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)

Polygraphic recordings of EEG, EMG, EKG and respiration were made on three patients with histologically verified subacute spongiform encephalopathy and one patient with anoxic encephalopathy both before and after intravenous diazepam. The records showed cyclical activity occurring about every half minute in which the EEG changes were correlated with myoclonus and cardio-respiratory changes. It is suggested that these cycles represent spontaneous cycles of arousal to which the 1/sec sharp waves are related wnd which result from the same mechanisms that produce other cyclical autonomic phenomena.
Electroencephalogr Clin Neurophysiol 1975 Dec
PMID:Cyclic EEG changes in subacute spongiform and anoxic encephalopathy. 5 37

Neuraminidase was assayed in the frozen autopsy tissues from three patients with I-cell disease and an adult patient with cherry-red spots, myoclonus, cerebellar ataxia and beta-galactosidase deficiency. Both diseases showed normal neuraminidase activity toward neuramine lactose and fetuin in cerebral gray matter, liver and kidney. These results suggest that the neuraminidase deficiency is limited only to some tissues and that this biochemical abnormality is not caused by a primary genetic mutation in these diseases.
Clin Chim Acta 1979 Dec 03
PMID:Neuraminidase in mucolipidoses: normal activity in frozen autopsy tissues from three patients with I-cell disease and adult beta-galactosidase deficiency. 11 81

Two patients had an illness characterized by a positive family history, juvenile onset, macular cherry-red spots, myoclonus, generalized convulsions, and cerebellar ataxia. Neither had dementia, gargoyle facies, bone or joint deformities, or visceromegaly. Vacuolated lymphocytes were not seen in the peripheral blood or bone marrow. Specimens from the rectum and vermiform appendix showed Sudan black B-, Sudan III-, and PAS-positive granules within the neurons of the myenteric plexus. On electron microscopic examination, lysosome-like bodies, membranous cytoplasmic bodies, pleomorphic lamellated bodies, dense bodies, and lipofuscin-like bodies in the neurons were seen, with a suggestion of morphological transitional forms among them. Sialoglycopeptides, especially sialic acid, were increased in the urine, but excretion of acid mucopolysaccharides was normal. Assays of lysosomal enzymes in leucocytes showed normal enzymatic activity. On the basis of the clinical, biochemical, and histological results, we suggest that these two cases and four similar cases reported in the literature be classified differently from the previously described lipidoses, although it is not known whether these cases represent a new entity or merely a clinical variant of juvenile lipidosis.
Arch Neurol 1978 Dec
PMID:Familial juvenile neuronal storage disease. New disease or variant of juvenile lipidosis? 21 59

Intracisternal injection of 5,7-dihydroxytryptamine (5,7-DHT) following treatment with desmethylimipramine induced development of behavioral supersensitivity to the intraperitoneally administered serotonin precursor 5-hydroxytryptophan (5-HTP) in the mouse. This behavioral syndrome, characterized by tremor and muscle twitches (myoclonus), showed a clear dose-response relationship with 5,7-DHT as well as with 5-HTP. Mice lesioned with a low dose of 5,7-DHT (20 micrograms) or a placebo were treated repeatedly with a protein synthesis inhibitor, sycloheximide (45 mg/kg, s.c., every 12 h for up to 10 days). This treatment resulted in a reversible decrease of cerebral protein synthesis varying between 70 and 20% with time between treatments. The myoclonic response to 5-HTP in animals pretreated with 5,7-DHT and by cycloheximide showed a decrease in intensity within 24 h when evaluated quantitatively by an electronic activity monitor, the results of which were confirmed by direct observation. Cycloheximide also exerted a similar, though smaller, effect following full development of sensitivity to 5-HTP over 10 days. These effects may de mediated by inhibition of rapidly turning over serotonin receptor proteins, although their interpretation is somewhat obscured by possible toxic effects of cycloheximide.
Brain Res 1978 Dec 22
PMID:Inhibition of 5,7-dihydroxytryptamine-induced supersensitivity to 5-hydroxytryptophan in mice by treatment with cycloheximide. 31 Mar 31

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.
Arch Neurol 1979 Dec
PMID:Myoclonus with electrocerebral silence in a patient receiving penicillin. 50 51

Two neuraminidase (EC 3.2.1.18) comonents, A and B, were distinguished in cultured skin fibroblasts on the basis of thermolability at 37 degrees C. The more labile component (A) t1/2 = 4.7--5.3 min at 37 degrees C, comprises 66--90% of total neuraminidase activity when determined using sodium (4-methylumbelliferyl-alpha-D-N-acetylneuraminate) (MU-alpha-N) as substrate. Activity was assayed at 0 degrees C for 18 h instead of 37 degrees C to fully determine both thermolabile and thermostable components. Diminished activity was noted in cultured fibroblasts from mucolipidoses I, II and III (MLI, MLII, MLIII) and the cherry-red spot myoclonus syndrome (CRSM) patients when assayed at both 0 and 37 degrees C with either MU-alpha-N or each of a series alpha (2 leads to 3)- and alpha (2 leads to 6)-linked N-acetylneuraminyloligosaccharides. Increased sensitivity and rapidity of analyses were achieved using MJ-alpha-N as substrate in determining neuraminidase activity. Results from two obligate heterozygote MLI cell lines (14.5 and 8.0% of control activity) indicate that the MU-alpha-N substrate could be useful for heterozygote detection.
Clin Chim Acta 1979 Dec 03
PMID:Neuraminidase activity in the mucolipidoses (types I, II and III) and the cherry-red spot myoclonus syndrome. 57 33

A historic review of the development of water-soluble contrast materials for lumbar myelography is presented. This is followed by a discussion of their advantages and complications, as well as a case report of convulsive segmental myoclonus, hypotension, and death after myelography associated with the use of dimeglumine locarmate (Dimer-X). A new nonionic material, metrizamide (Amipaque), is discussed, and its current clinical status is summarized.
Spine (Phila Pa 1976) 1978 Dec
PMID:Complications of water-soluble contrast lumbar myelography. Review of the literature and case report. 74 Dec 41

This article gives an overview of electroencephalographic results obtained in the case of subacute sclerosing panencephalitis. Severe disturbances of background activity have been observed in all patients. Repetitive complexes usually occur transitorily, so that they cannot always be detected in individual cases. Repetitive complexes must be regarded as being typical of the disease, and consideration must be given in this connection to their particular characteristics (repetition frequency, constancy of form, relations to disorders characterized by myoclonus) and the clinical picture.
Psychiatr Neurol Med Psychol (Leipz) 1976 Dec
PMID:[The EEG in subacute sclerosing panencephalitis (SSPE)]. 101 72

The serotonin precursor L-5-hydroxytryptophan is useful therapy for patients with posthypoxic intention myoclonus. L-5-hydroxytryptophan plus carbidopa was administered to eight patients with this disorder or other syndromes in which myoclonus is prominent. This treatment (1) decreased the frequency of occurrence and amplitude of intention myoclonus in two patients with posthypoxic intention myoclonus and in one with idiopathic myoclonus, (2) had no effect in one patient with congenital encephalopathy and myoclonus, and (3) increased the frequency of occurrence and amplitude of myoclonus in two patients with lipid storage disease, one with myoclonic epilepsy, and in an additional patient with idiopathic myoclonus. Therefore, L-5-hydroxytryptophan does not effect improvement in all forms of myoclonus; it should be given with caution because it produces a high incidence of side effects. A patient's response to L-5-hydroxytryptophan therapy may be important in a diagnostic classification of myoclonic syndromes based on differences in indoleamine neurotransmitter function.
Neurology 1976 Dec
PMID:L-5-hydroxytryptophan in treatment of several different syndromes in which myoclonus is prominent. 108 88

A 70 years-old man was admitted at our hospital because of unstable angina pectoris. He had essential hypertension and right hemiplegia from a ischemic stroke two years before admission. On neurologic examination, it was found mental disorientation, unstable emotionality, right spastic hemiparesis with right Babinski sign, and segmental myoclonus affecting the superior lip and the palate (palatal nystagmus) on the right side. On the CT scan, a giant aneurysm of the basilar artery was detected. We conclude that the segmental myoclonus could be explained by ischemic lesions in the Guillain-Mollaret triangle.
Arq Neuropsiquiatr 1992 Dec
PMID:Segmental myoclonus and basilar artery. Giant aneurysm. Case report. 130 61


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