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

Hypoglycaemia is possibly the most frequent metabolic emergency, in that insulin-induced hypoglycaemia is a common side-effect of treatment of a common disease. The symptoms are partly sympathetic and related to the release of catecholamines. These symptoms include sweating, tremor, palpitations, sensation of hunger, restlessness and anxiety. Other symptoms are caused by an insufficient supply of glucose to the brain, resulting in neuroglucopenia with symptoms like blurred vision, weakness, slurred speech, vertigo and difficulties in concentration. Symptom recognition is the primary and most effective defence against cerebral dysfunction which is the ultimate consequence of hypoglycaemia. Even in insulin-treated diabetic patients symptom failure might occur. Patients who experience severe episodes of hypoglycaemia do not constitute a special subgroup of patients. However, near-normalization of blood glucose levels have resulted in an increase in the incidence of severe hypoglycaemia. Moreover, the threshold for hormonal counter-regulatory responses in adrenaline, growth hormone and cortisol is lowered after a period of strict metabolic control in insulin-dependent diabetic patients. The glucose level at which the patients become subjectively aware of hypoglycaemia is correspondingly reduced. Other reasons for hypoglycaemia to occur are oral hypoglycaemic agents, especially sulfonylureas which may be potentiated by other drugs. Prolonged hypoglycaemia may be seen after first-order sulfonylureas, and may indicate glucose infusion as treatment. Next to insulin and sulfonylurea, ethanol is the most common cause of hypoglycaemia. In non-diabetics, hypoglycaemia will typically develop 6-24 h after a moderate or heavy intake of ethanol by a person who has had an insufficient intake of food for 1 or 2 days. Insulin-producing tumours, insulinomas and non-islet cell tumours may also be reasons for hypoglycaemia in non-diabetics. Treatment of mild episodes of hypoglycaemia is intake of fast-absorbing carbohydrates. Severe episodes can be treated with either i.v. dextrose or glucagon injected i.m. or i.v. The glycaemic response and recovery of a normal level of consciousness is 1-2 min slower after glucagon than after glucose.
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PMID:Endocrine emergencies. Hypoglycaemia. 173 95

As a possible preventive measure for brain dysfunction in Menkes disease, prenatal treatment by maternal administration of zinc, vitamin E and copper was examined in brindled mutant mice. During pregnancy and lactation, female heterozygous mice received 20 ppm zinc or 0.004% alpha-tocopherol acetate (vitamin E) throughout and 6 ppm copper from gestational day 13 in the drinking fluid, ad libitum. The maternal administration of zinc and vitamin E, as antioxidants, or copper resulted in decreased fetal and neonatal death of offspring, especially those of hemizygous males, as compared with the administration of water only. When offspring did not grow, maternal abnormal movements, which comprised rotatory movements of high speed with tremor and ataxia, were frequently observed. In the heterozygotes with abnormal movements, the level of lipid peroxidation in cerebrum and the concentration of copper in kidney were much higher than those in the heterozygotes with normal movement. Morphologically, in cerebellum of the heterozygotes with abnormal movements, the loss of Purkinje cells, abundance of lipofuscin granules and abnormal mitochondria or degenerative bodies of high electron density were frequently observed, as compared with heterozygotes with normal movement. These findings suggest that the development of hemizygous male mice may be influenced by both copper and oxygen radical metabolism.
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PMID:Abnormal movements in brindled mutant mouse heterozygotes: as related to the development of their offspring--biochemical and morphological studies. 216 11

It is useful to divide Parkinsonian patients into those whose signs are confined to tremor, rigidity and akinesia, and those with evidence of a more diffuse disturbance. The treatment of choice in the former is levodopa combined with a peripheral decarboxylase inhibitor. At the onset of the disease, when disability is minimal, amantadine or anticholinergic drugs may suffice. Bromocriptine is useful in some patients who derive only short-lived benefit from each dose of levodopa. The role of stereotactic surgery is now confined to patients with an incapacitating unilateral tremor which has not improved with drug therapy. In elderly patients with evidence of diffuse cerebral dysfunction such as dementia, grasp reflex, hyper-reflexia or severe postural hypotension, the beneficial effect of these drugs is often outweighed by the side effects. Small doses of levodopa alone may be tried. Anticholinergic drugs and amantadine should be avoided in such patients.
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PMID:The management of Parkinson's disease. 704 20

1. Very little is known about the behaviour of children in their second year and even less in their Peer-group. Aggressive behaviour among peers seems to be established at this age and it is interesting to observe it within the Peer-groups. 2. A useful definition of aggression seems to be the following one: Aggression is any physical contact, direct or indirect, by which another person is hurt. 3. We observe five different forms of aggression: 3.1 Aggression by chance, without any aim, happening out of awkwardness and motoric clumsiness. This kind of aggression becomes rarer during the second year. 3.2 Manipulating one another, as shaking arm or leg of another child, pulling his hair, tapping his body and so on, giving the impression of the aggressor exploring his partner. This kind of aggression also becomes rarer during the second year. 3.3 Robbery of objects, i.e. toys being in the hands of another; the reaction of the victim is increasing during the second year. 3.4 Aggression in order to control the group and to establish leadership in connection with the development of a group hierarchy. 3.5 Pathologic Aggression, observed on a child of the group with brain dysfunction. The child overthrows others from behind, climbs on them and bites them in the ear, the neck or the cheek. 4. We still need more observation on Peer-groups of this age to know more about the beginning and development of the here described phenomena.
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PMID:[Aggression in peer groups during the second year of life]. 731 89

Hypoglycaemia is the most frequent acute complication of insulin-dependent diabetes mellitus. The clinical symptoms of insulin-induced hypoglycaemia can be grouped into those attributable to the sympathetic and adrenergic responses, e.g. tremor, pallor, palpitation, sweating, mydriasis ('hypoglycaemia awareness'), and those attributable to brain dysfunction, ranging from headache to convulsions and coma. Hypoglycaemia in diabetic children can occur at any time of the day, but nocturnal hypoglycaemia is a particular fear and worry. The frequency of mild hypoglycaemia is almost impossible to ascertain and the incidence of severe hypoglycaemia varies between 0.07 and 3.6 episodes per patient-year, though most authors report a range of 0.1-0.2 episode per patient-year. The most frequent causes of hypoglycaemia in diabetic children are deviations from treatment routine such as strenuous exercise, omission of snacks or skipped meals, and gross deviations from the prescribed times of insulin injections and recommended doses of insulin. Other predisposing factors include intensified insulin treatment, improved glycaemic control, young age, longer duration of diabetes and defective counterregulation. The available paediatric studies do not seem to support the suggestion that human insulin impairs the perception of hypoglycaemic symptoms ('hypoglycaemia unawareness') and increases the frequency of hypoglycaemic episodes, but further conclusive studies are needed. Prolonged and recurrent severe hypoglycaemia, particularly in younger children, can cause permanent neuropsychological dysfunction (e.g. learning disabilities) and permanent electroencephalographic abnormalities. Mild hypoglycaemia has also been documented to affect cognitive function, and the performance of neuropsychological tasks can remain decreased for some time (up to several hours) after full clinical recovery from hypoglycaemia. An impending hypoglycaemic attack can usually be averted by the ingestion of 20 g of rapidly absorbed carbohydrate. A severe episode can be effectively treated outside hospital with subcutaneous or intramuscular glucagon (0.5-1.0 mg) or in the hospital by an intravenous bolus of 0.2-0.5 g/kg glucose followed by a continuous glucose infusion. Patient and parent education and vigilant application of diabetes self-care principles are perhaps the most effective means of prevention, but in very young children a less strict metabolic control (higher glycosylated haemoglobin levels) may be necessary.
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PMID:Hypoglycaemia in the diabetic child. 837 14

Human GM1-gangliosidosis is caused by a genetic deficiency of lysosomal acid beta-galactosidase (beta-gal). The disease manifests itself either as an infantile, juvenile or adult form and is primarily a neurological disorder with progressive brain dysfunction. A mouse model lacking a functional beta-gal gene has been generated by homologous recombination and embryonic stem cell technology. Tissues from affected mice are devoid of beta-gal mRNA and totally deficient in GM1-ganglioside-hydrolyzing capacity. Storage material was already conspicuous in the brain at 3 weeks. By 5 weeks, extensive storage of periodic acid Schiff-positive material was observed in neurons throughout the brain and spinal cord. Consistent with the neuropathology, abnormal accumulation of GM1-ganglioside in the brain progressed from twice to almost five times the normal amount during the period from 3 weeks to 3.5 months. Despite the accumulation of brain GM1-ganglioside at the level equal to or exceeding that seen in gravely ill human patients, these mice show no overt clinical phenotype up to 4-5 months. However, tremor, ataxia and abnormal gait become apparent in older mice. Thus, the beta-gal-deficient mice appear to mimic closely the pathological, biochemical and clinical abnormalities of the human disease.
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PMID:Generalized CNS disease and massive GM1-ganglioside accumulation in mice defective in lysosomal acid beta-galactosidase. 906 40

In this study, we use a parametric autoregressive (AR) model to obtain descriptive features of eye tremor movement during fixation. The interest consists in analyzing model parameters to determine the information that can be used as indicator of specific pathophysiology underlying cerebral dysfunction in schizophrenic subjects. We have tested healthy volunteers and schizophrenic medicated and unmedicated patients, to evaluate the treatment effect. The AR model is applied to the eye tremor movement extracted from the eye position signal recorded when subjects are fixating a stationary target. The analysis of the model parameters shows distinct classes, corresponding to a population of subjects among the three kinds included in this study.
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PMID:An autoregressive (AR) model applied to eye tremor movement, clinical application in schizophrenia. 1552 36

Motor disturbances are very common in paediatric neurology. Often families can be reassured that these are just variants of normal development. However, abnormal movements can also be the hallmark of severe brain dysfunction of different and complex origins. This review concentrates on motor disturbances as frequent and important symptoms of inborn errors of metabolism. A structured diagnostic approach is developed taking into account age-dependent physiological developments and pathophysiological responses of gross and fine motor functions. A series of investigations are presented with the primary aim of early diagnosis of treatable conditions. The correct recognition and differentiation of movement disorders (ataxia, rigid akinetic syndrome (Fparkinsonism_), dystonia, athetosis, tremor,and others), spasticity, and neuromuscular disorders, requires profound neurological expertise. A high level of suspicion and close interaction between paediatric neurologists and specialists in inborn errors of metabolism are indispensable to effectively and timely identify patients in whom motor disturbances are the presenting and/or main symptom of an inborn error.
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PMID:Inborn errors of metabolism and motor disturbances in children. 1973 Oct 74

A 71-year-old man developed postural tremor and was treated as an essential tremor patient. Nine years after the tremor onset, he developed symptoms resembling Fragile-X-associated tremor/ataxia syndrome (FXTAS), including exacerbated (increased coarseness and amplitude) tremor in the right arm, ataxic gait, and brain MRI showed lesions in the bilateral middle cerebellar peduncles (MCP). Evidence of premutation in the form of 83 CGG repeats of the Fragile-X-mental retardation 1 (FMR1) gene confirmed the diagnosis of FXTAS. FXTAS causes various neurological symptoms including in some cases tremor resembling essential tremor in the early stages. FMR1 gene premutation should be checked when the patient develops intention tremor, cerebral dysfunction and/or a brain MRI shows MCP lesions.
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PMID:A Japanese case of fragile-X-associated tremor/ataxia syndrome (FXTAS). 2055 44

Complete arginase I deficiency is the least severe urea cycle disorder, characterized by hyperargininemia and infrequent episodes of hyperammonemia. Patients suffer from neurological impairment with cortical and pyramidal tract deterioration, spasticity, loss of ambulation and seizures, and is associated with intellectual disability. In mice, onset is heralded by weight loss beginning around day 15; gait instability follows progressing to inability to stand and development of tail tremor with seizure-like activity and death. Here we report that hyperargininemic mice treated neonatally with an adeno-associated virus (AAV)-expressing arginase and followed long-term lack any presentation consistent with brain dysfunction. Behavioral and histopathological evaluation demonstrated that treated mice are indistinguishable from littermates, and that putative compounds associated with neurotoxicity are diminished. In addition, treatment results in near complete resolution of metabolic abnormalities early in life; however, there is the development of some derangement later with decline in transgene expression. Ammonium challenging revealed that treated mice are affected by exogenous loading much greater than littermates. These results demonstrate that AAV-based therapy for hyperargininemia is effective and prevents development of neurological abnormalities and cognitive dysfunction in a mouse model of hyperargininemia; however, nitrogen challenging reveals that these mice remain impaired in the handling of waste nitrogen.
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PMID:AAV-based gene therapy prevents neuropathology and results in normal cognitive development in the hyperargininemic mouse. 2338 1


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