摘要:

AbstractWe have reviewed 90 patients from the literature with histopathological features compatible with currently accepted criteria for the diagnosis of the Steele‐Richardson‐Olszewski syndrome (SROS). Only 62 patients (69%) had clinically definite SROS based on the criteria of Maher and Lees. Neurofibillary degeneration of subcortical structures with involvement of the internal pallidum, the subthalamic nucleus, and substantia nigra was considered essential for the pathological diagnosis. Thirty‐six cases (40%) had neocortical neurofibrillary change that bore no clear relationship to the degree of cognitive impairment (χ2= 9.293; p<0.4107). Grumose degeneration of the dentate nucleus was present in 25 cases (28%), and occasionally there were other, less usual, findings such as Pick bodies and Lewy

ISSN:0885-3185    

DOI:10.1002/mds.870090402

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

年代:1994

数据来源: WILEY

摘要:

AbstractAlthough dyskinesia is a frequent and important problem in Parkinson's disease (PD), a reliable assessment measure has not been thoroughly developed and tested. We modified the Obeso dyskinesia scale to creat an objective rating scale for dyskinesia assessment during activities of daily living. Thirteen physicians and 15 study coordinators involved in a clinical trial independently reviewed videotape segments of PD patients performing three tasks: walking, putting on a coat, and lifting a cup to the lips for drinking. Raters evaluated the severity of worst dyskinesia seen, the types of all dyskinesias seen, and the type of dyskinesia most associated with motoric disability. For all assessments, the total group showed statistically significant inter‐ and intrarater reliability. Physicians had a higher consistency than did coordinators, but for most measures the difference was not statistically significant. Physicians and coordinators found the scale easy to use and especially practical for rating dyskinesia severity and for identifying the most disabling dyskinesia. Dyskinesias can be assessed in clinical trials and warrant regular documentatio

ISSN:0885-3185    

DOI:10.1002/mds.870090403

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

年代:1994

数据来源: WILEY

摘要:

AbstractWe report on five patients with involuntary eyelid closure, diagnosed as blepharospasm and referred to us for treatment with botulinum A toxin. Synchronous electromyographic (EMG) recording was performed from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles. In the first two cases, EMG registration showed alternating, semirhythmic dystonic activities in both the LP and OO, clinically perceptible as “flickering” of the eyelids. While the eyelids were lowered, one of them alos showed involuntary upper eyelid tractions due to dystonic activities of LP. In the third patient, EMG patterns were characterized by a gradual decrease in the level of LP activity, followed by the contraction of OO, which facilitated the return of LP to its tonic activity, termed “postinhibition potentiation” of LP. In the fourth patient, EMG recording showed involuntary inhibition of LP in combination with blepharospasm. Involuntary closure of the eyelids in the fifth patient was caused by short or prolonged periods of involuntary LP inhibition, whereas OO activity remained normal. Our results provide further evidence that LP muscle activities are regulated by burst‐tonic motoneurons, and we suggest that these motoneurons, and/or the input signals regulating their activities, can be involved independently in a pathological process. Clinical symptoms are discussed that may be helpful to recognize those cases with LP motor dysfunction, whether or not accompanied by OO activity disorders. Because the term blepharospasm indicates an abnormal motor function of OO, we propose “blepharospasm‐plus” to designate those cases with a combined motor dysfunction of LP

ISSN:0885-3185    

DOI:10.1002/mds.870090404

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

年代:1994

数据来源: WILEY

摘要:

AbstractThe prevalence and characteristics of parkinsonism have been assessed in two separate geographic areas in Italy (Arcisate and San Giovanni Rotondo). A total of 28, 377 patients of 23 general practitioners (GPs) were the reference population. Fifty‐eight patients were traced by the GP as having typical parkinsonian features or being treated with antiparkinson drugs. Among 53 subjects, 21 of them untreated, parkinsonism was subsequently confirmed neurologically. The overall crude prevalence rate was 1.87 cases per 1,000 (Arcisate 1.81; San Giovanni Rotondo 2.01). The age‐ and sex‐adjusted prevalence rates were 1.84 in Arcisate and 2.04 in San Giovanni Rotondo. After exclusion of drug‐related parkinsonism, the rates were 1.48 and 1.90, respectively. Even with some inconsistencies within the two study areas, the prevalence tended to be higher in San Giovanni Rotondo, to prevail in women, and to increase significantly with age. Presumed etiological factors for parkinsonism were recorded in 34% of the cases (mostly drugs). In two thirds of the cases the disease ran a mild course. The present study shows that the GP proves a valuable reference for surveys of parkinsonism i

ISSN:0885-3185    

DOI:10.1002/mds.870090405

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

年代:1994

数据来源: WILEY

摘要:

AbstractDyskinesias commonly appear during L‐dihydroxyphenylalanine (L‐DOPA) therapy of advanced Parkinson's disease (PD) and can occur in both dose‐related and dose‐independent patterns. Clozapine exerts a dose‐related suppression of L‐DOPA‐induced dyskinesias by shifting the i.v. L‐DOPA dose‐response curve for production of dyskinesias without altering relief of parkinsonism. We report our outpatient experience with 13 patients on daily clozapine therapy (maximum dose 400 mg/day), followed for 3–21 months (median 10). Beneficial effects of clozapine, determined from twice‐weekly diaries, included increased “on time” and decreased “off time” and time “on with dyskinesia.” Improvements were statistically apparent by 75 mg/day and remained so through 200 mg/day. Sedation was a common problem, reflected by increased time “asleep” which was significant by 50 mg/day. Sedation was dose limiting in most patients. Orthostatic hypotension and sialorrhea were variably present. No patients had seizures, bone marrow toxicity, or detectable loss of efficacy of clozapine with chronic use. We conclude that clozapine is an effective agent for suppression of dyskinesias in PD with an effective daily dos

ISSN:0885-3185    

DOI:10.1002/mds.870090406

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

年代:1994

数据来源: WILEY

摘要:

AbstractMovement kinematics of the transport and manipulation components of a double‐step prehension task were studied in eight Parkinson patients and eight control subjects. The aims were to (a) assess the effects of a spatial perturbation upon the movement for the two groups and (b) add data to the controversy about the damage/preservation of predictive behaviour in Parkinson patients. The results showed: (a) Both groups are able to preprogram a movement. (b) In both groups, the perturbation results in an anticipation of all kinematic parameters, both of the transport and manipulation components. (c) Parkinson patients, when adopting a predictive behavior, show a delay between the beginning of the two components, and thus activate them in sequence rather than simultaneously. This delay is significantly reduced by the perturbation, indicating that Parkinson patients, when using a responsive behavior, can recouple the two motor component

ISSN:0885-3185    

DOI:10.1002/mds.870090407

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

年代:1994

数据来源: WILEY

摘要:

AbstractThis study examined the N20 and N30 waves of somatosensory evoked potentials (SEPs) from median nerve stimulation at three different stimulation rates (1, 3, and 6 Hz) in patients with idiopathic spasmodic torticollis (ST). The data were compared with those collected from a group of patients affected by Parkinson's disease (PD) and normal age‐matched subjects. N30 amplitude was significantly decreased in both groups of patients with respect to the controls. The decrease was larger in patients with ST. The N20 wave remained stable. The latencies of both waves were unchanged. When the stimulus rate was increased, the N30 amplitude decreased significantly, with a similar trend observed in both patients and controls. The isolated abnormalities of the N30 wave in both ST and PD support the hypothesis of a common physiopathogenetic mechanism that involves the basal ganglia or their connections with the supplementary motor are

ISSN:0885-3185    

DOI:10.1002/mds.870090408

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

年代:1994

数据来源: WILEY

摘要:

AbstractWe compared the efficacy and safety of pergolide and bromocriptine in 57 patients with Parkinson's disease (PD) with a declining response to levodopa therapy in a single‐blind, crossover study. Patients were placed randomly on the sequence bromocriptine‐pergolide (12 + 12 weeks) or vice versa. Regular evaluations using the New York University Parkinson's Disease Scale were performed by a clinician blinded to treatment assignment. Patients' and clinicians' impressions also were recorded. The average daily dose of pergolide was 2.3 ± 0.8 mg, and that of bromocriptine was 24.2 ± 8.4 mg. Significantly greater efficacy was demonstrated by both drugs as adjunctive therapy to levodopa compared with previous treatment of levodopa alone (pergolide, p = 0.0001; bromocriptine, p = 0.0005; Wilcoxonttest). Pergolide was more effective than bromocriptine in daily living scores (p = 0.020) and motor scores (p = 0.038). No difference in dyskinesias, dystonias, and psychosis was observed. Adverse events were more frequent in bromocriptine‐treated patients. Most patients and physicians preferred pergolide to bromocriptine. Pergolide as adjunctive therapy to levodopa was more effective than bromocriptine in this short‐t

ISSN:0885-3185    

DOI:10.1002/mds.870090409

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

年代:1994

数据来源: WILEY

摘要:

AbstractOne month of adjunct treatment with naltrexone (100 mg/day) was compared with placebo in a double‐blind, randomized, cross‐over design in two groups of patients with Parkinson's disease. The first group was composed of 10 patients with a moderate motor impairment insufficiently controlled by monotherapy with bromocriptine. The second group was composed of eight patients with L‐dopa‐induced peak‐dose dyskinesia. Naltrexone as compared with placebo did not demonstrate any significant change in motor function in either group. These negative clinical results do not support a significant role of endogenous opioid systems in the pathophysiology of motor impairment in Parkinson'

ISSN:0885-3185    

DOI:10.1002/mds.870090410

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

年代:1994

数据来源: WILEY

摘要:

AbstractWe report a patient with severe axial tardive dystonia who has had dramatic improvement for 4 years after treatment with the atypical antipsychotic drug clozapine (625 mg/day). Clozapine differs from conventional neuroleptics in that it has higher affinity for D1 and lower affinity for D2 dopamine receptors than do conventional antipsychotics, which are relatively selective D2 antagonists. We propose that repetitive stimulation of the D1 receptor by endogenous dopamine, resulting in sensitization of the D1‐mediated striatal output in the presence of D2 receptor blockade, is a fundamental mechanism mediating tardive dyskinesia, including the dystonic type. According to this hypothesis, it is primarily the D1 antagonist action of clozapine that accounts for its inability to cause tardive dyskinesia as well as its therapeutic effect in tardive dystonia. Regardless of its mechanism of action, the sustained improvement observed in this case suggests that clozapine should be tried in cases of severe refractory tardive dystoni

ISSN:0885-3185    

DOI:10.1002/mds.870090411

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

年代:1994

数据来源: WILEY