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

Thirty-one patients with Wilson's disease were evaluated with detailed neurologic and medical examinations. Mean age (+/- SD) at onset was 21 +/- 5 years and at examination was 28 +/- 6 years. Of the 90% of patients who were first treated with penicillamine, 31% deteriorated initially despite therapy, and half never recovered to pretherapy baseline. At the time of our evaluations, the most common neurologic findings were dysarthria (97%), dystonia (65%), dysdiadochokinesia (58%), rigidity (52%), gait and postural abnormalities (42%), and tremor (32%). Chorea and dementia were rare. Twenty-two patients underwent magnetic resonance imaging. All but one of the 19 symptomatic patients had abnormal scans. The three asymptomatic patients had normal scans. Most lesions were seen in the caudate, putamen, subcortical white matter, midbrain, and pons. Generalized brain atrophy was also common. Lesions were less common in the thalamus, cerebellar vermis, midbrain tegmentum, globus pallidus, red nucleus, and dentate nucleus. Dystonia and bradykinesia correlated with putamen lesions, and dysarthria correlated with both putamen and caudate lesions.
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PMID:Clinical assessment of 31 patients with Wilson's disease. Correlations with structural changes on magnetic resonance imaging. 382 91

Wilson's disease is a multisystem disorder. Heart involvement in Wilson's disease, however, has rarely been recognized. A prospective study was undertaken of 53 consecutive patients (28 men and 25 women, mean age of 21.4 years) with Wilson's disease. Electrocardiographic abnormalities occurred in 18 of 53 patients (34 percent), including left ventricular hypertrophy, biventricular hypertrophy, early repolarization, ST depression and T inversion, premature atrial or ventricular contractions, atrial fibrillation, sino-atrial block, Mobitz type 1 atrioventricular block, and tremor artifact. In contrast, 26 medical students and 14 carriers of Wilson's disease as control subjects (mean age of 22.6 years) all showed normal ECG. Eight out of 43 patients (19 percent) demonstrated asymptomatic orthostatic hypotension. An abnormal response to the Valsalva maneuver occurred in six of 18 patients (33 percent). There were two cardiac deaths; one died of repeated ventricular fibrillation (the copper content in the myocardium was 2.28 micrograms/g, and in the bundle of His 1.21 micrograms/g wet weight in the autopsy specimen); and the other, of dilated cardiomyopathy. It is concluded that four modes of cardiac manifestations in Wilson's disease include arrhythmias, cardiomyopathy, cardiac death, and autonomic dysfunction. Such possible cardiac involvement should be added to the clinical picture of Wilson's disease involving the hepatic and central nervous system.
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PMID:Cardiac Wilson's disease. 382 52

The authors report an experiment undertaken with trazodone in the treatment of different forms of pathological involuntary movements. Forty-five subjects were treated for two months; 15 were affected with L-DOPA + decarboxylase inhibitor induced dyskinesias, 9 with choreic or choreoathetosic syndromes, 6 with primary buccolingual dyskinesias, 4 with ticks, 9 with tremors--3 of whom had delirium tremens--and 1 case of Wilson's disease with severe postural dystonia. At the end of treatment there was a considerable improvement in 40 cases (88.9%), 17 of whom (37.8%) had a reduction of over 65% of symptoms. The results were good in all the groups considered; particularly interesting were those obtained in delirium tremens, alcoholic induced tremor, primary buccolingual dyskinesias, L-DOPA + decarboxylase inhibitor induced dyskinesias. Emphasis is placed on the efficacy of the drug in inhibiting postural dystonia in the one case of Wilson's disease. The good tolerance of the drug was confirmed.
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PMID:[Trazodone in involuntary pathologic movements]. 671 59

The per cent and absolute numbers of lymphocytes T and B were calculated in 37 patients with hereditary diseases of the nervous system (torsion dystonia, hepatolenticular degeneration, idiopathic tremor) and in 22 donors. Reactions of active and full spontaneous complement-induced formation of rosettes and B cell detection with surface immunoglobulin receptors were used for this purpose. In all hereditary diseases a reduction was found in the number of lymphocytes and a rise in the ability of the lymphocytes for complement-induced rosette formation. The obtained data should be evaluated, probably, as a secondary immunodeficiency state determined probably by metabolic disturbances playing an important role in the pathogenesis of hereditary nervous system diseases.
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PMID:[T and B immune systems in hereditary extrapyramidal disorders]. 697

In a case of Wilson's disease with flapping tremor, computed tomography demonstrated bilateral low-density areas in the thalamocapsular regions. Computed tomographic findings in Wilson's disease are discussed.
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PMID:Computed tomography in Wilson's disease. 730 6

The interrelationships were studied between catecholaminergic and cholinergic systems in 169 patients with extrapyramidal system diseases: 68 patients with torsion dystonia (58 with the rigid form and 10 with the hyperkinetic form), 10 with Hallervorden-Spatz disease, 61 with hepatolenticular degeneration, and in 40 with idiopathic tremor. The secretion of dopamine (DA), noradrenaline (NA), adrenaline (A) and their precursor--DOPA) as well as the activity of acetylcholinesterase (AChe)--the enzyme disintegrating acetylcholine--were determined. In the rigid form of torsion dystonia and in Hallervorden-Spatz disease reduced secretion of all catecholamines (mainly DA) and DOPA was observed, with decreased AChE activity. In the hyperkinetic form of torsion dystonia the secretion of DA was increased and AChE activity was higher. In the patients with idiopathic tremor the secretion of A and NA was decreased and AChE activity was reduced. In patients with hepatolenticular degeneration the secretion of NA and DA was decreased and that of their immediate precursor DOPA was increased. Changes of AChE activity showed a wide range. The observed disturbances reflect various forms of disturbances in the equilibrium between the catecholaminergic and cholinergic systems which are one of the leading pathogenetic mechanisms in the development of various extrapyramidal syndromes.
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PMID:[Characteristics of central neurotransmitter metabolism in hereditary extrapyramidal disorders]. 732 5

The authors analyse the polymorphism of certain hereditary extrapyramidal system diseases and call attention to their intrafamilial and interfamilial characteristics. Various diseases are described: 5 forms of hepatolenticular degeneration, 2 forms of torsion dystonia, 3 forms of idiopathic tremor, 3 forms of Huntington's chorea. Clinical peculiarities, peculiarities of the course of the disease, and biochemical changes in these diseases are discussed. In the forms associated with rigidity or with hyperkinesis in the same disease (torsion dystonia, Huntington's chorea) abnormalities of various directions were discovered in the metabolism of neurotransmitters and amino acids, which make possible application of differentiated pathogenetic therapy.
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PMID:[Problems of the clinical polymorphism of hereditary extrapyramidal disorders]. 732 4

Tremor is commonly encountered in medical practice, but can be difficult to diagnose and manage. It is an involuntary rhythmic oscillation of a body part produced by reciprocally innervated antagonist muscles. Tremors vary in frequency and amplitude and are influenced by physiologic and psychological factors and drugs. Categorization is based on position, posture, and the movement necessary to elicit the tremor. A resting tremor occurs when the body part is in repose. A postural tremor occurs with maintained posture and kinetic tremor with movement. Various pathologic conditions are associated with tremors. Essential tremor, which is the most common, is postural and kinetic, with a frequency between 4 and 8 Hz, and involves mainly the upper extremities and head. Essential tremor responds to treatment with primidone, beta-blockers, and benzodiazepines. Parkinson's disease causes a 4- to 6-Hz resting tremor in the arms and legs that responds to the use of anticholinergics and a combination of carbidopa and levodopa. Tremor can also be a manifestation of Wilson's disease, lesions of the cerebellum and midbrain, peripheral neuropathy, trauma, alcohol, and conversion disorders. Treatment should be directed to the underlying condition. Stereotactic thalamotomy of thalamic stimulation is a last resort.
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PMID:Tremor disorders. Diagnosis and management. 761 10

During the period 1957-1987, 189 patients with neurological signs and symptoms were referred to the Wilson's disease clinic at Addenbrooke's Hospital, Cambridge. The diagnosis was not confirmed in 52 patients. Thirty-three of these 52 patients were sent with a definite diagnosis of Wilson's disease, and 12 had received chelation treatment. Ten patients were labelled as probable Wilson's disease; in nine cases no diagnosis had been made but Wilson's disease was considered a possibility requiring exclusion. One patient only was mistakenly reported to have Kayser-Fleischer rings. The presenting symptoms were tremor (n = 17), involuntary movements (n = 16), difficulty in walking (n = 12), personality changes (n = 4) and epilepsy (n = 3). The mean delay in referral was 8 years (range 6 months to 20 years). Compared with the number of patients with confirmed neurological Wilson's disease seen in this period (137 patients) the referral diagnosis was correct in only 72% of cases. The reasons for error, both clinical and biochemical, are discussed.
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PMID:Not Wilson's disease: a review of misdiagnosed cases. 789 89

A variety of inheritable metabolic disorders produce movement disorders. A lists of conditions associated with tremor, athetosis, chorea, dystonia and myoclonus are presented as a guide for the differential diagnosis of such abnormal involuntary movements. The list includes aminoacidopathies, lipidoses, mucopolysaccharidoses, mucolipidoses, organic acidemias, mitochondrial cytopathies and disorders of carbohydrate, purine, and metal metabolism. Clinical, pathological and biochemical features of movement disorders of three typical examples, Wilson's disease, Lesch-Nyhan syndrome and glutaric acidemia type 1, are described.
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PMID:[Movement disorders in miscellaneous disorders--inherited metabolic diseases]. 827 72


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