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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Essential tremor can be surgically treated by thalamotomy or deep brain stimulation. During the surgical procedure for both of these procedures, a transient microthalamotomy effect has been observed. This benefit is usually short-lived and appears to be predictive of patient outcome. This report describes a patient who underwent a temporary placement of a stimulating electrode within the ventral intermediate nucleus of the motor thalamus. The procedure was aborted intraoperatively and the patient demonstrated a microthalamotomy effect. This microthalamotomy effect has remained persistent for 24 months, and the patient remains tremor free. This case report lends evidence to the idea that the volume of motor thalamus responsible for tremor may be small and precise. By recognizing the benefit obtained from small lesions with minimal tissue manipulation, surgeons can continue to refine the target for movement disorder surgery.
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PMID:Long-lasting microthalamotomy effect after temporary placement of a thalamic stimulating electrode. 1530 85

Essential tremor (ET) is a most common human movement disorder of unknown etiology. Previous reports have shown that the C677T polymorphism of methylenetetrahydrofolate reductase gene has been associated with neurodegenerative disorders. To investigate the role of methylenetetrahydrofolate reductase gene polymorphisms in essential tremor, we analyzed the alleles and genotypes of methylenetetrahydrofolate reductase (MTHFR) C677T and MTHFR A1298C in a total of 158 unrelated essential tremor patients and compared them with those of 246 unrelated healthy control subjects, using a polymerase chain reaction restriction fragment length polymorphism method. The allele frequency of MTHFR 677T was 35.76% in the essential tremor cases and 30.08% in the controls. We obtained statistically significant results for MTHFR677 and also for MTHFR1298. The MTHFR T677T genotype was overrepresented and was statistically significant. The T677T/A1298A and C677C/C1298C compound genotypes were similarly statistically significant. The C677C/A1298A compound genotype provided protection for essential tremor. In conclusion, the MTHFR 677T, 1298C alleles and MTHFR T677T genotype and T677T/A1298A, and C677C/C1298C compound genotypes are genetic risk factors for essential tremor in Turkey.
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PMID:Association of the C677T and A1298C polymorphisms of methylenetetrahydrofolate reductase gene in patients with essential tremor in Turkey. 1539 52

Essential tremor is the most common movement disorder and has an unknown etiology. Here we report that gamma-aminobutyric acidA (GABA(A)) receptor alpha1-/- mice exhibit postural and kinetic tremor and motor incoordination that is characteristic of essential tremor disease. We tested mice with essential-like tremor using current drug therapies that alleviate symptoms in essential tremor patients (primidone, propranolol, and gabapentin) and several candidates hypothesized to reduce tremor, including ethanol; the noncompetitive N-methyl-D-aspartate receptor antagonist MK-801; the adenosine A1 receptor agonist 2-chloro-N6-cyclopentyladenosine (CCPA); the GABA(A) receptor modulators diazepam, allopregnanolone, and Ro15-4513; and the L-type Ca2+ channel antagonist nitrendipine. Primidone, propranolol, and gabapentin reduced the amplitude (power) of the pathologic tremor. Nonsedative doses of ethanol eliminated tremor in mice. Diazepam, allopregnanolone, Ro15-4513, and nitrendipine had no effect or enhanced tremor, whereas MK-801 and CCPA reduced tremor. To understand the etiology of tremor in these mice, we studied the electrophysiological properties of cerebellar Purkinje cells. Cerebellar Purkinje cells in GABA(A) receptor alpha1-/- mice exhibited a profound loss of all responses to synaptic or exogenous GABA, but no differences in abundance, gross morphology, or spontaneous synaptic activity were observed. This genetic animal model elucidates a mechanism of GABAergic dysfunction in the major motor pathway and potential targets for pharmacotherapy of essential tremor.
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PMID:Genetic essential tremor in gamma-aminobutyric acidA receptor alpha1 subunit knockout mice. 1576 40

We electrophysiologically examined the transition from physiologic tremor to essential tremor in people at risk for familial essential tremor. Two healthy people from different families with hereditary essential tremor were studied on multiple occasions. A 23-year-old man was studied in 1995, 1997, and 2004, and a 44-year-old woman was studied in 1993, 1995, 1997, and 2003. Hand acceleration and forearm electromyographic readings were measured with and without 300-g loading to determine the characteristic frequency-invariant motor unit entrainment of essential tremor. Clinically and electrophysiologically, the man and woman had normal tremor until the last examination, when both exhibited a fine tremulousness in the outstretched hands and frequency-invariant motor unit entrainment at 7.5 and 6.5 Hz, respectively. At no time did either patient exhibit a prominent 8-12 Hz component of physiologic tremor. Essential tremor in young adults may begin at frequencies less than 8-12 Hz, and this electrophysiologic abnormality is detectable when clinical examinations reveal only questionably abnormal tremor. More young adults at risk for essential tremor must be studied to determine whether initial frequencies less than 8 Hz are the rule or the exception. Nevertheless, the data from our 2 patients demonstrate that a prominent 8-12 Hz component of physiologic tremor does not always precede the development of essential tremor; therefore, the origins of essential tremor and the 8-12 Hz component of physiologic tremor may be different.
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PMID:Electrophysiologic transition from physiologic tremor to essential tremor. 1585 70

To investigate the prevalence of Essential Tremor (ET) in Singapore and compare the rates between Singaporean Chinese, Malays, and Indians, a community-based survey among a disproportionate random sample of 15,000 individuals (9000 Chinese, 3000 Malays, 3000 Indians) aged 50 years and above was conducted. In phase 1, trained interviewers conducted a door-to-door survey using a screening questionnaire for Parkinson's disease. In phase 2, medical specialists examined participants who screened positive to evaluate for the presence of postural or kinetic tremor of the upper limbs, or head tremor. Participants with suspected ET had their diagnosis confirmed in phase 3 by a movement disorders specialist and fellow based on the latest core diagnostic criteria. Forty participants with classic ET were identified. The prevalence rate (PR) of ET was 2.37 per 1000 (95% CI: 1.65-3.32), age-adjusted to UICC world standard population. The PR was significantly higher in males (p=0.01) and increased significantly with age (p<0.001). Indians (PR=4.94 per 1000, 95% CI: 2.63-9.04) were 1.8 times more likely to have ET than Chinese (PR=2.77 per 1000, 95% CI: 1.78-4.17) (p=0.08). No Malays with ET were identified. The data suggest that the prevalence of ET increases with age, is higher in males and may be higher amongst Indians.
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PMID:Prevalence of essential tremor in Singapore: a study on three races in an Asian country. 1587 84

We studied essential tremor (ET) cases enrolled in the Essential Tremor Centralized Brain Repository to (1) assess the validity of their diagnoses and (2) characterize the clinical features in a group of highly selected cases who might reflect a far end of the disease spectrum. Our over-arching goal was to provide a perspective of ET that complements that derived from population-based and clinic-based studies. Based on a history and videotaped examination, 94 of 100 ET cases had their diagnoses confirmed; most of the remainder had Parkinson's disease. When compared with ET cases ascertained through populations and clinics, a large proportion had been prescribed medication for tremor (87.2%), had a family history of tremor (88.3%), had rest tremor (33.0%), or had neck tremor (60.6%). One patient had facial tremor, which has not been reported previously. As has been reported once before, a large proportion wore hearing aids (26.9% of the 67 participants age>or=70). In summary, diagnostic validity was high. In terms of their clinical characteristics, the high proportion of cases with severe tremor and varied disease manifestations (neck tremor, rest tremor) make these cases a valuable resource in pathological studies; the high proportion with familial tremor would provide an enriched sample for genetic studies.
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PMID:Essential tremor centralized brain repository: diagnostic validity and clinical characteristics of a highly selected group of essential tremor cases. 1600 7

Essential tremor (ET) is a neurological disease (and possibly a family of diseases) whose most recognisable feature is an action tremor of the hands and occasionally of the voice and head. Current data support the view that CNS gamma-amino-butyric acid (GABA)-ergic mechanisms may underlie ET and that the tremor may be further modulated by peripheral (muscle) adrenoreceptors. Potential pharmacotherapeutic options, targeted to influence the activity of the neurotransmitter GABA within the CNS and the peripheral adrenergic receptors, are part of the current armamentarium to treat ET. As such, primidone and propranolol remain the mainstays of the therapy for ET. Intramuscular injections of botulinum toxin A may play a role in the treatment of voice and head tremor. Surgical options, which are reserved for patients with severe, medically-refractory tremor, provide adequate tremor control in the majority of patients. As with other progressive neurological disorders of late life, the ability to use neuroprotective medications to intervene in the developing disease to either slow or halt the progression of the pathological process, would involve an understanding of underlying disease mechanisms. The understanding of these mechanisms in ET is limited and further study of these mechanisms is critical for the development of such therapies.
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PMID:Benefits and risks of pharmacological and surgical treatments for essential tremor: disease mechanisms and current management. 1611 52

Essential tremor (ET) is a common movement disorder causing an important functional disability. ET is generally regarded as a monosymptomatic disorder, but additional signs may be present. We analyzed postural sway in 19 patients with classic ET and in 19 sex- and age-matched normal controls (NC) to uncover possible abnormalities of balance control. Static posturography was performed with eyes open (EO) and closed during quiet stance and during performance of mental calculation or motor sequence of thumb opposition to the other fingers. No significant differences of center of foot pressure (COP) parameters were observed between patients and controls during quiet standing. Visual deprivation induced a similar worsening of postural sway in both groups. Concomitant performance of a cognitive or motor task did not affect COP area, whereas COP path was significantly modified by the cognitive task in both groups. In all EO conditions, the COP path was significantly lower in NC than in ET, but such offset was related only to the group of ET patients with head tremor. This study demonstrates that balance control is only minimally affected in ET, although patients with head involvement and longer disease duration tend to present a reduced postural stability. The "dual-task effect" is less important in ET than in Parkinson's disease patients.
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PMID:Posturographic analysis of balance control in patients with essential tremor. 1616 Nov 40

Essential tremor (ET) is a movement disorder characterized by a postural or kinetic tremor of the hands, head, or voice. It is typically a familial condition and affects 1% to 4% of the general population. The trait is genetically linked to chromosome 2p in some families. A variant (828C-->G) in exon 7 of the hematopoietic-specific protein 1 binding protein 3 gene (HS1-BP3) on chromosome 2p recently has been found to segregate with ET in 2 families. To determine the frequency of this variant in a larger series, we studied patients with ET, Parkinson disease (PD), and controls without tremor. Affected singletons representing 73 families from the United States with dominantly inherited ET, 35 individuals with PD, and 304 healthy controls older than age 60 were tested for the 828C-->G variant in exon 7 of the HS1-BP3 gene by a BseYI restriction enzyme digest of the polymerase chain reaction product. Heterozygous carriers of the mutant allele were identified in 12 individuals with ET (16.4%) and in 1 individual with PD and postural tremor (3%). All of the healthy controls (608 chromosomes) were homozygous for the wild-type allele. The 828C-->G genetic variant in the HS1-BP3 gene occurs relatively frequently in subjects with ET. The variant may also be found in some individuals with PD and postural tremor. The HS1-BP3 gene plays a putative role in regulating catecholamine and serotonin metabolism, but the functional consequences of the amino acid substitution (A265G) caused by this genetic variant is unknown.
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PMID:HS1-BP3 gene variant is common in familial essential tremor. 1621 13

Tremor is one of the most common involuntary movement disorders seen in clinical practice. In addition to the detailed history, the differential diagnosis is mainly clinical based on the distinction at rest, postural and intention, activation condition, frequency, and topographical distribution. The causes of tremor are heterogeneous and it can present alone (for example, essential tremor) or as a part of a neurological syndrome (for example, multiple sclerosis). Essential tremor and the tremor of Parkinson's disease are the most common tremors encountered in clinical practice. This article focuses on a practical approach to these different forms of tremor and how to distinguish them clinically. Evidence supporting various strategies used in the differentiation is then presented, followed by a review of formal guidelines or recommendations when they exist.
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PMID:Differential diagnosis of common tremor syndromes. 1634 98


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