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Olfaction in essential tremor patients with and without isolated rest tremor.

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.

Aged↗

Physiological tremor analysis of patients with anti-myelin-associated glycoprotein associated neuropathy and tremor.

Tremor is often associated with anti-myelin-associated glycoprotein (anti-MAG) peripheral neuropathy (PN), but its physiology has never been accurately described. This study quantified the physiological characteristics of tremors in patients with anti-MAG demyelinating PN. Eighteen patients with tremor and PN with demyelinating features (ages 30-86, mean = 66.5 years) were evaluated for anti-MAG antibodies (positive considered > or = 1:3200) and for tremor amplitude, frequency, side-to-side relationships, and electromyographic (EMG) activation patterns. Thirteen patients had anti-MAG titers >1:3200 and 8 had both positive anti-MAG titers and tremors. Anti-MAG PN patients revealed tremors higher in amplitude, lower in frequency, with greater side-to-side amplitude ratios, greater amplitude variability, and more consistent cocontracting antagonist EMG patterns that do not attenuate with inertial loading. We conclude that anti-MAG PN tremor is not due only to exaggerated physiologic mechanisms but may reflect a distinctive form of neurogenic tremor.

Adult↗

Effect of stimulation frequency on tremor suppression in essential tremor.

We sought to determine the effect of deep brain stimulation (DBS) frequency on tremor suppression in essential tremor (ET) patients with deep brain stimulators implanted in the ventral intermediate nucleus (VIM) of the thalamus. A uniaxial accelerometer was used to measure tremor in the right upper extremity of subjects with a diagnosis of ET who had DBS electrodes implanted in the left VIM. The root-mean-square acceleration was used as the index of tremor magnitude and normalized to the OFF DBS condition. There was a highly significant inverse sigmoidal relationship between stimulation frequency and normalized tremor acceleration (X(2)/DoF = 0.42, r(2) = 0.997). Tremor acceleration had a nearly linear response to stimulation frequencies between 45 and 100 Hz with little additional benefit above 100 Hz. These findings have two important implications. Clinically, frequency of thalamic stimulation is an important variable for optimal tremor control with maximal benefit achieved with 100 to 130 Hz in most patients. Second, thalamic DBS provides tremor benefit in a graded manner and is not an all-or-nothing phenomenon.

Adult↗

Intention tremor of the head in patients with essential tremor.

Patients with essential tremor (ET) have kinetic arm tremor; this tremor can also have an intentional component. We are unaware of reports of intention tremor of the head in ET. Our aims were to describe, provide electrophysiological data and video documentation of, and estimate the prevalence of intention tremor of the head in our sample. Ten (9.0%; 95% confidence interval = 4.7%-14.3%) of 111 patients had intention tremor of the head; in 7 it involved the neck and in 3 the chin. These patients trended toward having more severe kinetic arm tremor and they had more severe intention tremor of the arms. These observations provide further support for the evolving view that the cerebellum may be involved in ET.

Aged↗

A single family with writer's cramp, essential tremor, and primary writing tremor.

We studied seven members of a family who had different types of movement disorders: Two had typical essential tremor, two (twins) had typical writer's cramp, one had both essential tremor and writer's cramp, one had primary writing tremor, and one (deceased) had uncharacterized tremor. This appears to be the first report of writer's cramp in twins and in so many family members. This family links together primary writing tremor, essential tremor, and writer's cramp and may help resolve the apparent controversy about the relationship of primary writing tremor to essential tremor and writer's cramp.

Adult↗

Resetting of essential tremor and postural tremor in Parkinson's disease with transcranial magnetic stimulation.

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.

Adult↗

Device for quantitating tremor activity in mice: antitremor activity of atropine versus soman- and oxotremorine-induced tremors.

A tremor monitor was constructed and tested that quantitates soman- and oxotremorine-induced tremors. The device consisted of a force transducer, from which was suspended a plastic beaker containing a mouse. The signal from the force transducer was fed into a tremor monitor, which was essentially a low-pass filter, and quantitated using the Applecounter from Columbus Instruments (Columbus, Ohio) with the data stored on floppy diskette in a personal computer or on a magnetic tape cassette. The tremor monitor demonstrated a dose response relationship to both soman- and oxotremorine-induced tremors and the antagonism of these tremors by various doses of atropine. In addition, the duration of action of a dose of atropine (17.4 mg/kg, ip) normally used in the therapy of organophosphate poisoning was assessed using the atropine antagonism of oxotremorine-induced tremors as an indicator. This device permits the unbiased determination of the tremor activity of a drug and allows the use of statistical analysis on the data produced.

Animals↗

Thalamic stimulation for parkinsonian tremor: correlation between regional cerebral blood flow and physiological tremor characteristics.

We used (15)O-labeled water (H(2)(15)O) positron emission tomography (PET) to study eight Parkinson's disease (PD) patients with unilateral ventral intermediate (Vim) thalamic nucleus deep brain stimulation (DBS) for severe tremor. Triaxial accelerometry (TRIAX) was used during imaging to obtain on-line measures of tremor characteristics. Regional cerebral blood flow (rCBF) scans together with TRIAX recordings were collected in three stimulation conditions (OFF, MID, and ON, corresponding, respectively, to 0%, 50%, and 100% reductions in mean accelerometry signal). Statistical Parametric Mapping (SPM99) revealed significant rCBF reductions during stimulation in the ipsilateral sensorimotor cortex (SMC) and the contralateral cerebellum, as well as concurrent increases in the ipsilateral ventral thalamus (P < 0.05, corrected). Covariate analysis of rCBF with physiological tremor characteristics revealed that tremor acceleration correlated positively with changes in the SMC and supplementary motor cortex ipsilaterally (P < 0.05, uncorrected), and negatively with changes in the ipsilateral cuneus (P < 0.05, corrected). After removing tremor acceleration effects, changes in tremor frequency correlated negatively with changes in the contralateral dentate nucleus and pons (P < 0.05, uncorrected). Our results suggest that Vim DBS for PD tremor modulates the activity of cerebello-thalamo-cortical pathways. Specific tremor characteristics relate to activity in different nodes of this system.

Aged↗

Writing tremor: its relationship to benign essential tremor.

Nine patients with tremor on writing and one patient with tremor only on swinging a golf club were investigated. None of the patients had any other neurological symptoms or signs. The frequency of the tremor ranged from 5 to 6 Hz. Rapid passive supination or pronation of the forearm by a torque motor evoked a short burst of alternating tremor in seven patients. The tremor was improved by alcohol or propranolol in six patients. These characteristics of writing tremor (and of other isolated action tremors) suggest that it is a variant of benign essential tremor.

Adult↗

Deep brain stimulation of the ventral intermediate nucleus of the thalamus for control of tremors in Parkinson's disease and essential tremor.

The beneficial effects of ventral intermediate nucleus (VIM) stimulation were evaluated in 20 patients with tremor refractory to medical therapy. Thalamic stimulation is a non-ablative procedure which has the advantage of a reversible, non-destructive lesion. Eleven patients [7 with Parkinson's disease (PD) and 4 with essential tremor (ET)] received unilateral VIM implantation, while 9 patients had staged bilateral VIM implantation (4 with PD, 5 with ET). PD patients showed a significant improvement in contralateral arm and leg rest tremor and ipsilateral leg rest tremor (p < 0.02) at a mean follow-up period of 16.2 +/- 7.0 months. Patients with PD did not demonstrate any significant decrease in medication use at follow-up. ET patients demonstrated significant improvement in postural and action tremor in the contralateral arm (p < 0.001), but no significant improvement in the contralateral leg tremor at follow-up. Significant improvements were also seen in ET patients in the Clinical Rating Scale for Tremor (p < 0.001) with respect to several activities of daily living at a mean follow-up of 14.9 +/- 8. 1 months. Deep brain stimulation is a safe and effective treatment for severe tremor refractory to medications. It is a highly effective, reversible, adaptable, and predictable procedure which avoids the complication of cognitive deficit seen in patients with bilateral thalamotomies.

Aged↗

Thalamic deep brain stimulation for the treatment of tremor due to multiple sclerosis: a prospective study of tremor and quality of life.

OBJECT: In several studies a significant reduction in tremor after thalamic deep brain stimulation (DBS) has been reported among patients with multiple sclerosis (MS). It has not been determined if this results in an improved quality of life. In this study the authors prospectively evaluated the effects of thalamic DBS on tremor and quality of life. METHODS: Videotapes of the patients' tremor were made preoperatively and 2 and 12 months postoperatively, and tremor was scored by a neurologist blinded to the treatment. Patients were tested pre- and postoperatively to measure any changes in their reported ability to perform selected activities of daily living and in their health-related quality of life. Patients were asked to complete a questionnaire about their satisfaction with the surgery. Postoperative changes were examined using paired t-tests. There were significant reductions in postural, action, and overall tremor at 2 and 12 months postoperatively. The patients' reported ability to feed themselves was significantly improved 2 months after surgery (p = 0.01). There were short-term trends toward improvement in reported dressing ability, personal hygiene, and writing. There were no significant changes in the SF-36 subscales or total score. CONCLUSIONS: In this cohort of patients with MS who suffered from tremor, thalamic DBS significantly improved their tremor and ability to feed themselves. Patient satisfaction with the procedure, however, was variable. Preoperative patient education about what functions might (and might not) be improved is crucial to avoid unrealistic expectations. Our results indicate that younger patients with MS tremor who had a shorter disease duration and no superimposed ataxia benefited most from this surgery.

Activities of Daily Living↗

[Rubral tremor of Holmes, rare case of pathological tremor: case report].

The authors present a very rare case of Holmes tremor (previously known as rubral or midbrain tremor). In all described till now cases the tremor was due to a known and revealed in laboratory or neuroimaging cause. We present an unusual case of a 42-year old woman with unilateral tremor of right extremities (mostly proximal part of upper extremity) which started abruptly 3 years ago. She had no suffer any serious disease before the onset of symptoms and her family history was also negative. The tremor was present at rest but accelerated during specific postures and active movements. The laboratory tests including: copper and ceruloplasmin concentrations, blood analysis for acanthocytes, evoked potentials, EEG, CT, MRI, MRA and SPECT did not reveal any significant changes. Treatment attempts with neuroleptics, clonazepam, L-dopa, valproic acid, biperiden were almost completely ineffective except local injections of botulinum toxin (Botox, Allergan, 150 U) into the muscles of right arm girdle which moderately alleviated tremor. We did not find any underlying pathology as a cause of tremor, clinically the same as symptomatic cases described in literature. We suggest the possibility of idiopathic origin of tremor in our case, although a very small size of lesion (f.i. ischaemic) could be undetectable in the described tests.

Adult↗

Tremor classification and tremor time series analysis.

The separation between physiologic tremor (PT) in normal subjects and the pathological tremors of essential tremor (ET) or Parkinson's disease (PD) was investigated on the basis of monoaxial accelerometric recordings of 35 s hand tremor epochs. Frequency and amplitude were insufficient to separate between these conditions, except for the trivial distinction between normal and pathologic tremors that is already defined on the basis of amplitude. We found that waveform analysis revealed highly significant differences between normal and pathologic tremors, and, more importantly, among different forms of pathologic tremors. We found in our group of 25 patients with PT and 15 with ET a reasonable distinction with the third momentum and the time reversal invariance. A nearly complete distinction between these two conditions on the basis of the asymmetric decay of the autocorrelation function. We conclude that time series analysis can probably be developed into a powerful tool for the objective analysis of tremors. (c) 1995 American Institute of Physics.

Journal Article↗

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↗

Bilaterally coherent tremor resembling enhanced physiological tremor: report of three cases.

The contribution of the central nervous system to tremor pathogenesis is unclear. Poor side-to-side coherence in physiological, essential, and parkinsonian tremors suggests distinct bilateral generators. By contrast, significant bilateral coherence demonstrated in orthostatic tremor and in enhanced physiological tremor (EPT) in patients with persistent mirror movements favours single or closely linked bilateral oscillators. We describe three patients (aged 21-37 years) who developed unusual bilateral postural and kinetic tremors at 6-13 Hz resembling EPT. The tremor involved all limbs, and in two cases the face or jaw, in the absence of other significant neurological features. Significant side-to-side coherence was demonstrated in each case using cross-correlation of electromyographic recordings from homologous muscle pairs. We postulate that these unusual tremors originate from a single brainstem source or from bilateral oscillators closely linked at or below this level.

Adult↗

Stimulation of the ventral intermediate thalamic nucleus in tremor dominated Parkinson's disease and essential tremor.

Based on Benabid's experimental and clinical findings that low-frequency (50 Hz) electrical stimulation of the ventral intermediate thalamic nucleus may increase tremor, while higher frequencies (> 100 Hz) lead to suppression of the tremor, we implanted a stimulation electrode in 33 thalami among 27 patients. Six patients were implanted bilaterally. 23 suffered from Parkinson's disease, 4 from essential tremor. All patients had a drug-resistant tremor. The Vim target was calculated based on stereotactic ventriculography. An intra-operative neurophysiological target control was performed on all patients. After a monopolar (12 thalami) or quadripolar (21 thalami) lead was implanted we then connected it to a percutaneous extension lead. In the days following the surgery a test stimulation was performed. In all but one patient stimulation resulted in a suppression of the tremor. In a second procedure, a pulse generator (ITREL II; MEDTRONIC) was implanted and connected subcutaneously to the thalamic lead. After implantation of the pulse generator all patients stimulate chronically while some turn off the stimulator at night. In 21 thalami total suppression of tremor was observed, 6 showed major improvement, 4 only minor improvement. There was no significant effect on any other existing symptom of Parkinson's disease. Due to the proximity of Vim to the sensory thalamus the majority of the patients (27 thalami) report slight temporary paraesthesias when the pulse generator is turned on. Two report permanent paraesthesias when stimulation is on. In 4 cases a slight dysarthria occurs under stimulation. In 2 the dysarthria is marked. In one case dysequilibrium occurs under stimulation. All these side effects are reversible when stimulation is turned off. In 3 patients, the lead was displaced due to an insufficient lead fixation, thus making a second procedure necessary to correct the electrode position. We had one complication due to bleeding at the burr hole side. Follow-up ranges from 3 to 48 months. So far in no cases has the effect of stimulation worn off. In conclusion we regard Vim neurostimulation as an effective and safe alternative to conventional thalamotomy and recommend that it should be considered in cases in which drug therapy has failed to affect Parkinsonian or essential tremor. Moreover, we believe that this procedure is a less invasive and equally efficient alternative to classic thalamotomy and thus should be given preference.

Adult↗

Tremor magnitude: a single index to assess writing and drawing in essential tremor.

Hand tremor often causes disability in patients with essential tremor (ET). Aim of the study was to investigate whether tremor magnitude, a new single quantitative score obtained from digital tablet recordings of writing and drawing, is able to adequately reflect disability in ET patients. Mean tremor magnitude values showed significant difference between 14 ET patients and 14 healthy age matched controls (p<0.0001). The tremor magnitude values showed significant correlation with standard methods of clinical assessment (p<0.01). We present tremor magnitude as an index that reflects disability resulting from tremor and can help to evaluate ET.

Adult↗