ISSN:0885-3185    

DOI:10.1002/mds.870100102

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

年代:1995

数据来源: WILEY

摘要:

AbstractRestless legs syndrome (RLS) is a well‐defined clinical entity characterized by an unpleasant creeping sensation arising in the legs with an irresistible need to move them. The trouble is more pronounced when the affected people lie in a prolonged rest position and try to fall asleep. It is known that RLS may be consequent to systemic disorders and to diseases affecting the central or peripheral nervous system. The International Classification of Sleep Disorders states that peripheral neuropathy should be ruled out by medical history and clinical grounds before diagnosing primary RLS (pRLS). The present study extended peripheral nerve investigation in eight consecutive pRLS patients with normal neurological examination results and showed that all patients exhibited two or more electrical, psychophysiological, and/or morphological features of peripheral axonal neuropathy. Morphometric analysis of sural nerve showed a significant reduction in myelinated fiber density andgratio (axon diameter/fiber diameter) in the pRLS group compared with eight control biopsy specimens. These results suggest that axonal neuropathy is often present in patients with RLS. A comprehensive peripheral nerve investigation should be considered in RLS patient

ISSN:0885-3185    

DOI:10.1002/mds.870100103

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe measured the levels of dopamine, tyrosine hydroxylase (TH) protein, and dihydroxyphenylalanine (DOPA) decarboxylase (DDC) protein in the striatum of 10 patients with idiopathic Parkinson's disease (PD) and 23 patients with dominantly inherited olivopontocerebellar atrophy (OPCA). The levels of dopamine were markedly reduced (2% of control) in the striatum of the patients with PD, whereas striatal dopamine in the patients with OPCA ranged from normal (<60% of control) to moderately reduced (20–60% of control) to severely depleted (<20% of control). Both TH and DDC protein levels were significantly lower than those of the controls in the striatum of all of the patients with PD and in the subgroup of patients with OPCA having severely depleted dopamine. In contradistinction, TH but not DDC protein levels were reduced in those patients with OPCA having moderately reduced dopamine levels. This suggests that in the early stage of nigrostriatal dopamine neurone degeneration, DDC levels may be less susceptible to neurodegenerative influences than is TH synthesis or, alternatively, DDC synthesis may be more aggressively upregulated. Unexpectedly, from the blot immunolabeling analysis an additional DDC‐immunoreactive band of slightly lower apparent molecular mass was detected in two of the patients with PD and in 12 of the patients with OPCA. This additional DDC band, which was not present in any of the control subjects, may reflect posttranslational modification(s) of DDC related to the neurodegenerative proc

ISSN:0885-3185    

DOI:10.1002/mds.870100104

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe cerebral activity preceding voluntary, self‐paced ankle dorsiflexion movements while seated was compared with that before a stepping movement of the same foot while standing in four patients with isolated gait ignition failure. Results within the patient group were variable, but all exhibited some increase in the Bereitschaftspotential (BP) amplitude when stepping as compared with sitting, similar to that seen in normal subjects. This differs from previous results in patients with Parkinson's disease in which stepping was not accompanied by a normal increase in BP amplitude. This difference suggests that the mechanisms responsible for the start hesitation that is common to both Parkinson's disease and gait ignition failure may differ in the two condition

ISSN:0885-3185    

DOI:10.1002/mds.870100105

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe present three cases of the adult opsoclonus‐myoclonus syndrome in patients with systemic carcinoma. In addition to opsoclonus and myoclonus, other clinical components of the syndrome can include ataxia, tremor, gait and stance dysfunction, altered mental status, and head and face dyskinesias. The most common etiologies are idiopathic, paraneoplastic, and infectious encephalitis. Radiographic and pathological studies suggest brainstem stem dysfunctiion with associated cerebellar and/or cerebellar pathway dysfunction. In many cases, there is evidence for the involvement of immunologic and/or inflammatory processes in the pathogenesis of this syndrome. The timely recognition of this syndrome is important because of its implications for the underlying etiology and prognosis. The appearance of this syndrome should prompt the search for an occult malignanc

ISSN:0885-3185    

DOI:10.1002/mds.870100106

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

年代:1995

数据来源: WILEY

摘要:

AbstractApomorphine is a dopamine receptor agonist increasingly used in the treatment of Parkinson's disease (PD). In the present study, we examined the plasma and ventricular cerebrospinal fluid (CSF) pharmacokinetics of apomorphine as well as its effects on dopamine metabolism in six patients (one woman and five men, mean age 79.5 years) without evidence of PD who underwent 48‐h intracranial pressure monitoring for suspected normal pressure hydrocephalus. Maximal plasma apomorphine concentration (25.04 ng/ml) is found 20 min after subcutaneous injection (50μg/kg), and the mean area under the curve is 1,439.37 ng/ml for 120 min. In contrast to plasma values, the maximal ventricular CSF apomorphine concentration (1.08 ng/ml) is found 30 min after injection and the mean area under that curve is 7% of that of plasma (96.69 ng/ml for 120 min). Apomorphine administration causes a significant reduction in ventricular CSF concentrations of dopamine and of its major metabolites sulfoconjugated dopamine, 3,4‐dihydroxyphenylacetic acid (DOPAC), and homovanillic acid (HVA). This effect starts 10 min after the injection of apomorphine, is maximal after 30 min (free dopamine, ‐ 30%; sulfoconjugated dopamine, −28%; HVA, −21%; DOPAC, −31%) and is still present, although to a lesser extent ( −5 to −10%), 120 min after the injection of apomorphine. This study shows that in humans a dose of apomorphine commonly used in PD causes significant inhibition of dopamine metabolism lasting>120 min. In addition to their symptomatic effects, dopamine agonists such as apomorphine may play a role in preventing or slowing the neurodegeneration in PD by autoreceptor‐mediated inhibition of d

ISSN:0885-3185    

DOI:10.1002/mds.870100107

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe assessed the clinical utility of apomorphine infusional therapy in patients with parkinsonism and motor fluctuations and sought evidence for alterations in drug response resulting from chronic treatment. Six patients with Parkinson's disease were treated for 3 months with s. c. infusions of apomorphine administered during waking hours. At the beginning and the end of the study, test doses of apomorphine (12.5–100 μg/kg) were administered to establish a dose‐response curve. Over the study, the patients reported a significant improvement in the number of “on” hours experienced per day and substantially reduced the dose and frequency of levodopa and other antiparkinsonian medications. No average change in apomorphine dose‐response relationships or pharmacokinetics was observed during the study. However, two patients lowered the infusion rate during the 3‐month observation and exhibited higher drug levels and longer responses following test doses of apomorphine given at the end of the study. Although pragmatic concerns with the use of infusion pumps solutions and adverse effects limited the overall benefit afforded by the treatment, this kind of drug treatment may be useful in selected patients with severe parkinsonism and

ISSN:0885-3185    

DOI:10.1002/mds.870100108

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

年代:1995

数据来源: WILEY

摘要:

AbstractVibratory inhibition, the homonymous recovery curve and the ratio of the maximal H‐reflex to direct muscle potential (H/M ratio) of the soleus H‐reflex were assessed in 10 patients with leg dystonia and in six patients with arm or neck dystonia. The results were compared with those obtained in 48 healthy control subjects. H‐reflex variables most helpful for the discrimination of patients and healthy subjects were identified. In patients with leg dystonia, vibratory inhibition was less marked than in control subjects, whereas late facilitation of the recovery curve was increased. In patients with leg dystonia, area values of test reflexes in the late facilitatory phase of the recovery curve exceeded peak‐peak values, in contrast to findings in control subjects. This finding may be attributable to less synchronization of enhanced test reflexes in dystonia than in the control condition. In differentiating patients with leg dystonia from control subjects, a combination of parameters of vibratory inhibition and the late facilitatory phase of the recovery curve appeared most useful. In patients with arm or neck dystonia and in the unaffected legs of hemidystonic patients, soleus H‐reflex test results were in the normal range. Abnormalities in the results of the soleus H‐reflex tests we used appear to be related to the presence of clinical signs in the extremity under examination and not to the severity

ISSN:0885-3185    

DOI:10.1002/mds.870100109

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe performances of 12 patients with Parkinson's disease (PD), 16 with Huntington's disease (HD), and young and old healthy controls were assessed on a number of tests of verbal and nonverbal declarative memory, on a test of nonmotor conditional associative learning (words and colors), and on a number of reaction time (RT) tasks. The RT tasks consisted of cued simple and choice reactions. The relationship between the precue and the imperative stimulus in the S1–S2 paradigm was nonarbitrary in the first series and arbitrary in the second series. The series with arbitrary S1–S2 associations was repeated across two successive blocks of trials. The rationale of the study was to investigate the function of the basal ganglia “complex loop,” and it was postulated that HD patients would show greater deficits because of greater involvement of the caudate nucleus. The patients with HD had the slowest RTs. Across the two blocks with arbitrary S1–S2 associations, the patients with HD but not PD nevertheless showed evidence of learning in their precued RTs. In contrast, the patients with PD were better able to remember the associations in free recall than were the HD patients. It is concluded that patients with PD have relatively greater deficits in procedural learning, whereas those with HD have relatively more impairments in declarative memory, and the greater level of cognitive impairment in HD overall is interpreted as being due to more serious damage to the cau

ISSN:0885-3185    

DOI:10.1002/mds.870100110

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

年代:1995

数据来源: WILEY

摘要:

AbstractCisapride (CIS) is a prokinetic agent that increases gastrointestinal motility in normal individuals and improves constipation in Parkinson's disease (PD). We studied the effects of CIS on the clinical response and the peripheral pharmacokinetics of orally administeredL‐dopa given to patients with PD. Twenty patients with idiopathic PD and chronic constipation, whose response toL‐dopa was suboptimal or characterized by fluctuations, agreed to participate in an open study that lasted for 2 weeks. Fourteen patients completed the study (mean age 65 ± 9.3 years, mean duration of treatment 5.7 ± 4.2 years, meanL‐dopa daily doses 658.9 ± 269.9 mg); six patients were excluded due to lack of compliance or changes in medication during the study. The end points of the study included the mean levels ofL‐dopa, the height of the peak ofL‐dopa in plasma, mean plasma levels of 3‐OM‐dopa, and the speed and quality of gait and visuomanual corrdination before and during treatment with CIS. CIS increased peak plasma levels ofL‐dopa by 37% and the mean plasma levels ofL‐dopa by 13% with respect to those obtained with the same dose ofL‐dopa before the addition of CIS. Therefor, CIS appears to increase early absorption ofL‐dopa through acceleration of gastric emptying. CIS also increased plasma 3‐OM‐dopa levels, improved visuomanual coordination, and reduced gait disability. CIS improves gastrointestinal function and response toL‐dopa in patients with PD and could be a helpful add

ISSN:0885-3185    

DOI:10.1002/mds.870100111

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

年代:1995

数据来源: WILEY