal dystonia.
Electromyographic biofeedback is becoming widely used to help patients regain voluntary control of specific muscles affected by neuromuscular disorders. Electromyographic feedback training has been employed in the rehabilitation of patients affected by poliomyelitis, cerebrovascular accident, torticollis, nerve injury, temporomandibular joint syndrome, bruxism, and other disorders. While EMG biofeedback appears to be a promising treatment technique, the research literature on its effectiveness consists mainly of uncontrolled case reports and clinical trials. It is concluded that new studies with more sophisticated design and more careful control are needed to demonstrate that EMG biofeedback makes a unique contribution to the treatment of neuromuscular disorders. Research is needed to identify relevant patients characteristics predictive of success, specify appropriate muscle groups for the treatment of particular disorders, determine how feedback can be most efficiently combined with more conventional techniques in achieving a therapeutic effect, and establish meaningful criteria of success in the treatment of neuromuscular disorders.
We report two case histories of previously healthy patients who both developed persistent dyskinetic syndromes (spasmodic torticollis and cranial dystonia, respectively) following the intake of norpseudoephedrine (NPE) as an appetite suppressant. The symptoms took a chronic course even after NPE intake was discontinued. Similar drug-induced dyskinesias have been described for amphetamine and neuroleptic drugs. This side effect has, however, not yet been reported for NPE, which is pharmacologically related to amphetamine. One of the patients may also have had multiple sclerosis. Structural lesions in the basal ganglia area might predispose the development of such a movement disorder. The potential relationship between NPE intake and the development of dyskinesia is discussed. Appetite suppressants, often taken without the neurologist's knowledge, may be the cause of dyskinetic syndromes.