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

Report is made of two patients with Epilepsia Partialis Continua (E.P.C.) from brain organic damage (since carcinoma metastasis and localized ischemia). Clinical EEG, neuroradiological and anatomo-pathological and therapeutical problems are dealth with the light of a review on previous papers. The EEG by itself is assumed as a not sufficient neurophysiological mean. Long time poligraphic enregistrations during awakeness and sleep havae, on the contrary, produced interesting data. The continuous and localized more or less rhythmic myoclonus, which are the distinctive feature in the E.P.C., were in both the patients confined to the first two fingers of their hands; the more they decreased the deeper was sleep (phase II and III-IV) and almost disappeared in the REM phase. Thus poligraphic enregistrations for E.P.C. patients are maintained as very significant.
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PMID:[Poligraphy during awakeness and sleep in patients with epilepsia partialis continua]. 12 64

The authors report the case of a 71 year-old woman presenting right velopharyngo-laryngeal and skeletal rhythmic myoclonus, associated with visual and phasic disorders. They appeared four months after an infarct in the region of the left posterior cerebral artery. The ischemia was of temporo-occipital location, very probably extending to the region of the left red nucleus, thereby interrupting the dento-olivary pathway. Several drugs which are usually active in the treatment of myoclonus proved ineffective. But carbamazepine at a dose of 600 mg per day was effective from the third day and remained so after seven months. It would seem to act by inhibiting the occurrence of paroxystic discharges of an epileptic type in the olivary body disinhibited by the underlying lesion.
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PMID:[Skeletal and velar myoclonus with vision disorders. Efficacy of carbamazepine]. 357 58

Cardiopulmonary arrest is a test of the brain's tolerance to global ischemia. New insights into the pathophysiology of global ischemia have led to the potential use of early prophylactic anticonvulsants, hypothermia, barbiturate coma, glucose manipulations, calcium-blocking agents, and hemodilution. A wide spectrum of neurologic sequelae may follow global ischemia, ranging from brain death, vegetative states, and impairment of higher intellectual function to syndromes of amnesia and cortical blindness, post-anoxic myoclonus, delayed leukoencephalopathy, and spinal stroke. The distinctive features of these sequelae and their pathophysiologic aspects are discussed. Special attention is given to brain death and prognostication.
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PMID:Cardiopulmonary arrest. Pathophysiology and neurologic complications. 390 62

The present article describes a patient with ischemia in the vascular territory of the posterior inferior cerebellar artery, who developed in addition to other typical signs of the dorsolateral medulla oblongata, myoclonic movements of the ipsilateral vocal cord. Within a period of one year, the myoclonic movement progressed, leading to bilateral involvement of the velo-pharyngeal muscles. The essential neuropathological lesion is considered to be an impairment of the dentato-olivary System. In this respect there is a close relationship to other types of rhythmical myoclonus due to lesions of the brain-stem and cerebellum.
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PMID:[So-called laryngeal nystagmus. Progressive myoclonus as a symptom of a cerebellar and brain stem infarct]. 403 11

The present article describes a patient with ischemia in the vascular territory of the posterior inferior cerebellar artery, who developed, in addition to other typical signs of the dorsolateral medulla oblongata, myoclonic movements of the ipsilateral vocal cord. The essential neuropathological lesion is considered to be an impairment of the dentato-olivary system. In this respect there is a close relationship to other types of rhythmical myoclonus due to lesions of the brain-stem and cerebellum.
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PMID:[Vocal cord myoclonus. A case report on the posterior inferior cerebellar artery syndrome]. 665 4

We studied 53 patients (64% females) with static brain lesions who developed progressive movement disorders. Of these, 50 (94%) had dystonia, 17 (32%) tremor, eight (15%) parkinsonism, seven (13%) myoclonus, and three (6%) chorea. The precipitating insults included perinatal hypoxia/ischemia in 22 (42%), stroke in 12 (23%), head injury in eight (15%), encephalitis in eight (15%), and carbon monoxide poisoning, kernicterus, and radiation necrosis in one patient (2%) each. Among the 30 patients with initial insult occurring at age 2 years or younger (Infant group), distribution of dystonia at follow-up was focal in three (10%), segmental in eight (27%), unilateral in 10 (33%), and generalized in nine (30%). The mean latency between the original injury and onset of movement disorder was 25.5 +/- 16.7 years. Among the nine patients who developed dystonia after an insult occurring between ages 6 and 17 (Childhood group), the distribution of dystonia at follow-up was segmental in two (33%) and unilateral in seven (78%); the mean latency of dystonia onset was 4.9 +/- 7.8 years. Of the 14 patients in the Adult group (injury at age 25 or older), 11 developed dystonia, two developed parkinsonism, and one had carbon monoxide encephalopathy and parkinsonism. The distribution of dystonia in the 11 patients at follow-up was segmental in three (27%) and unilateral in eight (73%). The mean latency of movement disorder onset in the 14 patients of the Adult group was 2.5 +/- 4.9 years. No individuals in the Childhood or Adult groups became left-hand dominant; by comparison, nine of the 30 individuals in the Infant group became left-handed. In conclusion, brain injury at a young age is associated with a longer latency to onset of subsequent movement disorder, a greater tendency to development of generalized dystonia, and a greater probability of altered handedness. These tendencies may result from differences in age-related neuroplasticity.
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PMID:Delayed-onset progressive movement disorders after static brain lesions. 890 76

The antimyoclonic property of the novel antiepileptic drug, gabapentin (1-(aminomethyl) cyclohexane acetic acid), was tested in cardiac arrest-and p,p'-DDT(1,1,1-trichloro-2,2-bis (p-chlorophenyl)ethane)-induced animal models of myoclonus. Gabapentin dose-dependently attenuated myoclonus in posthypoxic rats for more than 3 h. The drug was also found to be effective in controlling the early stages of seizures following the anoxic insult. In contrast, the drug was ineffective in controlling either myoclonus or seizures in p,p'-DDT-treated animals. These results suggest that gabapentin can be used used as an effective therapeutic agent in an acute hypoxia/ischemia-induced neurological disorder. The data further indicate that distinct neurological mechanisms may be operating in the expression of myoclonus among posthypoxic and p,p'-DDT-induced animal models.
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PMID:Antimyoclonic effect of gabapentin in a posthypoxic animal model of myoclonus. 866 53

A 47-year-old male with a 5-year history of palatal myoclonus was found on magnetic resonance imaging (MRI) examination to have an ectatic dominant left vertebral artery that compressed the left inferior olive. Microvascular decompression effectively eliminated his symptoms. This case and a similar case presented here with an ectatic vertebral-basilar system illustrate the value of standard MRI in conjunction with magnetic resonance angiography (MRA) in evaluating palatal myoclonus, and they suggest a potential role for decompressive surgery when persistent, highly symptomatic inferior olivary ischemia or compression occurs.
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PMID:Palatal myoclonus secondary to vertebral artery compression of the inferior olive. 1114 1

Mongolian gerbils are epilepsy-prone animals. In adult gerbils two major groups can be differentiated according to their seizure behavior: Highly seizure-sensitive gerbils exhibit facial and forelimb clonus or generalized tonic-clonic seizures from the first test on, while kindled-like gerbils are seizure free for the first three to six consecutive tests, later develop forelimb myoclonus, and eventually progress to generalized tonic-clonic seizures. In the hippocampus, seizure history of the individual animal is mirrored in the intensity in which GABAergic neurons are immunostained for the calcium-binding protein parvalbumin: they lose parvalbumin with increasing seizure incidence. In a first step to clarify the influence of hippocampal projection neurons on spontaneous seizure behavior and related parvalbumin expression, we induced degeneration of the CA1 pyramidal cells by transient forebrain ischemia. This results in a decreased seizure sensitivity in highly seizure-sensitive gerbils. The kindling-like process, however, is not permanently blocked by the ischemic nerve cell loss, suggesting that an intact CA1 field is not a prerequisite for the development of seizure behavior. The seizure-induced loss of parvalbumin from the ischemia-resistant interneurons recovers after ischemia. Thus, changes in parvalbumin content brought about by repeated seizures are not permanent but can rather be modulated by novel stimuli.
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PMID:Ischemia-induced degeneration of CA1 pyramidal cells decreases seizure severity in a subgroup of epileptic gerbils and affects parvalbumin immunoreactivity of CA1 interneurons. 1125 24

A quantitative analysis of two rat syndromes of myoclonus are presented, modeling myoclonic epilepsy and postanoxic myoclonus. Like the human conditions, both of the models benefit therapeutically from drugs that act on the serotonin system. The rat model of myoclonic epilepsy is associated with a profound loss of serotonin throughout the brain (except in the striatum) and is generated by an oscillator that is synchronized around the midline. The rat model of posthypoxic myoclonus does not demonstrate a significant reduction in serotonin in any location of its brain and is generated by a non-oscillating circuit in the medulla. Although some forms of myoclonic epilepsy may benefit from serotonin drugs because they are caused by a decrease in brain serotonin, our data indicate that posthypoxic myoclonus is not caused by a decrease in the serotonergic innervation of any region of the brain. That the raphe nuclei do not degenerate after global brain ischemia was noted by C. David Marsden in a discussion of the histologic findings of three of his human cases of posthypoxic myoclonus (page 117 of reference 10) and led him to question the hypothesis that posthypoxic myoclonus was due to a loss of serotonin neurons. Our data confirm his observation in the rat, but also indicate that density of serotonin fibers and terminals throughout the brain is not reduced by the brain ischemia that produces posthypoxic myoclonus. It remains to be determined whether the physiologic responsiveness of serotonin neurons is altered by global brain ischemia and whether changes in serotonin release or serotonin receptor properties are associated with posthypoxic myoclonus. The stability of the serotonin system in posthypoxic myoclonic rats is remarkable when one considers the wide range of disorders that is produced by the prolonged brain ischemia. The inability of the most severely posthypoxic myoclonic rats to perform 7-Hz tongue protrusions indicates substantial physiologic disruption of brainstem motor function. Moreover, the posthypoxic myoclonic rat suffers from ataxia, seizures, retrograde amnesia, and impaired ability to learn. The wide spectrum of these deficits is sharply constrasted by its apparently intact serotonin system. We have identified the inferior olive as a locus that may generate the rhythmic components of tremor and myoclonus in syndromes that are truly associated with a dramatic loss of brainstem serotonin. Serotonin acts within the inferior olive to constrain its rhythmic firing. Without intraolivary serotonin, olivary neurons are predisposed to oscillate continuously, providing a substrate upon which sustained rhythmic spiking may be superimposed. It is clear that such unconstrained rhythmicity produces synchronized whole-body tremor at 10 Hz (33, 41-43). The effects of serotonin to suppress olivocerebellar rhythmicity are mediated by postsynaptic 5-HT2 receptors that reduce the magnitude of the low-threshold calcium conductance, IT. It is notable that dysregulation of this conductance has been associated with hyper-rhythmic states in the thalamus underlying cognitive disorders ranging from depression to tinnitus (49), indicating a common mechanism underlying a variety of neurologic conditions. The identification of a specific brainstem locus (inferior olive), serotonin receptor 5-HT2, and ionic current IT involved in a form of rhythmic myoclonus may provide multiple clues toward which future pharmacotherapies can be directed.
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PMID:The serotonin hypothesis of myoclonus from the perspective of neuronal rhythmicity. 1196 57


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