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1.
An olfactory deficit is present in patients with essential tremor (ET), but it is often milder than that in patients with Parkinson's disease (PD). In both, the deficit occurs early in the disease. Isolated rest tremor without other signs of parkinsonism can occur in patients with ET. If the rest tremor in these patients represents a manifestation of ET rather than early PD, we hypothesized that their University of Pennsylvania Smell Identification Test (UPSIT) scores would be similar to those of ET patients without rest tremor. The mean UPSIT score in 13 ET patients with isolated rest tremor did not differ from that of 58 ET patients without rest tremor (29.3 +/- 4.3 vs. 29.4 +/- 6.4; P = 0.69). Several ET patients with rest tremor had UPSIT scores that fell outside of the range that is seen in 95% of patients with PD. These data raise the possibility that some ET patients with isolated rest tremor may not have early PD and that the pathological process that is responsible for their ET is also involving the basal ganglia.  相似文献   

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Cardiac sympathetic dysfunction was investigated using 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy in 20 patients with drug induced parkinsonism (DIP). The mean heart to mediastinum ratio was significantly greater in patients with DIP than in those with Parkinson's disease (mean (SD): 2.07 (0.39) v 1.28 (0.15), p<0.001). MIBG uptake was not different between the DIP patients and controls. Two DIP patients whose MIBG uptake was significantly reduced showed persistent parkinsonism and responded dramatically to levodopa.  相似文献   

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We investigated an association between olfaction and cardiac (123)I-metaiodobenzylguanidine (MIBG) uptake in patients with Parkinson's disease (PD) and multiple system atrophy (MSA). There was a significant positive correlation between cardiac MIBG uptake and the Cross-Cultural Smell Identification (CCSI) score in patients with PD (r = 0.56; P = 0.003) independent of the disease duration or clinical rating of motor status. However, patients with MSA did not show a significant correlation between cardiac MIBG uptake and the CCSI score. Our findings suggest that the functional losses of the olfactory and cardiac sympathetic systems are closely coupled in PD.  相似文献   

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Clinical literature suggests an association between essential tremor (ET) and Parkinson's disease (PD), and recent pathological studies describe Lewy bodies in some ET patients. If some ET were an expression of Lewy body disease, one could hypothesize that ET patients who develop parkinsonism would more likely develop PD (a Lewy body disease) than non-Lewy body forms of parkinsonism. The objective was to compare the proportions of patients with PD vs. Parkinson-plus syndromes who had diagnoses of ET. Retrospective chart review at the Neurological Institute (NI) of New York. A larger proportion of the 210 PD than 210 Parkinson-plus syndrome patients had kinetic tremor on examination (119 [56.7%] vs. 70 [33.3%], P < 0.001). Patients with PD were more likely to have a prior diagnosis of ET than were patients with Parkinson-plus syndromes (7.1% vs. 2.4%, OR 3.16, 95% CI 1.13-8.85, P = 0.02) and more likely to have a diagnosis of ET assigned by an NI neurologist (5.3% vs. 0.0%, OR 12.85, 95% CI 1.66-99.8, P = 0.001). Patients with PD were three to thirteen times more likely to have diagnoses of ET than were patients with Parkinson-plus syndromes. These data further confirm the link between ET and PD, and possibly, between ET and Lewy body disease.  相似文献   

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The purpose of our study was to prospectively evaluate cardiac [(123)I]metaiodobenzylguanidine (MIBG) uptake in patients with cerebrovascular disease (CVD) who develop clinical symptoms of vascular Parkinsonism (VP). A total of 19 consecutive patients who developed Parkinsonism during the course of their CVD were enrolled in the study; 16 age-matched subjects, and 30 patients with Parkinson's disease (PD) were also evaluated with cardiac MIBG uptake. MIBG uptake was assessed using the ratio of the heart to the upper mediastinum (H/M) according to planar scintigraphic data. The mean H/M ratio was significantly higher in patients with VP than in those with PD (2.28 +/- 0.41 vs. 1.27 +/- 0.13; P < 0.001). MIBG uptake did not differ between VP and controls (2.46 +/- 0.33; P > 0.05). Our findings suggest that myocardial postganglionic sympathetic dysfunction found in PD is absent in most patients with VP. MIBG single photon emission computed tomography imaging may be useful to help distinguish between PD and VP patients in clinical practice.  相似文献   

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We studied the effects of transcranial motor cortex stimulation on the electromyographic characteristics of tremor in 9 patients with familial essential tremor and in 12 patients with postural tremor associated with Parkinson's disease. Transcranial magnetic stimulation reset both types of tremor equally. The resetting depended on the stimulus intensity, but was most closely correlated with the duration of the electromyographic silent period that followed the stimulus-induced motor evoked potential. Tremor resetting was present bilaterally even after focal, unilateral stimulation. Transcranial electrical stimulation failed to reset the tremor in either patient group. These results emphasize the role of central, intracortical structures in the generation of essential tremor and postural tremor in Parkinson's disease. © 1994 John Wiley & Sons, Inc.  相似文献   

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We conducted a historical cohort study of 981 first-degree relatives of 162 patients with Parkinson's disease (PD) and of 838 first-degree relatives of 147 controls representative of the population of Olmsted County, Minnesota. In addition, we studied 2,684 first-degree relatives of 411 patients with PD referred to the Mayo Clinic. Relatives were interviewed and screened for tremor either directly or through a proxy, and those who screened positive were examined or copies of their medical records were obtained to confirm the diagnosis of essential tremor (ET). We also obtained ET information from a medical records-linkage system (family study method). In the population-based sample, the risk of ET was significantly increased for relatives of patients with onset of PD相似文献   

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Several studies have reported an increased risk to develop Parkinson's disease (PD) in essential tremor (ET) populations. Hyperechogenicity of the substantia nigra (SN) is a common transcranial sonography (TCS) finding in PD and has been suggested as a PD risk-marker in nonparkinsonian subjects. This study compared SN areas of 44 ET patients with 100 controls and 100 PD patients. Sixteen percent of ET patients had SN hyperechogenicity as compared with 3% of controls and 75% of PD patients. These findings might correspond to an increased risk of ET patients to develop PD. Long-term follow-up will show if those with hyperechogenic SN develop PD, while these without will not.  相似文献   

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Electrical stimulation of human upper limb muscle tendons produces a reflex inhibition (I(1)) in the same muscles. This inhibition is reduced in Parkinson's disease (PD), prompting a similar study of essential tremor (ET). In essential tremor, two of eight subjects had no discernible inhibition, even following supramaximal stimulation (< 80 mA) of the tendons from extensor digitorum communis and extensor pollicis brevis. In the remaining six subjects, the mean thresholds for I(1) in these muscles were increased by 270 and 320%, respectively, relative to controls. The maximal amplitude of the inhibition was significantly reduced in the ET group, as was the following excitation (E(1)). The latency and duration of I(1) were not different in ET subjects and controls. The maximal duration of I(1) was correlated with tremor frequency in individuals, and tendon stimulation was effective in initiating ongoing tremor cycles. These results disclose a peripheral reflex abnormality in ET that is mediated by tendon afferents and can be linked to the coexistent tremor. The response in ET was distinguished from that in PD by its different time-course and by failure of the response to appear in the antagonist muscle. The abnormality may prove a useful marker for ET, which currently lacks a definitive pathological or neurophysiological marker to support objective clinical diagnosis.  相似文献   

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ObjectiveTo investigate heart rate variability (HRV) in patients with Essential Tremor (ET) in comparison with patients with Parkinson's Disease (PD).MethodsThis is a cross sectional control study including 10 patients with ET, 10 patients with PD and 10 age-sex-matched controls. In patients and controls, we measured the components of HRV analysis in the frequency domain during a daytime period of 12-h. Selected HRV variables were low-frequency (LF) and high-frequency (HF), conventionally considered to be influenced by the sympathetic system and the parasympathetic system respectively.ResultsHRV variables, in patients with ET, were significantly different from those detected in PD patients and similar to those of controls while in PD patients, they were significantly different from those of controls. At cut off level of 654 ms2, LF component correctly distinguished ET patients versus PD with sensitivity, specificity, PPV and accuracy of 100%. By contrast, at cut off level of 737 ms2, HF component showed sensitivity, specificity, PPV and accuracy of 80%, 100%, 100%, and 86.67% respectively. DAT-SPECT and cardiac MIBG uptake were both normal in ET patients whereas they were markedly decreased in those with PD.ConclusionsIn our study, the LF component of HRV analysis distinguishes ET patients from those with PD on an individual basis, thus representing a valid help in everyday clinical practice for differentiation between these patients in absence of scintigraphic investigations.  相似文献   

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BACKGROUND: Jaw tremor in the context of dystonia is not well recognized. METHOD: We describe 7 women (average onset age 70) with jaw tremor and dystonia. RESULTS: All had features of primary dystonia. Applying MDS criteria for tremor, 3 had "dystonic jaw tremor"; 4 had "jaw tremor associated with dystonia". Drugs were unhelpful. Botulinum toxin injections proved effective in 1 case. CONCLUSION: We propose the entity of "jaw tremor and dystonia". It is distinct from jaw tremor due to other causes including Parkinson's disease and essential tremor.  相似文献   

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We studied the effect of high-frequency electrical stimulation of the ventral-intermediate nucleus of the thalamus (Vim) in four patients implanted with chronic stimulators to determine whether this procedure adversely affects reaction time to a proprioceptive stimulus. Two patients had undergone this surgery for treatment of tremor resulting from Parkinson's disease insufficiently responsive to levodopa therapy and two patients for treatment of essential tremor. Reaction times to auditory, visual, cutaneous, and proprioceptive stimuli were tested in a simple motor task requiring flexion of the elbow joint to a visual target in response to each stimulus. Reaction times were tested postoperatively with and without the stimulator turned on. We found that reaction time for all stimulus modalities was not increased when the stimulator was turned on; in fact, reaction times were, on average, slightly shorter during stimulation, but this difference was not statistically significant. We conclude that transmission of somatosensory inputs, necessary for initiating voluntary movement, from the periphery to the cortex is not significantly impaired by stimulation of the ventral-intermediate nucleus of the thalamus in patients with pathological tremor.  相似文献   

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