ISSN:0885-3185    

DOI:10.1002/mds.870090102

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

年代:1994

数据来源: WILEY

摘要:

AbstractThe aim of this study was to provide further insight into the phenomenology and pathophysiology of monophasic and biphasic dyskinesias induced by levodopa in Parkinson's disease. For this purpose, the type, localization, severity, and timing of dyskinesias were evaluated in 15 parkinsonian patients in relation to motor disability after administration of levodopa using a video‐electromyographic recording device. Foot‐dystonia, myoclonus, and akathisia were observed in most patients. The dyskinesias started in the foot, usually on the side most affected by the disease, and spread in an “ascending wave” to the contralateral side, the trunk, and upper extremities. In a few patients, onset was axial, spreading almost instantaneously to all limbs. The dyskinesias were dystonic and ballistic at the start, and became increasingly choreic as they attained the upper limbs. Their intensity was maximal in the lower limbs, then progressively decreased, while increasing in upper limbs and head. The results indicate that there is no strict dichotomy between biphasic and monophasic dyskinesias. In other words, there is a “continuum” between the first dyskinesias and those observed during the period of maximal clinical improvement. These dyskinesias can also appear in reverse order, as if there were an “oscillator” determining a sequence of alter

ISSN:0885-3185    

DOI:10.1002/mds.870090103

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

年代:1994

数据来源: WILEY

摘要:

AbstractThe clinical presentation, symptoms, and signs in 20 new patients with the painful legs and moving toes syndrome are presented. Painful legs and moving toes may develop in the setting of spinal cord and cauda equina trauma, lumbar root lesions, injuries to bony or soft tissues of the feet, and peripheral neuropathy. In 4 of the 20 cases in the present study, no definite cause was found. Pain preceded the onset of toe movements in 18 cases, but in 2 the reverse sequence occurred. The pain had many of the characteristics of causalgia, but none of the patients exhibited the full picture of reflex sympathetic dystrophy, and peripheral trauma was the trigger in only 5 cases. Several patients reported that the occurrence of toe movements was closely related to the pain, although abolition of pain with lumbar sympathetic blocks was not necessarily associated with disappearance of the movements. Several features suggest a central origin for the movements. Symptoms may begin one side and become bilateral; movements may be momentarily suppressed by voluntary action or exacerbated by changing posture; and electromyography reveals complex patterns of rhythmic activity with normal recruitment of motor units involving several myotomes. Three other patients with similar moving toes but no pain are also described. The occurrence of similar movements in the absence of pain raises the possibility that these cases represent examples at one end of a spectrum of disorders, with pain alone (causalgia) at the other end and the syndrome of painful legs and moving toes in between. Common precipitating factors are peripheral tissue, nerve, or root injury, which may lead to alterations in afferent sensory information with subsequent reorganisation of segmental or suprasegmental efferent motor activity. The altered sensory input may result in pain, abnormal efferent motor activity, or both via segmental or suprasegmental sensorimotor circuits.

ISSN:0885-3185    

DOI:10.1002/mds.870090104

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

年代:1994

数据来源: WILEY

摘要:

AbstractGlutaric aciduria type I (GA‐I) is an inborn error in the degradation of lysine, hydroxylysine, and tryptophan due to a defiency of glutary‐CoA dehydrogenase. Glutaric, 3‐OH‐glutaric, and glutaconic acids are excreted in the urine, particularly during intercurrent illness. The enzyme may be assayed in leukocytes, cultured fibroblasts and chorionic villi. Twelve new cases, 9 months‐16 years of age, are reported, comprising all known cases of GA‐I in Sweden and Norway. Ten had a severe dystonic‐dyskinetic disorder, one had a mild hyperkinetic disorder, and one was asymptomatic. Two children died in a state of hyperthermia. Carnitine deficiency and malnutrition developed in patients with severe dystonia and dysphagia, which necessitated substitution and gastrostomy. A slowly progressive dyskinetic disorder developed in spite of adequate early dietary treatment in one subject. Macrocephaly was found in three. Computed tomography and magnetic resonance investigations in 10 showed deep bitemporal spaces in 7. Neuropsychological testing of 8 of 12 subjects demonstrated receptive language function to be superior to expressive language and motor function. Cognitive functions were obviously less affected than motor functions. A review of 57 pooled cases showed that a severe dystonic syndrome developed in 77%, a mild extrapyramidal syndrome in 10%, and 12% were asymptomatic. This disorder may pass undetected in the cerebral palsy and mentally retarded child and adult populations. Repeated urine examinations of organic acids in the urine and enzyme assay may be necessary to

ISSN:0885-3185    

DOI:10.1002/mds.870090105

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

年代:1994

数据来源: WILEY

摘要:

AbstractFiber diameter variability, acetylcholinesterase staining properties, and average fiber diameter were determined 5 weeks after varying doses of botulinum A toxin were administered into albino rabbit longissimus dorsi muscle. The average fiber diameter within the muscle appeared to be a function of the dose of botulinum toxin injected. Fiber diameter variability correlated with the dose of botulinum toxin administered. Both fiber diameter variability and acetylcholinesterase spread characteristics showed a distinct diffusion gradient over a defined field within a muscle. At lower doses (1 IU), collapse of the diffusion gradient occurred over a 15–30‐mm segment of muscle. At higher doses (5–10 IU), diffusion of botulinum A toxin effect occurred throughout the entire muscle with no apparent end point. This study demonstrated that botulinum A toxin produces a gradient of denervation in a given muscle and that both the magnitude of denervation and the extent of the gradient are dose dependent. Furthermore, both muscle fiber diameter variability and acetylcholinesterase staining were useful as measures of chemodenerv

ISSN:0885-3185    

DOI:10.1002/mds.870090106

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

年代:1994

数据来源: WILEY

摘要:

AbstractThree hundred and seventy‐six subjects with advanced Parkinson's disease participated in a prospective, double‐blind placebo‐controlled study of the dopamine agonist pergolide mesylate as an adjunct to Sinemet®. At 6 months, patients randomized to pergolide had a statistically singnificant improvement in total Parkinson's score, scores of activities of daily living, motor function, number of “off” hours, Hoehn and Yahr stage, and numerous parameters of parkinsonian function including bradykinesia, rigidity, gait, and dexterity. This benefit was obtained with the addition of a mean dose of 2.94 mg of pergolide, which permitted a 24.7% reduction in dose of levodopa. Adverse reactions were, for the most part, mild, reversible, and not of major clinical significance. No significant cardiac or electrocardiographic abnormalities were detected. This study demonstrates that pergolide mesylate, as an adjunct to levodopa, is an effective antiparkinsonian agent that provides clinical improvement while permitting a reduction in lev

ISSN:0885-3185    

DOI:10.1002/mds.870090107

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

年代:1994

数据来源: WILEY

摘要:

AbstractPatients with Parkinson's disease (PD) are more dependent on visual information during movements than normals. To investigate the mechanisms underlying deterioration of movement under nonvisual conditions, we studied two‐dimensional pointing movements to randomly occurring targets. The experimental design allowed us to systematically manipulate visual feedback during the movement by removing vision of the target, of the moving hand, or of both. Execution of pointing movements in PD deviated most severely from that of normals when PD patients moved without vision of their own moving hand. Under this condition, undershooting of the target appeared, and movements were particularly slow. In contrast, with complete vision or when only vision of the target was occluded, pointing movements of PD patients were accurate and faster. PD patients had no difficulties selecting the correct movement direction. Reaction times were longer in PD patients irrespective of the availability of visual feedback. Our findings suggest that the ability of PD patients to use nonvisual feedback during execution of arm movements is impaire

ISSN:0885-3185    

DOI:10.1002/mds.870090108

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

年代:1994

数据来源: WILEY

摘要:

AbstractThe need for objectivity in the assessment of parkinsonism prompted the development of a portable transducer capable of quantifying muscular rigidity. This paper describes the development and use of a device for measuring wrist rigidity and reports the preliminary findings from 25 normal healthy controls and 29 patients, many of whom were undergoing antiparkinsonian treatment to alleviate rigidity or antipsychotic treatment, which produced parkinsonian rigidity. An objective rigidity score, representing the degree to which motor activity increases muscular stiffness in the wrist, correlates highly with clinical ratings of parkinsonian rigidity and demonstrates 89% specificity and 82% sensitivity. Unlike previous techniques for quantifying rigidity, this transducer offers greater portability and apparent face validity.

ISSN:0885-3185    

DOI:10.1002/mds.870090109

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

年代:1994

数据来源: WILEY

摘要:

AbstractWe present two families with adult‐onset focal dystonias with marked variability in disease expression, discuss the clinical findings in relation to the literature, and comment on the difficulties in determining the pattern of inheritanc

ISSN:0885-3185    

DOI:10.1002/mds.870090110

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

年代:1994

数据来源: WILEY

摘要:

AbstractWe compared personal histories of 127 cases and 245 controls to identify possible environmental risk factors for idiopathic parkinsonism (IP). Of our controls, 121 had cardiac disease (CD) and 124 were randomly selected from electoral lists (voters). Using logistic regression and adjusting for sex and age, we ran separate analyses: IP versus CD and IP versus voters. A full occupational history was collected, as was known contact with all pesticides associated with the tree fruit sector of the agricultural industry. We found a significant association between IP and having had an occupation in which exposure through handling or directly contacting pesticides was probable, but no specific chemicals were associated with IP. We conclude that although occupations involving the use of agricultural chemicals may predispose to the development of IP, it seems likely that the pathogenesis is multifactorial rather than related to a specific agent.

ISSN:0885-3185    

DOI:10.1002/mds.870090111

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

年代:1994

数据来源: WILEY