Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Etomidate has been studied in two groups of patients. In Group 1, 50 patients received etomidate 100 micrograms/kg/minute with fentanyl and a muscle relaxant, ventilation being with air and oxygen (50%). The technique gave a smooth, pleasant induction with all patients asleep within 2 minutes. The incidence of pain on infusion was 6% and of myoclonus 6%. Cardiovascular changes were minimal, the most common finding being persistent tachycardia. The mean recovery time was 9.1 minutes. There was no incidence of awareness, recall, or thrombophlebitis, but a 20% incidence of nausea and vomiting. In Group 2, 20 patients received the same dosage of etomidate to supplement spinal anaesthesia for lower abdominal surgery. The technique worked most satisfactorily, with patients falling quietly to sleep within 2-3 minutes with no hiccoughs, coughing or laryngospasm. Six patients exhibited myoclonus, one being severe. In no case did myoclonus interfere with the operation. The cardiovascular system remained stable in all patients. Mean recovery time was 16.1 minutes (range 3-38 minutes). Twitching and restlessness were the main complications during recovery.
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PMID:Etomidate infusion. Its use in anaesthesia for general surgery. 686 59

We report on a new maternally-inherited syndrome characterized by a combination of sensorineural hearing loss, ataxia and myoclonus in a large kindred from Sicily. Hearing loss was the most widespread and sometimes the only symptom found in family members. Sequence analysis of the mitochondrial DNA regions encompassing the tRNA genes revealed the presence of a heteroplasmic insertion at nucleotide position 7472. The insertion adds a seventh cytosine to a six-cytosine run that is part of the mitochondrial tRNASer(UCN) gene. Conformational analysis showed that this mutation is likely to alter the structure of the T psi C loop in the tRNASer(UCN) clover leaf secondary structure. Moreover, the degree of heteroplasmy in blood and muscle was correlated with the clinical phenotype, and homoplasmic mutant hybrids showed decreased complex I activity, low oxygen consumption and high lactic acid output, indicating faulty oxidative phosphorylation. Finally, mutation was absent in 381 unrelated maternal lineages, suggesting specific segregation with the disease. We propose that the C7472 insertion-mutation is pathogenic, and etiologically related to hearing loss and other symptoms that define a novel maternally-inherited clinical entity.
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PMID:Maternally inherited hearing loss, ataxia and myoclonus associated with a novel point mutation in mitochondrial tRNASer(UCN) gene. 758 83

A 38-year-old man with chronic low back pain underwent myelography and was inadvertently injected with ionic contrast medium. Within minutes, he started complaining of muscle spasms in his lower extremities, followed by respiratory distress and myoclonus. Immediate intravenous treatment with fluids, antihistamines, and supplemental oxygen was started. Within 1 hour after the myelogram, he was intubated and paralyzed with a neuromuscular blocking agent. Shortly thereafter, he began receiving triple anticonvulsant therapy and a lumbar drain was inserted to allow for the evacuation of cerebrospinal fluid. Electroencephalographic monitoring, which initially showed that the patient was in status epilepticus, subsequently showed no more episodes of seizure activity. Massive rhabdomyolysis, renal failure, and metabolic derangement were prevented. He was then extubated and regained full consciousness. He was discharged on the 13th day of hospitalization with mild amnesia and some cognitive dysfunction. A review of the literature reveals descriptions of 9 of 15 patients who survived similar episodes. We conclude that prompt identification of the contrast medium error and prompt intervention are crucial to increase significantly the chances of survival. Elective paralysis, anticonvulsant therapy, and cerebrospinal fluid drainage are the recommended modes of treatment.
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PMID:Inadvertent use of ionic contrast material in myelography: case report and management guidelines. 773 26

We reported anesthetic as well as perioperative management for a patient with Creutzfeldt-Jakob disease, a very rare transmissible neuropathy. A 51 year-old woman was scheduled for extirpation of recurrence of acoustic neurinoma. Three months before the operation, she had complained vertigo. After admission to our hospital, she had become progressively dementiated and developed disturbed consciousness. Anesthesia was induced with thiamylal and maintained with 0.7-1.5% isoflurane, nitrous oxide and oxygen. The anesthetic course was uneventful and the recovery from anesthesia was smooth. Postoperatively dementia progressed and myoclonus of extremeties appeared sixth weeks after the operation. Two months after the operation, a diagnosis of Creutzfeldt-Jakob disease was established by characteristic EEG and clinical course. Anesthesia for a patient with dementia was discussed.
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PMID:[Anesthesia for a patient with Creutzfeldt-Jakob disease]. 777 22

An 18-year-old male manifesting intellectual deterioration and negative myoclonus was diagnosed as Lafora disease by the demonstration of Lafora bodies in the skin and muscle biopsy. The cortical evoked potential to electric stimulation of the median nerve at wrist showed the giant SEP, suggesting hyperexcitability of the sensory motor cortex. Cerebral blood flow, and cerebral glucose and oxygen metabolism were investigated by positron emission tomography (PET) in order to clarify the pathophysiological process in Lafora disease. The result showed diffusely decreased cortical glucose metabolic rate and cerebral blood flow, and moderately lowered oxygen metabolic rate. There was no increase in blood flow or glucose and oxygen metabolism in the sensorimotor cortex in spite of the presence of giant SEP. The ratio of the glucose to oxygen metabolism was not different from the mean value obtained from nine normal subjects. Based on these findings and review of the literature, it is unlikely that there is a significant deficit in cortical energy metabolism in patients with Lafora disease and it would be less plausible to have some enzymatic deficits in glucose metabolic process.
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PMID:[Cerebral blood flow and metabolism in Lafora disease]. 778 Dec 35

This study investigated the ability of hyperbaric exposure to antagonize ethanol's anticonvulsant effect on isoniazid (INH)-induced seizures. Drug-naive, male C57BL/6 mice were injected intraperitoneally with saline, 1.5, 2.0, or 2.5 g/kg ethanol followed immediately by an intramuscular injection of 300 mg/kg of INH. The mice were then exposed to either 1 atmosphere absolute (1 ATA) air, 1 ATA helium-oxygen gas mixture (heliox), or 12 ATA heliox at temperatures that offset the hypothermic effects of helium. Ethanol increased the latency to onset of myoclonus in a dose-dependent manner. Exposure to 12 ATA heliox antagonized ethanol's anticonvulsant effect at 2.0 and 2.5 g/kg, but not at 1.5 g/kg. Ethanol also increased the latency to onset of clonus in a dose-dependent manner beginning at 2.0 g/kg. Exposure to 12 ATA heliox antagonized this anticonvulsant effect. When exposed to 12 ATA heliox, the blood ethanol concentrations at time to onset of myoclonus were significantly higher in mice treated with 2.5 g/kg of ethanol as compared with blood ethanol concentrations of mice exposed to 1 ATA air. These findings extend the acute behavioral effects of ethanol known to be antagonized by hyperbaric exposure and support the hypothesis that low-level hyperbaric exposure blocks or reverses the initial action(s) of ethanol leading to its acute behavioral effects.
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PMID:Low-level hyperbaric antagonism of ethanol's anticonvulsant property in C57BL/6J mice. 784 5

In the last 4 years much progress has been made in the understanding of mitochondrial disorders. Point-mutations, deletions and depletion of the mitochondrial genome are associated with disorders like Leber's disease, MERRF (Myoclonus Epilepsia with Ragged Red Fibers), MELAS (mitochondrial Myopathy, Encephalopathy, Lactic acidosis and Stroke-like episodes) and several others. Recently, mitochondrial dysfunctions have been also related to neurodegenerative disorders like Parkinson's disease and to aging. Since the brain depends mostly on mitochondrial energy supply, mitochondrial dysfunctions may affect the nervous system more severely than other tissues causing or worsening diseases and playing a role in the biological deterioration of aging. Furthermore, the mitochondrial energy supply is associated with the production of highly reactive oxygen species. Ninety-five percent of the molecular oxygen is metabolized within the mitochondria by the electron-transport chain so that mitochondria are highly exposed to oxidative stress which may damage selected neuronal populations. Oxygen radicals created during respiration induce mitochondrial dysfunction which accelerates the production of more deleterious species of oxygen. The latter step further increases mitochondrial malfunction, thus intensifying and perpetuating the cycle. These two mechanisms combined may lead to cell death in brain and other tissues with high metabolic rate. Therefore, in neurodegenerative disorders such as Parkinson's disease mitochondrial dysfunction and oxidative stress may cause or worsen the clinical features.
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PMID:Oxidative stress and mitochondrial dysfunction in neurodegeneration. 784 18

The neurophysiological effects of the novel anticonvulsant lamotrigine on the high pressure neurological syndrome, HPNS, were investigated in the rat and nonhuman primate Papio anubis. Rats were exposed to pressure at a rate of 3 ATA per min in a helium/oxygen environment. They were pretreated with either lamotrigine isethionate 15, 30, or 60 mg/kg IP or control vehicle. After 15 and 30 mg/kg there were no changes in onset pressures for any of the grades of tremor or myoclonus. After 60 mg/kg, tremor was much slower, at 7-9 Hz, than the 15-20 Hz seen in controls. Four baboons were exposed to pressure at 0.33 ATA per min in the same environment and treated with lamotrigine isethionate at 7.5 mg/kg/h i.v. Each animal underwent a control and a drug-treated exposure. No changes in the onset or severity of HPNS behavioural signs were observed. However, an increase in alpha wave amplitude of the EEG was almost prevented. In both species sustained myoclonic jerking occurred at pressures similar to those at which seizure activity was observed in control exposures. It is concluded that although lamotrigine is protective in several models of neuronal excitation, it is ineffective in protecting against behavioural signs associated with high atmospheric pressure.
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PMID:Lack of effect of lamotrigine against HPNS in rodent and primate models. 791 27

The neurophysiological effects of 2 novel AMPA/kainate receptor antagonists, GYKI 52466 and LY 293558, on the high pressure neurological syndrome have been investigated in the rat and baboon (GYKI 52466) and rat (LY 293558). Rats were exposed to increasing ambient pressures of helium and oxygen at 3 ATA/min, on one occasion each. GYKI 52466 at 20 mumol/kg i.v. immediately before, followed by 70 mumol/kg/hr i.v. during compression delayed tremor by 85% and myoclonus by 30%, compared with control vehicle, and no side effects were observed. Seizure activity was not affected by any of the doses used. LY 293558 at 36 mumol/kg i.p. delayed tremor and myoclonus (44% and 12%), LY 293558 72 mumol/kg additionally delayed seizure activity (21%). Side effects, principally tranquilization at the higher dose, were also noted. Six baboons were exposed to a maximum pressure of 91 ATA at 0.3 ATA/min, in the same environment, on two occasions. One exposure was treated with an i.v. infusion of GYKI 52466 15.2 mumol/kg/hr, the other with the same volume of control vehicle. Limb and face tremor and myoclonus were delayed and the severity of signs reduced. No seizures were observed in the drug treated group before 91 ATA. EEG changes associated with exposure to pressure were not affected. It is concluded that antagonism at the AMPA/kainate receptor by GYKI 52466 and LY 293558 beneficially alters HPNS signs but in a manner which is dependent on both the drug and species being studied.
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PMID:Protection from high pressure induced hyperexcitability by the AMPA/kainate receptor antagonists GYKI 52466 and LY 293558. 793 94

A 64-year-old male with a history of tuberculous pleuritis at age 29 had received home oxygen therapy since age 58 because of chronic respiratory failure. He was admitted with symptoms of dyspnea at rest and myoclonus at age 62. Because CO2 narcosis occurred twice, we performed intermittent negative pressure ventilation (INPV) after short-term positive pressure ventilation with transnasal intubation. He has received INPV for 7 hours every day at his home for 20 months without acute exacerbation of respiratory failure, and his activity of daily life subsequently improved. In conclusion, INPV seems to be useful for patients with chronic hypercapnic respiratory failure due to lung disease.
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PMID:[Home intermittent negative pressure ventilation in a case of chronic respiratory failure due to old tuberculous pleuritis]. 834 10


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