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

Continuous myoclonus in a localised area of the body was observed in three patients. In two cases the myocloni sometimes developed into motor Jacksonian convulsions. All three patients had neurological signs on the same side as their continuous twitching indicating a lesion of the contralateral hemisphere. The surface EEG did not show changes which could be directly correlated with continuous convulsions in any of the cases. The cause was found to be a malformation of the hemisphere in one case, a recent encephalomalacia in the second and a severe hyperosomolar diabetic electrolyte imbalance in the third. Epilepsia partialis continua Kozevnikov differs from motor Jacksonian epilepsy in the continuous non-attack character and the absence of a "march of convulsions". Pathophysiologically they are both forms of focal cortical status epilepticus.
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PMID:[Epilepsia partialis continua of Kozevnikov (author's transl)]. 40 31

Sodium valproate (Epilim) has been used in the management of 100 patients with previously uncontrolled epilepsy for periods up to 2 years. If all manifestations of epilepsy are considered together, 75% to 100% control of seizures was achieved in 43% of patients, 25% to 74% control in 26%, and no improvement occurred in 31% of patients. Control of 75% to 100% was achieved in 57% of patients with a spike and wave electroencephalogram (EEG) disturbance but only in 35% of those with focal abnormalities, excessive slow activity, or normal records. When the various manifestations of epilepsy were considered individually, the greatest improvement was found among the patients with the minor forms of generalized epilepsy (petit mal absences, myoclonus and atonic attacks) in whom 75% to 100% control was obtained in 67%, compared with 43% of those with major generalized seizures (grand mal) and 30% of those with temporal lobe attacks and other forms of focal epilepsy. Gastrointestinal disturbances and drowsiness were noted as side effects in the early stages of treatment, but the majority of patients tolerated the drug well and many commented on increased mental alertness while taking it. Two patients were over-stimulated and some noticed tremor or twitching as side effects. Some minor abnormalities in blood coagulation studies were noted, but these were transient and did not appear to be of clinical significance. Regular blood counts and biochemical studies have not shown any significant changes. Sodium valproate appears to be a safe and useful anticonvulsant with the advantage that it usually makes patients brighter rather than drowsier. Abnormalities of platelet function have been described in some overseas reports, so that any unexplained bruising or bleeding in a patient taking valproate is an indication for a platelet count and coagulation studies.
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PMID:The anticonvulsant action of sodium valproate (Epilim) in 100 patients with various forms of epilepsy. 40 31

A progressive form of myoclonus-epilepsy was described in an 21 year old girl. Electrophysiological and neuropharmacological studies were carried out. The typical EEG-pattern, the close association of cortical discharges and myoclonic jerks, the form of myoclonus indicated a "pyramidal" myoclonus. Pharmacological data suggested that two possible mechanisms--a partial deafferentation of cortical neurones and a failure of thalamic, extrapyramidal and brain stem reticular formations--might be responsible for the EEG-abnormalities and concomitant myoclonus. Results from neuropharmacological studies demonstrated a suppressive effect (on EEG-discharges and myoclonic jerks) of clonazepam, diazepam, phenobarbital, taurine, levodopa and budipin. Taurine, levodopa and budipin might induce a new aspect in therapy of progressive myoclonus epilepsy and of myoclonus caused by other etiologic factors.
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PMID:[Electrophysiogical and neuropharmacological studies in a patient with progressive myoclonus-epilepsy (author's transl)]. 40 23

Fifteen patients with a variety of myoclonic syndromes were studied clinically, pharmacologically, and physiologically. CSF tryptophan, 5HIAA, and HVA were also measured. Of these patients, 8 were improved to varying degrees by therapy with 5HTP, tryptophan in combination with MAOI (but not tryptophan alone), and clonazepam. This group included 6 cases of post-anoxic myoclonus, one case of post-traumatic myoclonus and one undiagnosed case of non-progressive focal myoclonus and epilepsy. In this group low levels of CSF 5HIAA were found compared to non-responsive cases and controls. Two cases of dysynergia cerebellaris myoclonica, 2 cases of undiagnosed aetiology, 2 cases of essential myoclonus, and one case of palatal myoclonus failed to respond to drug therapy. However, even amongst the responsive group the improvement varied. The most dramatic responses were seen in those patients in whom physiological study suggested that myoclonus was mediated by brain-stem structures. Less dramatic responses were seen in patients in whom the myoclonus appeared to originate from cortical structures. The neurochemical basis of myoclonus responding to 5HT precursors and clonazepam is discussed. It is suggested that such myoclonus arises from a relative hypoactivity of the 5HT neuronal system which results in a release of abnormal responses to sensory stimuli which characterize this type of myoclonus.
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PMID:Clinical, biochemical, and physiological features distinguishing myoclonus responsive to 5-hydroxytryptophan, tryptophan with a monoamine oxidase inhibitor, and clonazepam. 41 60

Action myoclonus secondary to posthypoxic encephalopathy is being seen increasingly with improved resuscitation techniques. A case report describes 5 specific physical and occupational therapeutic techniques for achieving independence in ambulation, transfers and self-care: (1) analysis and segmentation of complex motions into small steps; (2) controlled progression of training; (3) voluntary cessation of abnormal activity (pacing); (4) progressive densensitization to external stimuli; and (5) quantification of progress. Literature review suggests that posthypoxic action myoclonus is secondary to a loss of inhibitory synapses in the brainstem reticular formation due to low serotonin levels. The proposed therapeutic effect of clonazepam, the drug used in this patient, is decreased serotinin degredation. L-5-hydroxytryptamine, an investigative drug, is also therapeutic, for it stimulates increased serotonin production.
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PMID:Action myoclonus following acute cerebral anoxia. 42 May 69

Short-term visual-motor adaptation to magnifying spectacle lenses was studied in normal subjects and in patients with nonacute posterior fossa lesions. When normal subjects, looking through magnifying lenses, pointed open loop to targets without viewing their hands, they initially underestimated the distance (magnification effect). After a 20-minute close-loop training or adaptation exposure period during which they viewed the performance of their hands, a modified visual-motor scheme evolved, compensating for about half of the lens-induced pointing error (adaptation effect). Removal of the lenses after adaptation caused open-loop, overshooting pointing errors (adaptation after-effect). Four patients with remission of cerebellar signs showed normal visual-motor adaptive performance, evidence of ability to recalibrate gain. One patient with persisting cerebellar ataxia was unable to recalibrate gain during close-loop visual-motor training. His history of transient palatal myoclonus implicates a role for the cerebellar-olivary system in calibration of visual-motor gain.
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PMID:Visual-motor adaptation. Quantitative demonstration in patients with posterior fossa involvement. 43 35

Nerve conduction, EEG, visual evoked potentials, electroretinograms and somatosensory evoked potentials were investigated in 3 children with the Bielschowsky-Jansky-type and in 1 child diagnosed as Spielmeyer-Vogt-type of neuronal ceroid lipofuscinosis. Electroretinographic responses were abolished in all of them. Electroencephalograms showed high amplitude, irregular delta-theta activity and spike- or polyspike-wave discharges without localized preponderance. As a characteristic feature for the Bielschowsky-Jansky type grossly enlarged evoked responses to single light flashes were recorded. Somatosensory evoked potentials were increased in amplitude in 2 patients. Myoclonic jerks of the pyramidal type could be elicited by electrical stimuli to the median nerve. The possibility to differentiate certain neurometabolic disorders of childhood by simple electrophysiological parameters is discussed.
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PMID:Evoked potentials in neuronal ceroid lipofuscinosis. 48 41

Observations have been made on two brothers who had progressive ataxia, intention myoclonus and visual failure starting early in the third decade of life. Their parents were consanguineous. The brothers showed bilateral cherry red spots at the maculae and bilateral perinuclear cataracts; their intelligence was preserved. Urine was found to contain large amounts of sialylated oligosaccharides; cultured skin fibroblasts showed deficiency of the enzyme sialidase (neuraminidase). Studies on leucocytes and cultured skin fibroblasts showed aberrant electrophoretic mobilities of six enzymes all of which are known to be glycoproteins, and this has been attributed to excessive amounts of sialic acid on the enzyme molecules. The clinical features together with the biochemical findings indicate that these are further cases of the newly described condition Sialidosis Type 1 and it is suggested that the electrophoretic findings might be typical of the condition.
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PMID:Sialidosis type 1: cherry red spot-myoclonus syndrome with sialidase deficiency and altered electrophoretic mobility of some enzymes known to be glycoproteins. II. Enzymes studies. 49 93

The clinical and pathological features of a fourth patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. It is suggested that the muscular rigidity, abnormal postures, painful muscular spasms, and myoclonus are a product of excessive and abnormal discharges of alpha motor neurones caused by their release from control by spinal internuncial neurones. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurones, most of which were spinal internuncial neurones. Experimental, clinical, and pathological evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar.
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PMID:Spinal internuncial neurones in progressive encephalomyelitis with rigidity. 50 76

Multifocal myoclonus is a well-recognized complication of high doses of penicillin. In man, the site of origin of penicillin-induced myoclonus has not been clearly established, but there is evidence from animal studies that it may originate at a cortical, subcoritcal, or spinal level. We report a case of multifocal myoclonus occurring in a patient receiving large doses of penicillin. The myoclonus appeared when there was no clinical or EEG evidence of upper brain stem or cerebral function. The observations reported suggest that penicillin-induced myoclonus may occur in man and may originate at a caudal brain stem or spinal level.
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PMID:Myoclonus with electrocerebral silence in a patient receiving penicillin. 50 51


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