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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myoclonus is a phenomenon which cuts through a considerable number of neurological conditions. It occurs in a variety of epileptic conditions (Primary generalized epilepsy, hypsarrhythmia, Lennox-Gastaut syndrome, also known as "petit mal variant"), in inborn errors of metabolism (Tay-Sachs disease, forms of ceroid lipofuscinosis), in neurobiochemically still poorly understood forms of degenerative processes such as Essential hereditary myoclonus epilepsy (Lafora-Unverricht-Lundborg), in benign heredo-degenerative disorders (Hartung's syndrome), in CNS infections (SSPE, Jakob-Creutzfeldt disease), in metabolic encephalopathies (renal failure, hypoglycemia), in CNS poisoning, in acute cerebral anoxia and in post-anoxic states. The EEG plays a crucial role in the differential diagnosis of these conditions by the demonstration of a) presence or absence of typical inter-ictal abnormalities, and b) various correlates of the myoclonic ictal event.
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PMID:Myoclonus and the electroencephalogram, a review. 11 May 3

A mother and two of her daughters had deafness and cortical reflex myoclonus; the mother also had mild truncal ataxia. Muscle and skin biopsy specimens revealed abundant ragged-red fibres and abnormal mitochondria. The son of one of the daughters had sensorineural deafness. Three other grandchildren were asymptomatic. The two daughters also had diabetes mellitus, hypertension and cardiomyopathy. Another daughter died of renal failure. The mother lost her hearing in her 70s, one daughter in her 30s, and the other daughter and the grandson in their 20s. The mother has had transient episodes (24-48 hours) of temporal disorientation, severe action myoclonus, and ataxia for about eight years. This is the first reported family with inherited deafness, myoclonus, and ataxia with mitochondrial pathology.
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PMID:Abnormal muscle and skin mitochondria in family with myoclonus, ataxia, and deafness (May and White syndrome). 153 18

We studied the effect of apomorphine, a dopamine receptor agonist, on epileptic photosensitivity in 7 patients with progressive myoclonus epilepsy (PME). Specific diagnoses included Baltic PME (Unverricht-Lundborg disease), Lafora disease, Kufs' disease, juvenile neuroaxonal dystrophy, and action myoclonus-renal failure syndrome; 2 patients had PME of uncertain etiology. Apomorphine blocked the epileptic photosensitivity in all patients and also reduced intention myoclonus in a patient with Baltic PME. There is a common deficit of dopaminergic inhibitory neurotransmission at the level of the striate cortex in patients with PME, regardless of the nature of the specific underlying neuropathologic process.
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PMID:Common dopaminergic mechanism for epileptic photosensitivity in progressive myoclonus epilepsies. 190 98

The authors report the development of a rapidly progressive encephalopathy marked by confusion, myoclonus, seizures, coma, and death in a group of women with renal failure who received an oral solution of citrate and aluminum hydroxide gel concurrently. Two patients were documented as having marked hyperaluminemia far exceeding blood aluminum levels encountered in the chronic state of aluminum intoxication. We ascribe the toxicity to enhanced gastrointestinal absorption of aluminum when complexed with citrate.
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PMID:Acute aluminum toxicity associated with oral citrate and aluminum-containing antacids. 291 1

Among patients with renal failure, there have been impressive modifications of both the duration and quality of life as a result of dialysis, renal transplantation, and improved medical management. However, patients who have renal failure continue to manifest a variety of neurologic disorders. Patients with chronic renal failure who have not yet received dialytic therapy may develop a symptom complex progressing from mild sensorial clouding to delirium and coma, with tremor, asterixis, multifocal myoclonus, and seizures. Even after the institution of otherwise adequate maintenance dialysis therapy, patients may continue to be afflicted with more subtle nervous system dysfunction, including impaired mentation, generalized weakness, and peripheral neuropathy. The central nervous system disorders of both untreated renal failure and that persisting despite dialysis are referred to as uremic encephalopathy. The dialytic treatment of end stage renal disease has itself been associated with the emergence of two distinct, new disorders of the central nervous system: Dialysis dysequilibrium and dialysis dementia. The dialysis disequilibrium syndrome consists of headache, nausea, muscle cramps, obtundation and seizures, and is a consequence of the initiation of dialysis therapy in some patients. Dialysis dementia is a progressive, generally fatal encephalopathy which affects patients on chronic hemodialysis. This disease also appears to be a complication of the therapy for renal failure.
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PMID:Pathogenesis of dialysis encephalopathy. 636 3

Metoclopramide hydrochloride is increasingly used as an antiemetic agent. Clinical and experimental studies have demonstrated dopamine antagonism, and extrapyramidal side effects have been reported in patients given the drug for gastrointestinal disorders. Multifocal myoclonic jerking developed in our patient after he received metoclopramide therapy for gastroparesis due to renal failure. He had had no previous neurologic symptoms, and no evidence of CNS abnormality was found; the myoclonic jerking subsided when metoclopramide therapy was discontinued. Multifocal myoclonus must be differentiated from seizure activity in patients with renal failure and other metabolic encephalopathies. Metoclopramide clearance is reduced in renal failure, and myoclonus or other neurologic complications may be precipitated in such patients by usual doses of this drug.
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PMID:Myoclonus induced by metoclopramide therapy. 663 45

Patients with renal failure may manifest a variety of neurologic disorders. Patients with chronic renal failure who have not yet received dialytic therapy may develop a symptom complex progressing from mild sensorial clouding to delirium and coma, with tremor, asterixis, multifocal myoclonus, and seizures. After the institution of adequate maintenance dialysis therapy, patients may continue to be afflicted with more subtle nervous dysfunction, including impaired mentation, generalized weakness, and peripheral neuropathy. These central nervous system disorders are referred to as uremic encephalopathy. The dialytic treatment of end-stage renal disease has itself been associated with the emergence of two distinct, new disorders of the central nervous system; dialysis dysequilibrium and dialysis dementia. The dialysis disequilibrium syndrome consists of headache, nausea, muscle cramps, obtundation, and seizures, and is a consequence of the initiation of dialysis therapy in some patients. Dialysis dementia is a progressive, generally fatal encephalopathy which affects patients on chronic hemodialysis. There are at least three different forms of dialysis encephalopathy: sporadic, epidemic; and that associated with renal disease in children. In addition to the foregoing neurologic diseases which are specifically related to uremia and/or dialysis, a number of other neurologic disorders occur with increased frequency in patients with end-stage renal disease on chronic hemodialysis. These include subdural hematoma, electrolyte disorders, vitamin deficiencies, drug intoxication, hypertensive encephalopathy, and acute trace element intoxication. Renal transplantation is associated with a variety of central nervous system infections, reticulum cell sarcoma, and central pontine myelinosis. The present manuscript will review the clinical, structural, and biochemical components of those neurologic disorders which are peculiar to the uremic state and its treatment with dialysis.
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PMID:Uremic encephalopathies: clinical, biochemical, and experimental features. 675 30

This study is part of a five-year project to investigate the long term effect of chronic hemodialysis on patients with end-stage renal failure. Previous research has associated hemodialysis with progressive dialysis encephalopathy (PDE), which is characterized by speech disturbances, cognitive impairment, myoclonus and behavioral changes. Little is known about the cause or the course of this syndrome except that it begins 14-36 months after treatment onset and usually culminates in death. The purpose of this study was to investigate neuropsychological (cognitive and behavioral) functioning in dialysis patients over a period of years. To date, 34 patients have been studied for 22 months utilizing a cross-sectional method comparing patients at different stages of treatment combined with a longitudinal method of repeated evaluations over time. Current findings show improved cognitive functioning during at least the first year of treatment and no evidence of cognitive deterioration in patients on dialysis for more than one year (M = 4.3). These findings offer strong evidence that PDE is not necessarily a general phenomenon among patients on chronic hemodialysis.
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PMID:Longitudinal study of neuropsychological functioning in patients on chronic hemodialysis: a preliminary report. 715 43

The examination of five pediatric patients with encephalopathy secondary to chronic renal failure has indicated a stereotyped sequence of neurologic signs and symptoms including ataxia, loss of motor abilities, myoclonus, seizures, dementia, and bulbar dysfunction. Both the patients with CNS dysfunction and a control group selected for a similar degree of renal failure had increased levels of serum phosphate, alkaline phosphatase, and parathyroid hormone. Serial EEGs in the affected group revealed progressive slowing and an increase in paroxysmal features. No specific neuropathologic findings were noted in one patient.
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PMID:Encephalopathy in infants and children with chronic renal disease. 729 12

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


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