Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030567 (Parkinson's disease)
 63,064 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four cases of dystonia occurring in two families are reported. The first symptoms consisting of dystonia and rigidity appeared early in childhood, in the first months in one family and of ages two and five years respectively in the other. In two cases, transient tremor was noted. These four children have been treated with L-dopa with prompt spectacular results, in cases 1 and 2, with more gradual less complete results in the others. L-dopa treatment was continued twelve, eleven, six, and five years, respectively, without any developmental problems. Motor function remains satisfactory and school work is normal. The only secondary effect observed was the occurrence of dyskinesia. The relation between L-dopa responsive dystonia and Parkinson's disease is discussed.
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PMID:Dystonia--L-dopa responsive or juvenile parkinsonism? 658 12

The long term consequences of the use of a dopamine agonist, bromocriptine, in the treatment of Parkinson's disease are reported. In a first study in 82 patients showing late side effects of levodopa, bromocriptine permitted a significant decrease of the gastro-intestinal adverse effects. In contrast, no significant improvement of end of dose deterioration from levodopa was noted. In cases where levodopa had ceased to be active, bromocriptine produced an improvement in the clinical state. The drug was ineffective in the very advanced stages of the disease or in the cases of dyskinesias without "on-off" effects. Bromocriptine did not significantly improve freezing or "on-off" effects, but reduced other side effects of levodopa, in particular dystonia. In a second group of 29 patients who had never received levodopa treatment, bromocriptine was shown to be very effective as a first treatment of the disease. The most important finding was the absence of long term side effects similar to those usually observed under levodopa: in this group and in comparison with 38 patients taking levodopa, dyskinesia, dystonia, oscillations in performance and especially "on-off" effects were not noted. However, a partial loss of efficacy of bromocriptine was observed in 27% of cases. In a third group of 10 patients, bromocriptine introduced according to a low and slow protocol was found to be active in limited number of patients only.
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PMID:Should dopamine agonists be given early or late in the treatment of Parkinson's disease? 671 21

Forty-four previously untreated patients with early idiopathic Parkinson's disease were treated from the time of diagnosis with submaximum doses of levodopa (mean 420 mg) in combination with a peripheral dopa decarboxylase inhibitor and followed for a minimum period of 3 years. Forty-one gained initial worthwhile improvement, and 17 achieved a reduction in pretreatment disability scores of more than 50%. After 3 years of sustained therapy, only 14 had completely maintained their initial response, and 12 patients were experiencing end-of-dose deterioration; 13 patients had mild peak-dose dyskinesias, but none developed the on-off phenomenon, hypotonic freezing, or end-of-dose dystonia. Retrospective comparison with patients matched for duration of disease and pretreatment severity who had been given maximum tolerated doses of levodopa (a comparable mean dose of 950 mg) showed that the smaller dose regimen reduces peak-dose dyskinesias but not waning of response or end-of-dose deterioration.
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PMID:Sustained low-dose levodopa therapy in Parkinson's disease: a 3-year follow-up. 685 81

Dystonia musculorum deformans is a descriptive diagnosis. A number of other conditions such as Hallervorden-Spatz disease and juvenile paralysis agitans have to be excluded. Then the history of a child's illness may suggest a particular syndrome such as the progressive dystonia with marked diurnal fluctuations. Two case reports are given of children who show fluctuation of symptoms but their histories varied from those previously described. Problems of treatment are discussed and it is suggested that fluctuation of symptoms may indicate a response to levadopa while those with a relentless progression of symptoms may respond to other drugs such as orphenadrine.
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PMID:Fluctuating dystonia and allied syndromes. 713 34

The progress of 178 patients with Parkinson's disease who began treatment with levodopa between November 1969 and December 1972 is reviewed after six years. One hundred and twenty-five patients showed an initial improvement of their individual total disability scores exceeding 25 per cent, but after six years of sustained treatment only 37 patients still obtained similar benefit. By 1978 only five patients had maintained their initial improvement compared to 69 patients after two years therapy; however, 47 patients were still better than before treatment. The overall mortality ratio--the ratio of observed to expected death rate--for all the patients was 1.45:1. In those patients who unable to tolerate levodopa for longer than two years the ratio was 2.38:1; in those who were able to tolerate sustained medication, life expectancy was normal (ratio of 0.91:1 for males and 1.14:1 for females). Involuntary movements were the commonest complication of treatment. Three main types were distinguished. Peak dose dyskinesias, beginning 20 to 90 minutes after an oral dose and most severe midway through the inter-dose period, affected 80 per cent of patients. Early morning and end-of-dose dystonia occurred in 20 per cent of patients and biphasic dyskinesia--two distinct episodes of involuntary movements within each inter-dose period--was the least common pattern affecting 3 per cent of patients. Involuntary movements increased in frequency and severity as treatment continued. End-of-dose deterioration ('wearing-off' effect of individual doses) occurred in 65 per cent of patients: unpredictable oscillations in motor performance (the 'on-off' phenomenon) unrelated to the time and dosage of levodopa, occurred in 10 per cent. Psychiatric side effects included toxic confusional states, visual pseudohallucinations and paranoid psychoses and constituted the most frequent reason for stopping medication. Forty (22 per cent) of the patients had suffered severe depression before the onset of disease and levodopa had no sustained antidepressant effect in this group. After six years of treatment with levodopa, 32 per cent of the patients had unequivocal dementia.
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PMID:The impact of treatment with levodopa on Parkinson's disease. 746 63

It is controversial if early onset Parkinson's disease (EOPD) (onset at < 41 years of age) is Parkinson's disease (PD) occurring at a younger age or a different disease. This controversy is due to some clinical and pathological differences between EOPD and PD. Within EOPD, there appear to be two groups namely: young onset Parkinson's disease (YOPD), with onset between 21 and 40 years, and juvenile parkinsonism (JP), with onset at < 20 years. The two major clinical differences between these groups are a higher familial occurrence of PD and dystonia in JP. In this study, we determine if the two groups have the classical features of PD, namely rest tremors, rigidity, bradykinesia, and postural instability, and have a meaningful response to levodopa. Furthermore, we compare their other clinical features, autonomic and cognitive functions, and levels of CSF monoamine metabolites to determine differences between these groups. We observe that all YOPD (100%) and JP (85%) patients had rest tremors. Most of these patients also had a meaningful response to levodopa (YOPD: 72%; JP: 100%). The prevalence of family history of PD was similar, whereas dystonia was more frequent in JP (43%) compared to YOPD (9%). Autonomic symptoms were twice as common in JP (42%) compared to YOPD (17%). However, bedside autonomic functions were abnormal in similar proportions and, like in PD, suggest involvement of parasympathetic nervous system. Cognitive dysfunction does occur but with no difference in severity between the two groups. The difference in number of patients between YOPD and JP groups makes statistical comparison of the occurrence of clinical features like dystonia and autonomic dysfunction difficult.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Early onset Parkinson's disease: are juvenile- and young-onset different? 752 83

A 45-year-old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right-sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion-induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism-hemiatrophy.
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PMID:Hemiatrophy, juvenile-onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult-onset hemiparkinsonism: the spectrum of hemiparkinsonism-hemiatrophy syndrome. 756 31

Determinations of biopterin (BP), homovanilic acid (HVA), glutamic acid (GTA), and glutamine (GT) levels in cerebrospinal fluid (CSF) obtained through a lumbar tap were performed in 20 parkinsonian patients in different stages of evolution and without medication. In patients with motor symptoms not related to Parkinson's disease (dystonia, dyskinesia and essential tremor) (n = 4). In 7 other neurological patients subjected to spinal tap for diagnostic procedures neurotransmitters were also determined and taken as control groups. In 14 of the patients with Parkinson's disease, the symptoms were evaluated using conventional scales (UPDS, NYPDS, NWPDS, Schwab and England, and Hoehn and Yahr scale). The amplitude and the frequency of tremor were quantitatively evaluated through a single plane accelerometer Grass SP-1, akinesia was measured through reaction time to auditory stimuli, and rigidity through the speed of lineal movement. Evaluations were performed with the patient not on any medication for 1 week and repeated 1 h after the intake of 250 mg of 200/50 L-dopa/carbidopa preparation (Sinemet) and on a different day after the intake of biperiden (Akineton) 6 mg/day. Differences in neurotransmitter or metabolites levels between Parkinson's disease and control groups were determined through an independent Student's t test. Correlation between severity of symptoms in the scales and for each individual symptom measured through the quantitative tests and the levels of neurotransmitters in CSF were evaluated through the Pearson correlation analysis test. Modifications in the motor performance after administration of Sinemet and Akineton, and the levels of neurotransmitters were indirectly determined. RESULTS. (1) There were significant differences between the levels of BP and GT in patients with Parkinson's disease and control groups, (2) lower GTA levels correlated with more severe rigidity and akinesia, and with the best response to the administration of L-dopa and may be an important marker for prognosis, and (3) lower levels of GT correlated with least akinesia, but not with tremor, which may indicate that the akinesia depends on other biochemical abnormalities besides dopamine depletion.
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PMID:Neurotransmitter levels in cerebrospinal fluid in relation to severity of symptoms and response to medical therapy in Parkinson's disease. 763 Oct 94

In a preliminary study, the effects of ventroposterior medial pallidotomy were evaluated in five patients with advanced Parkinson's disease in whom medical therapy had failed. The mean age was 67.0 +/- 5.6 years, and the mean Hoehn and Yahr stage when "off" was 3.9 +/- 1.3. Three patients received unilateral pallidotomies; two of these received another pallidotomy after 8 weeks. Two other patients received staged bilateral pallidotomies. No significant differences in overall function could be seen before and after the first surgical procedure. All three patients with peak-dose dyskinesias or dystonia had marked contralateral reduction in these symptoms. Ventroposterior medial pallidotomy can ameliorate peak-dose dyskinesias in patients with advanced Parkinson's disease. Overall function improvement is not remarkable.
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PMID:Ventroposterior medial pallidotomy in patients with advanced Parkinson's disease. 872 63

In the past, stereotactic surgical intervention for Parkinson's disease was considered indicated only in those patients with active motor manifestations that were refractory to pharmacological therapy, manifestations such as tremor, rigidity, dystonia, and dyskinesia. With the reintroduction and refinement of Leksell's posteroventral pallidotomy, both akinetic and hyperkinetic symptoms are now amenable to surgical treatment. We have analyzed the results of 126 patients who underwent either unilateral (n = 58) or bilateral (n = 68) posteroventral pallidotomies. The Unified Parkinson's Disease Rating Scale and Hoehn and Yahr Staging Scale were used for preoperative and postoperative objective assessments. Postoperative follow-up evaluation occurred initially at 1 week and subsequently at intervals between 1 and 12 months (mean = 4.5 months) after surgery. Although individual motor subscores on the Unified Parkinson's Disease Rating Scale were significantly reduced (n = 126, P < or = 0.01), the most dramatic findings were the reversal of akinetic symptoms and the elimination of dyskinesia and profound "off" periods. These clinical results, combined with intraoperative microelectrode records revealing pallidal neuronal hyperactivity, suggest a reconsideration of the pathophysiology of akinesia and point to possible mechanisms of akinesia improvement by posteroventral pallidotomy in some parkinsonian subgroups.
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PMID:The results, indications, and physiology of posteroventral pallidotomy for patients with Parkinson's disease. 764 96


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