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)

In 13 healthy adult cats chronically implanted with parasagittal electrodes applied to the dural surface, curarization was performed and baseline recordings of the visual evoked response (VER), auditory evoked response (AER), and brainstem auditory evoked response (BAER) were made. Following the procedure of Prince and Farrell (1969), the animals were then given intramuscular doses of 300,000 to 500,000 U/kg of penicillin with the subsequent development of diffuse, bilaterally symmetrical, photosensitive spike-and-wave discharges in the EEG from 1 to 1 1/2 hr later and concomitant facial myoclonus, arrest of movement, and "absence-like" staring in non-curarized animals. The VER, AER, and BAER were monitored at 15-min intervals for several hours during which time the VER consistently decreased in amplitude up to the time at which the first spike-and wave bursts could be elicited by photic stimulation, approximately 1 hr after injection, after which all early components (0-200 msec) of the VER were progressively increased from 150 to 300% until spontaneous spike-and-wave bursts were consistently recorded (1 1/2-2 hr). Coincident with this change, a marked increase in late components (200-500 msec) was also observed. Both th early diminution and later augmentation of the VER were equally observable in visual and nonvisual cortex. Changes in the AER were also recorded with the development of this model, and were similar to those of the VER but of a lesser degree. The amplitudes of waves I through V of the BAER were found to increase from 28 to 88% maximal at 1 1/2 hr following penicillin injection. These data and the similarity of this model to human petit mal epilepsy argue against increased inhibitory impulses to the visual system during the ictal discharge being responsible for the subjective loss of visual information during petit mal absence. If the amplitude of the evoked response is directly related to the functional integrity of a sensory system, this suggests that the impairment of sensory input, or absence, during spike-and-wave paroxysm is due to interference with sensory processing rostral to the brainstem ascending auditory pathway, and probably does not occur in primary sensory cortex but rather in cortical or subcortical association tracts.
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PMID:Visual and auditory evoked responses during penicillin-induced generalized spike-and-wave activity in cats. 82 23

Pecularities attending the fixation of antibodies from the sera of patients with arterial hypertension, amyotrophic lateral sclerosis, multiple sclerosis, hepato-cerebral disease, and myoclonus-epilepsy were studied by the indirect Coons' method. Antibrain antibodies, complimentary to various nervous tissue structures always formed in the patients suffering from the mentioned diseases. The antigenic properties of individual components of the brain tissue of the patients failed to change as much as to differ completely from the antigenic properties of the same formations in normal animals. At the same time various components of different neurons and glial cells, myelin membranes of various conductive systems, and, to a lesser degree, cells of the ependyma and vascular walls located in various portions of the brain, posessed not only common, but also different antigenic properties.
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PMID:[Fixation of human serum anticerebral antibodies in different regions of the rabbit brain]. 82 60

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.
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PMID:L-5-hydroxytryptophan in treatment of several different syndromes in which myoclonus is prominent. 108 88

An account is given of a form of hereditary, cerebellar ataxia and photomyoclonus. Eight cases from 5 generations were affected. The disease seemed to be transmitted as an autosomal dominant trait. The age at onset usually varied between 35 to 40 years. The symptoms and signs consisted of a cerebellar ataxia, dysarthria and intention tremor. There was no nystagmus. All patients exhibited photomyoclonus and were extremely sensitive to photic stimuli. Other signs were dementia, kyphosis, pes cavus and lipoma localized in the nape of the neck, shoulders and back. Two patients had a partial syndrome with photonyoclonus and skeletal deformities. None of the patients suffered from epilepsy, In one case, histopathological examination revealed atrophy within the cerebellar cortex, dentate nucleus and the posterior columns of the spinal cord. It is concluded that this syndrome belongs to a groups of hereditary ataxias and myoclonus, and differs from myoclonic cerebellar dyssynergia (Ramsay Hunt) and alos from a variety of familial myoclonus and ataxia (Gilbert et al. 1963);
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PMID:Hereditary ataxia, photomyoclonus, skeletal deformities and lipoma. 113 Jan 71

Effects of acute and chronic paleocerebellar stimulation were evaluated in four experimental models of epilepsy in 24 adult cats chronically implanted with bilaterally symmetric parasagittal electrocorticographic electrodes and anterior lobe cerebellar stimulation electrodes. Pentylenetetrazol was given intraveneously in 50-mg increments or 4% enflurane was inspired until grand mal seizures occurred spontaneously or were triggered by photic or auditory stimuli. Alpha-chloralose, 50 mg/kg, was injected intraperitoneally to produce a model of stimulus-sensitive myoclonus and sodium penicillin G, 350,000 units/kg, was injected intramuscularly to produce a model of petit mal epilepsy. One- to 250-Hz electrical stimulation of paleocerebellar cortical surfaces was performed with constant-voltage or constant-current stimulators at threshold and suprathreshold intensities with average intensities of 8 V and 2.5 mA, respectively. Acute or chronic, threshold or suprathreshold paleocerebellar stimulation did not predictably alter the electrographic or clinical manifestations in any of these four models.
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PMID:Effects of acute and chronic paleocerebellar stimulation on experimental models of epilepsy in the cat: studies with enflurane, pentylenetetrazol, penicillin, and chloralose. 114 12

A case of progressive myoclonic epilepsy (P.M.E.) is described. The clinical picture consisted of epileptic seizures, myoclonus and slight mental deterioration associated with a severe progressive cerebellar syndrome. The disease had a course of almost 20 years. Histological studies of the C.N.S. showed severe loss of Purkinje cells, sligth regressive changes in both dentate and olivary nuclei, nerve cells atrophy of anterior horn motoneurons, degeneration of Goll's and Burdach's spino-olivary and anterior spino-cerebellar tracts. Features of cellular lipidosis and/or neuronal amiloid inclusions were not seen. The case was therefore classified in the group of degenerative P.M.E. Its peculiar pathologic aspects consisted of slight but diffuse brain stem regressive changes associated with systemic degeneration involving the spino-cerebellar pathways. The clinical features of our patient emphasize the problem of differential diagnosis between. P.M.E. and D.C.M. thought of by French authors to be an autonomous entity both clinically )severe cerebellar syndrome, intentional myoclonus, absence or late appearance of epilepsy, slight or absent mental deterioration) and anatomically (primary atrophy of the dentate system). However there are many cases, similar to the one reported, which have a clinical course suggesting a diagnosis of D.C.M., but which differ neuropathologically from Hunt's syndrome because of the absence of primary atrophy of the dentate system. These borderline cases give supporting evidence to the concept of continuity between classical P.M.E. and D.C.M., as well as to the authors' opinion that 1unt's syndrome must be classified as a varient of degenerative P.M.E.
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PMID:[Progressive myoclonic epilepsy: anatomo - clinical study of a sporadic case with a marked cerebellar symptomatology (author's transl)]. 123 66

Diagnosis of respiratory chain defects in cultured skin fibroblasts is a difficult diagnostic procedure. We investigated the feasibility of using survival of skin fibroblasts in culture medium with galactose as the major carbon source as a method of quickly diagnosing cell lines that were compromised in oxidative metabolism. We found that cells from patients with most forms of cytochrome oxidase deficiency, cells with complex I deficiency, cells with multiple respiratory chain defects and cells with severe pyruvate dehydrogenase (PDH) complex deficiency failed to survive when subcultured into galactose (5 mM) medium. Cells from patients with Lebers hereditary optic neuropathy (LHON), Kearns-Sayre syndrome (KSS), myoclonus-epilepsy-lactic acidosis-stroke (MELAS), the hepatic form of cytochrome oxidase deficiency, and mild PDH complex deficiency survived well in galactose (5 mM)-containing medium. This could be used as a rapid screening test for skin fibroblasts with major oxidative defects.
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PMID:Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts. 132 73

Two potent glutamate antagonists, NBQX and GYKI 52466, that act selectively on non-NMDA receptors, have been tested for anticonvulsant activity in 3 models of reflex epilepsy (sound-induced seizures in DBA/2 mice and in genetically epilepsy-prone rats and photically-induced myoclonus in Papio papio) and in amygdala kindled rats. Both compounds potently but transiently suppress reflexly-induced epileptic responses. GYKI 52466 also reduces behavioral seizures and afterdischarge duration in amygdala kindled rats, but with a lower potency than it suppresses reflex epilepsy. These data are similar to earlier results with antagonists acting selectively on NMDA receptors; they do not support a specific involvement of enhanced AMPA receptor sensitivity as a major factor in the expression of kindled seizures.
Epilepsy Res Suppl 1992
PMID:The effects of AMPA receptor antagonists on kindled seizures and on reflex epilepsy in rodents and primates. 133 44

The clinical and pathological features of a sporadic case of juvenile neuroaxonal dystrophy beginning at the age of 10 and leading to death at the age of 26 are described. Clinical manifestation began with cerebellar symptoms. The subject subsequently developed dementia, pes cavus (Friedreich's feet), epilepsy, myoclonus, and Parkinsonian syndrome, but demonstrated neither tremor nor choreoathetoid movement. Pathological examination showed typical generalized axonal dystrophy throughout the central nervous system (Seitelberger's disease). Iron-positive pigmentation was seen in the pallidonigral system, diffuse Lewy bodies (brainstem type and cerebral type) were demonstrated in the brainstem nuclei and cerebral cortex, and neurofibrillary tangles were observed.
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PMID:An autopsy case of late infantile and juvenile neuroaxonal dystrophy with diffuse Lewy bodies and neurofibrillary tangles. 137 97

The effects of a serotonin (5-HT) receptor agonist, 5-methoxy-N,N-dimethyltryptamine (5-MeODMT), on epileptic photosensitivity were studied in the lateral geniculate-kindled cat. 5-MeODMT at 4 mg/kg significantly suppressed photically induced myoclonus, but not paroxysmal EEG activity, at 0.5-1 h after injection. This antiepileptic effect was seen in association with the appearance of behavioral signs similar to those seen in the 5-HT syndrome. The present data provide further evidence that 5-HT plays an important role in photosensitive epilepsy, and suggest that the inhibitory effect of 5-MeODMT on photosensitivity results from its agonist action at 5-HT1 receptors.
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PMID:Behavioral and electroencephalographic effects of a serotonin receptor agonist (5-methoxy-N,N-dimethyltryptamine) in a feline model of photosensitive epilepsy. 140 49


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