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Rest tremor in patients with essential tremor: prevalence, clinical correlates, and electrophysiologic characteristics.

BACKGROUND: Isolated rest tremor, which is observed in some patients with essential tremor (ET), poses a diagnostic challenge. The phenomenon has been examined in few studies and is poorly understood. OBJECTIVES: To determine the prevalence and study the clinical correlates of rest tremor in ET and to examine the electrophysiologic features in a subgroup of patients. METHODS: Sixty-four patients with ET cared for at a tertiary referral center underwent neurologic examination. Five of 12 patients with rest tremor also underwent quantitative computerized tremor analysis using accelerometry and handwritten spiral analysis. RESULTS: Twelve of 64 patients with ET had rest tremor (prevalence, 18.8%; 95% confidence interval, 9.2%-28.4%). Compared with the 52 patients with ET without rest tremor, these 12 had disease of longer duration and greater severity. Also, their ET was more widely disseminated, as evidenced by a larger proportion with head tremor. None had clinical signs of bradykinesia or rigidity. The 5 patients with rest tremor who underwent electrophysiologic study had electrophysiologic features consistent with parkinsonism (eg, slow spiral speed and increased decrement of spiral speed with radius). CONCLUSIONS: In our sample, 1 in 5 patients with ET had rest tremor. The tremor was associated with disease that was more severe, more disseminated, and of longer duration. Some of these patients had electrophysiologic features consistent with parkinsonism. The basis for the rest tremor could be basal ganglia involvement, raising the possibility that the pathologic process responsible for ET may extend to these structures.

Aged↗

Increased tremor severity in bilineal essential tremor: a report of two families.

Up to 6% of the general population have essential tremor (ET). In a number of couples both partners may have ET. The clinical profile of ET in children and the parents where both parents and the child have ET remains to be established. We report on two families where both parents and one child have ET. The severity of ET was greater in the children than in either parent. Such families could provide special opportunity to determine relation between genotype and phenotypic expression of ET.

Adult↗

Increased risk of head tremor in women with essential tremor: longitudinal data from the Rochester Epidemiology Project.

In one cross-sectional study of a community in northern Manhattan, women with essential tremor (ET) were more likely to have head tremor than were men. In that study, patients were seen at one point in time, rather than followed longitudinally. Head tremor often develops after arm tremor, and its appearance in patients with ET may therefore be a function of duration of follow-up. In a second epidemiological study utilizing the Rochester Epidemiology Project, in which ET subjects were followed from disease diagnosis to death, we determined whether there was an association between female gender and head tremor. We utilized the records-linkage system of the Rochester Epidemiology Project to identify ET cases. Records were reviewed and clinical data abstracted by a neurologist specializing in movement disorders. A second neurologist reviewed a subsample of records. There were 107 ET cases (69 women, 38 men) followed for 10.1 +/- 9.1 years from ET diagnosis to death. Head tremor was present in 37 (53.6%) women and 5 (13.2%) men (odds ratio [OR] = 7.6, 95% confidence interval [CI] = 2.7-21.9, P < 0.001). In a multivariate linear regression analysis, women remained at high risk for head tremor (OR = 6.5, 95% CI = 2.2-19.0, P = 0.001) independent of disease duration. We found in this longitudinal epidemiological study that women with ET were six times more likely to develop head tremor over the course of their illness than were men. The reason for the association between gender and head tremor, which has now been demonstrated in several studies, is not known, but it could reflect gender differences in the distribution of disease pathology within the brain.

Aged↗

"Orthostatic tremor' in familial-essential tremor.

We studied a family with essential tremor of the arms. Some members also had tremor of the trunk and legs on standing, but not on walking, sitting, or reclining. Tremor was evoked, regardless of body or limb position, by strong tonic contraction of the appropriate muscles; it was a type of static postural tremor. Beta-adrenergic blockers had no effect on the tremor of the trunk or legs, but clonazepam was beneficial.

Adult↗

[Stereotactic ventrolateral thalamotomy in the treatment of essential tremor].

BACKGROUND AND PURPOSE: Essential tremor is the most common tremorogenic movement disorder. In the majority of patients the progression of the disease is slow and the pharmacological treatment effectively alleviates tremor. In rare cases of increased essential tremor the surgical treatment (ventrolateral thalamotomy) is indicated. The goal of this study was to assess the effectiveness of unilateral thalamotomy in the treatment of essential tremor. MATERIAL AND METHODS: 10 stereotactic ventrolateral thalamotomies were performed in 9 patients for pharmacologically intractable essential tremor. Right thalamotomy was done in 5 patients and left thalamotomy in 4 patients. The study included five men and four women. Patients were assessed according to the Clinical Rating Scale for Tremor (CRST) before surgery and at 3, 12 and 24 months after thalamotomy. RESULTS: In the postoperative period there was a marked reduction of essential tremor in the contralateral arm and, to a lower extent, in the contralateral leg. The mean presurgery value for contralateral upper extremity postural tremor (scores 5/6 CRST) decreased from 3.5 to mean postsurgery value of 0.6. The tremor reduction contributed to 59% improvement in specific motor tasks of upper extremities (Part B of CRST) at 2 years follow-up. There was also improvement of functional disabilities (Part C of CRST) by 62% when compared to preoperative value 2 years postoperatively. CONCLUSIONS: Ventrolateral thalamotomy is a highly effective method in the treatment of essential tremor. The side effects related to surgery are rare and most of them are transient.

Adult↗

Classification of essential tremor.

Classification of essential tremor was attempted using tremor frequency; tremor duration; family history of tremor; responsiveness to alcohol, propranolol, and primidone; muscle contraction pattern; and long-latency reflexes. Sixty-one patients were evaluated. The majority of patients had a tremor frequency less than 7.0 Hz, a positive family history, and a positive response to alcohol. Approximately 46% of patients had a beneficial response with propranolol and 71% with primidone. Tremor frequency was inversely correlated with age and directly correlated with an antagonist pattern of muscle contraction. Enhanced long-latency reflexes were not found. Other characteristics of essential tremor were not significantly correlated. It is concluded that essential tremor can not be classified into subtypes.

Aged↗

The nature of essential tremor.

The literature concerning essential tremor is confusing. Some accounts describe tremor of a resting type and its accompaniment by other neurological abnormalities. Critical analysis of the pertinent literature leaves some question as to the validity of these observations.Clinical analysis of 34 patients with essential tremor, personally observed during a four-year period, reveals this to be a monosymptomatic condition. The tremor was usually of both a postural and action type and resting tremor was not observed. There were no other neurological abnormalities. Serious disability was infrequent and, when present, was related to upper limb tremor. Essential tremor should be readily distinguishable from other central nervous system diseases but it may be confused with Parkinson's disease or cerebellar ataxia.

Adolescent↗

Disappearance of essential tremor after small thalamic hemorrhage.

Stereotactic thalamotomy has been reported to be effective in the control of various tremors, including essential tremor. We report a 69-year-old female who experienced spontaneous thalamotomy with a small thalamic hemorrhage, resulting in disappearance of essential tremor on the contralateral side to the hematoma, which involved the nucleus ventralis intermedius (Vim) of the thalamus and a portion of the posterior limb of the internal capsule.

Aged↗

Thalamic neuronal activity correlated with essential tremor.

Animal studies suggest that an olivocerebello-bulbospinal pathway mediates harmaline induced tremor, which resembles essential tremor in humans. However, recent evidence suggests that thalamocortical pathways participate in essential tremor. Thalamic single neuron activity has been analysed during thalamotomy for essential tremor. It has been shown by spectral cross correlation analysis that thalamic activity has a significant, linear relation to forearm EMG activity during tremor. The presence of this tremor related activity at the site where a lesion abolishes essential tremor suggests that the thalamus has an important role in the mechanism of essential tremor.

Adult↗

Resetting of tremor by mechanical perturbations: a comparison of essential tremor and parkinsonian tremor.

With the use of a computer-controlled torque motor, experiments were carried out on 11 patients with essential tremor and 13 with parkinsonian tremor to determine the effect of mechanical displacements at the wrist joint on the established pattern of tremor. Analysis of the timing of tremor bursts in electromyographic recordings before and following the stimuli revealed that the phase of essential tremor could be readily reset by external perturbations. In the majority of the parkinsonian patients the same type of stimuli had very little effect on the pattern of tremor. Differences between predicted and actual times of occurrence of tremor bursts following the stimuli were used to calculate a normalized resetting index, with 0 representing no resetting and 1, complete resetting. For the patients with essential tremor the mean resetting index was 0.64 +/- 0.14 (SD); for the parkinsonian patients the generation of essential tremor. Reflex mechanisms are less important in parkinsonian tremor, which is more likely dependent on oscillations originating in the central nervous system.

Biomechanical Phenomena↗

Essential tremor: an overview.

Essential tremor (ET) is typically 4 to 12 Hz frequency, absent at rest, maximal during maintenance of a posture, attenuated during movement and often accentuated at the termination of movement. Prevalence in Americans is 300 to 415 per 100,000 population, and it is frequently disabling. There is controversy about the central or peripheral origin of ET. There is no specific diagnostic test for ET; the diagnosis is made clinically. Ethanol is the most effective suppressor of ET. Treatment is with beta-adrenergic blockers, primidone, and benzodiazepines. The first systematic description of essential tremor (ET) was 100 years ago by Dana in 1887, who regarded the disorder as a hereditary tremor that was a form of "motor neurosis." Most subsequent contributions to the English literature consisted predominantly of case reports until Critchley's exhaustive survey of the subject in 1949. The disorder has been variously termed essential, benign essential, hereditary, familial, idiopathic, juvenile, presenile or senile tremor.

Adrenergic beta-Antagonists↗

Unusual forehead tremor in a patient with essential tremor.

Voice and head (neck) tremor commonly occur in patients with essential tremor (ET), but involvement of cranial musculature is generally limited to these specific cranial structures, and action tremor of the forehead has not been reported. We describe a patient with ET who had forehead tremor. The tremor seemed to be task-specific, and neurophysiological features suggested that the forehead tremor was dystonic. The presence of forehead tremor in a patient with ET probably indicates an additional pathophysiologic process. The explanation for the specificity of involvement of cranial musculature in ET is not known, but this clinical observation might help guide investigators who are interested in the underlying pathophysiology of this condition.

Electromyography↗

Alcohol consumption of patients with essential tremor.

Most patients with essential tremor experience a transient improvement after ingesting a small amount of alcohol. It has been accordingly suggested that essential tremor patients may have an increased risk of developing alcoholism. In this study, the frequency and amount of alcohol intake of essential tremor patients were found to be largely similar to the drinking habits of a control sample from the general population. This indicates that essential tremor does not generally augment alcohol consumption, nor is it a common cause of alcoholism.

Alcohol Drinking↗