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Query: UMLS:C0270736 (Essential tremor)
 404 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Forty-seven railroad workers who were exposed to polychlorinated phenols, including dioxin (TCDD), during 1979 while cleaning up the chemical spillage following damage to a tank car filled with these chemicals were followed medically for the subsequent 6 years. Two committed suicide. The initial neurological complaints included a sense of fatigue and muscle aching, both of which have been reported in other individuals following dioxin exposure. On detailed neurological examination in December, 1985, 24 of 45 had dystonic writer's cramp and/or other action dystonias of the hands. None of the involved individuals had a family history of dystonia, and all 24 dated the onset of the dystonia to the first 2 to 3 years subsequent to their toxic exposure. The dystonias varied in severity but were usually mild. No other types of dystonic involvement were recognized. Thirty-five of the 45 individuals also manifested postural and terminal intention tremor which resembled benign essential tremor. None of the involved individuals had a family history of tremor, and all 35 of those affected dated the onset of the tremor to some time subsequent to their toxic exposure. Forty-three of 45 patients had histories and findings suggestive of peripheral neuropathy. This is the first report relating any type of dystonia to prior dioxin exposure and the first report relating action dystonia, such as dystonic writer's cramp, and postural/terminal intention tremor, to toxic exposure of any type.
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PMID:Dystonia and tremor following exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin. 284 55

Among involuntary movements, dystonia is defined as abnormal posturing produced by slow sustained muscle contractions. On the contrary, myoclonus is characterized by sudden shock-like contractions of a muscle or a group of muscles. The electromyogram (EMG) in dystonia shows continuous activity lasting 5 seconds or more. The muscles usually co-contract in the antagonists. In myoclonus the muscle bursts on EMG last usually between 10 and 50 ms. In some cases the bursts last longer, but they are 200 ms at most. Thus, the characteristics of myoclonus is quite different from those of dystonia. There are, however, unusual combination of dystonia and myoclonus. Myoclonic dystonia, in which myoclonic jerks are so severe that crucial dystonic posturing may be ignored, has been reported. Essential tremor, writing tremor and writer's cramp (writing dystonia) and myoclonic writer's cramp are sometimes seen in one family in various combination. It is suggested that there may be pathophysiological relationship between dystonia and myoclonus, although these two movement disorders have different clinical characteristics.
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PMID:[Pathophysiology of involuntary movements--dystonia and myoclonus. Symptomatological view]. 875 4

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is generally considered to be nonfamilial. We report a brother and sister with clinical and pathologic findings characteristic of PSP. Both developed parkinsonism in the eighth decade of life and within 5 years exhibited severe postural instability, bradykinesia, rigidity, dystonia, dysarthria, dysphagia, urinary incontinence, pseudobulbar palsy, and supranuclear oculomotor dysfunction but no tremor. Neither responded to levodopa and/or carbidopa. Their mother and, possibly, maternal grandfather reportedly suffered from a parkinsonian syndrome. Essential tremor occurred in the siblings' father and in two of the brother's three children. Autopsy in the brother at age 81 years and sister at age 79 years revealed changes typical of PSP with atrophy and neurofibrillary tangles in the globus pallidus, subthalamic nucleus, and rostral tegmental brainstem. No Lewy bodies were present. These cases are the first pair of relatives reported with autopsy confirmation of PSP in both and raise the question of genetic predisposition to PSP.
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PMID:Autopsy-proven progressive supranuclear palsy in two siblings. 878 66

Essential tremor (ET), the most common movement disorder in humans, appears to be inherited as an autosomal dominant trait in many families. The familial form is called familial essential tremor (FET), which seems similar to sporadic essential tremor. ET is a cause of substantial disability, particularly in the elderly. The prevalence of Parkinson's disease and dystonia may be increased in families with ET, but other movement disorders are seldom encountered in these families. Here we report the results of a genome-wide scan for FET genes in 16 Icelandic families with 75 affected individuals, in whom FET was apparently inherited as a dominant trait. The scan, which was performed with a 10-cM framework map, revealed one locus on chromosome 3q13 to which FET mapped with a genome-wide significance when the data were analysed either parametrically, assuming an autosomal dominant model (lod score = 3.71), or non-parametrically (NPL Z score = 4.70, p < 6.4 x 10(-6).
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PMID:Mapping of a familial essential tremor gene, FET1, to chromosome 3q13. 1110 30

Essential tremor (ET) is one of the most common movement disorders. However, the etiology and pathogenesis are as yet unknown. Continued research will give us clues to understanding the impact on society, identifying genetic and environmental contributors to the disease, understanding the significance of a sporadic case, the phenotypic spectrum and timing of presentation, and the relationship with other neurologic disorders. Because the condition is both clinically and genetically heterogeneous and there is overlap with these other disorders, such as dystonia, parkinsonism, peripheral neuropathy, and migraine, the definition of phenotype plagues research in this area. Advances in understanding the genetic and molecular underpinnings of tremor should provide additional tools to unravel the clinical phenotype (including physiology), genotype-phenotype relationships, and the epidemiology of tremor.
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PMID:Epidemiology and genetics of essential tremor. 982 96

Essential tremor (ET) is probably the most common movement disorder and is a common cause of social, physical, and psychological handicaps. Its etiology and pathogenesis are unknown. Phenomenologically, ET overlaps and is associated with other disorders of movement, such as parkinsonism and dystonia. There is large variation in the stated prevalence of ET as well as limited availability of epidemiologic studies. Prevalence variations reflect differences in the definition of ET and the methodologies of investigation. The familial and sporadic forms of ET are generally assumed to be similar. The familial form appears to have a narrow phenotype. Wide variation in the reported percentage of patients with positive family history reflects ascertainment and classification differences. Linkage of ET to two different chromosome locations has been reported.
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PMID:Epidemiology and genetics of essential tremor. 1085 46

Implantable devices that interact directly with the human nervous system have been gaining acceptance in the field of medicine since the 1960's. More recently, as is noted by the FDA approval of a deep brain stimulator for movement disorders, interest has shifted toward direct communication with the central nervous system (CNS). Deep brain stimulation (DBS) can have a remarkable effect on the lives of those with certain types of disabilities such as Parkinson's disease, Essential Tremor, and dystonia. To correct for many of the motor impairments not treatable by DBS (e.g. quadriplegia), it would be desirable to extract from the CNS a control signal for movement. A direct interface with motor cortical neurons could provide an optimal signal for restoring movement. In order to accomplish this, a real-time conversion of simultaneously recorded neural activity to an online command for movement is required. A system has been established to isolate the cellular activity of a group of motor neurons and interpret their movement-related information with a minimal delay. The real-time interpretation of cortical activity on a millisecond time scale provides an integral first step in the development of a direct brain-computer interface (BCI).
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PMID:Work toward real-time control of a cortical neural prothesis. 1089 85

Based on the claims that transcutaneous electrical nerve stimulation is effective in myoclonic dystonia and essential tremor, we evaluated its acute effects in 5 patients with essential tremor and 2 patients with tremor attributed to peripheral neuropathy using as parameters the Washington Heights-Inwood Genetic Study of Essential Tremor rating scale, self-reported impression, and recording of electromyographic activity. We found no significant improvement in any of the parameters tested.
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PMID:Acute effect of transcutaneous electrical nerve stimulation on tremor. 1253 14

Essential tremor (ET) is one of the most common movement disorders. Although often considered a monosymptomatic disorder (postural and kinetic tremor), ET has more recently been considered a more heterogeneous syndrome, with motor and nonmotor features. The diagnosis is clinical and pharmacologic and surgical therapies exist. ET is frequently misdiagnosed as Parkinson disease or dystonia. The traditional notion of ET as a benign disorder has been challenged by those who view ET as a slowly progressive neurodegenerative disorder.
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PMID:Essential tremor. 2094 25

Essential tremor (ET) is the most common adult movement disorder. Traditionally considered as a benign disease, it can cause an important physical and psychosocial disability. Drug treatment for ET remains poor and often unsatisfactory. Current therapeutic strategies for ET are reviewed according to the level of discomfort caused by tremor. For mild tremor, nonpharmacological strategies consist of alcohol and acute pharmacological therapy; for moderate tremor, pharmacological therapies (propranolol, gabapentin, primidone, topiramate, alprazolam and other drugs); and for severe tremor, the role of functional surgery is emphasised (thalamic deep brain stimulation, thalamotomy). The more specific treatment of head tremor with the use of botulinum toxin is also discussed. Several points are discussed to guide the immediate research into this disease in the near future. Dystonic tremor is a common symptom in dystonia. Diagnostic criteria for dystonic tremor and differential diagnosis with psychogenic tremor and ET are described. Treatment of dystonic tremor matches the treatment of dystonia. In cases of symptomatic dystonic tremor similar to ET, therapeutic strategies would be the same as for ET.
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PMID:Diagnosis and management of essential tremor and dystonic tremor. 2117 30


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