摘要:

AbstractOccupational dystonia is a frequent clinical symptom in musicians and has been described as muscle spasms and hand cramps in pianists. Robert Schumann had a neurologica impairment of his right hand that was not clinically diagnosed during his life and that impaired his career as pianist from his early 20s. This disturbance was characterized by pain and by rigidity of the fingers, which extended to other segments of his right upper extremity while he was performing and that increased with stress and improved with muscle relaxation. This disturbance produced a progressive impairment of his writing. We here hypothesize that Schumann's neurological problem was consistent with dystonia.

ISSN:0885-3185    

DOI:10.1002/mds.870100402

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractMovement disorders (MD), other than tremor, associated with multiple sclerosis (MS) occur infrequently. We report 14 new cases of whom nine had dystonia, three parkinsonism, and two had myoclonus. We also reviewed 135 such cases from the literature. From an analysis of the individual MDs and the site of the lesions described, we conclude that paroxysmal dystonias (tonic spasms), ballism/chorea, and palatal myoclonus can be caused by demyelinating lesions. Parkinsonism, dystonia, and other types of myoclonus, however, often appear to be coincidental.

ISSN:0885-3185    

DOI:10.1002/mds.870100403

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThe aim of this cross‐sectional study was to compare the role of aging in measures reflecting diurnal activity and immobility in 60 parkinsonian patients with predominant features of hypokinesia and rigidity and 100 healthy subjects aged 50 to 98 years. In the patients, we also studied the relation between disease duration and subjective and objective measures of disease severity. Motor acitivity was recorded during 5 successive days at home with a wrist‐worn activity monitor. For each subject, two mean measures reflecting the diurnal activity level and the relative proportion of activity and immobility were calculated. Diurnal measures of activity revealed in both groups a prominent absolute reduction of activity and an increase of the time spent without movement (“immobility”) with advancing age. Parkinsonian patients showed significantly lower values for both motor‐activity measures than did the healthy subjects. The rate of the age‐related decline of both diurnal activity measures in both groups, however, is comparable. Disease duration showed no relation with subjective and objective measures reflecting disease severity. This study shows that if care is taken to control for disease severity, the rate of the age‐related decline of measures reflecting diurnal activity and immobility is similar in both groups. The lack of relation between disease duration and subjective and objective measures of disease severity suggests that the rate of progression of Parkinson's disease can be reliably studied only by means of longitu

ISSN:0885-3185    

DOI:10.1002/mds.870100404

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThe pharmacokinetics and clinical effects of apomorphine after rectal administration were determined in five patients with idiopathic Parkinson's disease (PD). Three different pharmaceutical formulations were tested: a rectal solution of apomorphine (10 or 15 mg), a gelatin suppository (25 and 50 mg), and a Witepsol‐H15 suppository (50 and 100 mg). The pharmacokinetics of apomorphine were determined by measuring plasma concentrations using a sensitive and specific high‐performance liquid chromatography method. The mean bioavailability varied between 14.7% and 40.2%, which was the bioavailability until the end of clinical benefit. Also, despite the differences in dose, the values of the Cmax were similar, with average values of 12.7‐25.6 ng/ml. Wide differences in Tmax were observed, with values varying between 16 min for the enema and 127.5 min for the Witepsol‐H15 100‐mg suppository. The time course of the clinical effect was determined by assessing the time needed for walking a 25‐m course and by calculating a tremor and dyskinesia score. Onset of effect was similar for each of the preparations, with an average onset time of 14‐28 min. Significant differences with respect to the duration of the effect were observed. The duration of effect after administration of the Witepsol‐H15 100‐mg suppository was 156 ± 43 min versus 50 ± 13 min after rectal administration of the apomorphine solution. These results show that rectal administration of apomorphine may present an alternative to subcutaneous administration. The sustained‐release properties of the Witepsol‐H15 suppositories are especially of interest in the treatment of on

ISSN:0885-3185    

DOI:10.1002/mds.870100405

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractWe 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.

ISSN:0885-3185    

DOI:10.1002/mds.870100406

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractAssessment of the functional status in patients with blepharospasm is of major importance for clinical practice and outcome studies. The Blepharospasm Disability Scale (BDS) is specifically directed to measure the disability in these patients. The metric properties of this instrument were evaluated. Reliability, validity, and responsiveness to within‐patient health changes over time of the BDS were assessed in 40 patients with essential blepharospasm treated with botulinum toxin injections. The reliability of the scale was sufficient for use on group level (Cronbach's α coefficient, 0.69). Evidence of discriminant validity was provided by the difference in median score on the BDS between 21 newly admitted patients and 19 patients already under treatment (p<0.001). Convergent validity was supported by correlations between BDS and neurological impairment scores (range, Spearman correlation coefficients, 0.65‐0.79). Responsiveness to health changes was demonstrated by a significant difference between median BDS scores before treatment and 2 weeks after treatment with botulinum toxin (p<0.01). The BDS is a useful disease‐specific instrument to assess disability. Completion of the questionnaire is easy and takes only a few minutes. The instrument is suitable for use in patient care, descriptive outcome studies, and should be considered in controlled clinical

ISSN:0885-3185    

DOI:10.1002/mds.870100407

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractWe report three cases of autopsy‐proven Binswanger's disease (subcortical arteriosclerotic encephalopathy) with unusual clinical features. Two patients had supranuclear gaze disturbances, early gait dysfunction, and speech disorders suggestive of progressive supranuclear palsy. One of these patients was not demented at the time of death. The third patient had features typical of Parkinson's disease. All three patients were responsive to treatment with levodopa. The clinical spectrum of Binswanger's disease should be expanded to include levodopa‐responsive parkinson

ISSN:0885-3185    

DOI:10.1002/mds.870100408

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractA total of 26 patients with torticollis were studied using a recently developed technique for recording vestibulocollic reflexes form the sternocleidomastid muscles in addition to conventional caloric tests of vestibular function. Previous reports of abnormalities of vestibulo‐ocular reflexes in these patients were confirmed with just fewer than half having significant canal pareses or directional preponderances (nine of 20 tested). In addition, there was a high incidence of abnormal click‐evoked vestibulocollic reflexes (17 of 26 tested), which were not simply the result of prior treatment with botulinum toxin, nor due to unequal levels of muscle activation. In patients never previously treated with botulinum toxin (14 patients), the effect almost always consisted of suppressed responses in the sternocleidomastoid muscle ipsilateral to the direction of head turning. Because responses were not abnormal in all patients tested, and more commonly so in those with a history of torticollis of ≥5 years (eight of nine patients) than in de novo patients, we suggest that the changes are more likely to be compensatory than c

ISSN:0885-3185    

DOI:10.1002/mds.870100409

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractPatients suffering from Parkinson's disease (PD) are more dependent on visual feedback during movement than are normals. Studying two‐dimensional pointing movements, we recently found that PD patients undershoot targets when vision of their own moving hand is occluded but not when complete vision is provided or when the target is extinguished immediately before movement onset. In the absence of vision, information about position of the moving hand may originate from peripheral kinesthetic feedback and from corollary discharges derved from the efferent motor signal. To find out which of both mechanisms–kinesthetic feedback or corollary discharge–is defective in PD, we compared active movements with imposed movements in which the hand is passively moved by the experimenter, whereas vision of the hand was occluded under either condition. In agreement with our earlier findings, slow, active pointing movements of PD patients were hypometric. In addition, PD patients terminated passively imposed movements of comparable speed earlier than did normals, with the consequence that imposed movements were equally hypometric. Our results make it unlikely that disturbed corollary discharge is responsible for hypometria under nonvisual conditions. Instead, the data suggest that PD patients have a defect of kinesthesia in slowly executed move

ISSN:0885-3185    

DOI:10.1002/mds.870100410

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractTen patients with focal dystonia of the hand, all of whom had benefited in an open‐label study of botulinum toxin, were treated with botulinum toxin‐A in a double‐blind study. Response was assessed by three measures: (a) subjective rating, provided by patients' reports of the effect of the injections on the dystonia; (b) objective testing, consisting of manual muscle testing (MRC scale) to measure muscle strength in all patients, timing of a writing sample and counting the number of errors of writing off‐the‐line in six patients with writer's cramp, counting the number of errors on a standard test of transcription in two patients with stenographer's cramp, and rating by professional musicians of the performances of two patients with musician's cramp; and (c) physicians'rating, provided by a review of the patients' videotaped performance by neurologists who were unaware of which treatment was administered. Eight of the 10 patients had greater subjective improvement with botulinum toxin than with placebo, and this impression was verified by at least one objective test in six patients. Two patients failed to have a better response to botulinum toxin than to placebo, and their reports were verified by the objective tests. This study confirms the efficacy of botulinum toxin in many patients with focal hand

ISSN:0885-3185    

DOI:10.1002/mds.870100411

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY