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)

Chronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Little has been added to the understanding of this disorder since the detailed, early clinical and pathological descriptions. The spectrum of clinical presentations can be neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical laminar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Alzheimer type II astrocytes and cytoplasmic glycogen granules are characteristic. Recent neuroradiological observations in patients with liver failure have shown a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the pallidum, putamen, and, rarely, mesencephalon. Using clues from a similar MR appearance in patients receiving total parenteral nutrition as well as animals given parenteral manganese, and the knowledge that manganese is cleared by the hepatobiliary system, deposition of manganese in the brain is postulated in patients with CAHD. In this review we describe three cases of CAHD with detailed clinical and radiological documentation and discuss the aforementioned pathogenetic mechanisms.
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PMID:Chronic acquired hepatocerebral degeneration: case reports and new insights. 886 9

Three patients, aged 71, 50, and 41 years, presented with myoclonic activity arising in the relaxation period preceding sleep onset and causing severe insomnia. Polygraphic studies showed that the myoclonic activity began in spinally innervated muscles, propagating at low speed to rostral and caudal muscular segments. Myoclonus arose whenever patients relaxed mentally and showed diffuse electroencephalographic alpha activity and was independent of posture. The jerks disappeared during sleep. Clonazepam afforded symptomatic improvement. Propriospinal myoclonus arising from a spinal generator may be facilitated by changes in supraspinal control related to vigilance levels. When arising during relaxation and drowsiness, it may cause severe insomnia.
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PMID:Propriospinal myoclonus upon relaxation and drowsiness: a cause of severe insomnia. 899 56

Over the past 15 years we have treated 526 patients with severe hyperkinetic movement disorders with tetrabenazine (TBZ), a monoamine-depleting and a dopamine-receptor-blocking drug. We report here the results in 400 patients with adequate follow-up. The response was rated on a scale of 1 to 5 (1 = marked improvement, 4 = no response, 5 = worsening) and was assessed initially and at the last clinic visit. The average duration of TBZ treatment was 28.9 months (+/- 31.1; range, 0.25 to 180 months). The global response rating of 1 (marked improvement) was recorded in 89.2% of 93 patients with tardive stereotypy, 83.3% of 12 with myoclonus, 82.8% of 29 with Huntington's disease, 80.5% of 82 with tardive dystonia, 79.3% of 29 with other movement disorders, 62.9% of 108 with idiopathic dystonia, and in 57.4% of 47 with Tourette's syndrome. The most common side effects included drowsiness (36.5%), parkinsonism (28.5%), depression (15.0%), insomnia (11.0%), nervousness or anxiety (10.3%), and akathisia (9.5%). The side effects were controlled with reduction in the dosage. TBZ is an effective and safe drug for the treatment of a variety of hyperkinetic movement disorders. In contrast to typical neuroleptics, TBZ has not been demonstrated to cause tardive dyskinesia.
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PMID:Long-term effects of tetrabenazine in hyperkinetic movement disorders. 904 Jul 21

A man who showed excessive twitch movement, such as fragmentary myoclonus (FM) and periodic movements in sleep (PMS) predominantly during REM sleep, is reported. He complained of excessive daytime sleepiness (EDS). After examination, his twitch movements were shown not to accompany narcolepsy, and his EDS were considered to originate from nocturnal sleep disturbance caused by FM and PMS.
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PMID:Excessive twitch movements in rapid eye movement sleep with daytime sleepiness. 947 25

Propriospinal myoclonus is a subtype of spinal myoclonus characterized by axial flexion or extension jerks, arrhythmic and prolonged muscle bursts with a pattern of activation consistent with a slow conduction within the propriospinal pathways. We describe a new idiopathic case of flexion propriospinal myoclonus occurring upon relaxation and drowsiness. This syndrome is to be added to the spectrum of movement disorders occurring during drowsiness.
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PMID:[Propriospinal myoclonus induced by relaxation and drowsiness]. 977 76

We studied a 53 year old right-handed patient who presented isolated myoclonus of right facial muscles induced exclusively by language. Twitching significantly hindered speaking and reading performance. MRI and CT-scan revealed no brain lesion. Conventional EEG showed a few spike-waves predominantly in the left hemisphere. Spike-waves increased during drowsiness. An EEG-EMG polygraphic study was performed during stimulation tests which included linguistic tasks and non-verbal/non-linguistic tests. Myoclonus was triggered by speaking and writing but not by non-linguistic tasks. The severity of myoclonus was dependent on the complexity of the language task. Back-averaging of right facial EMG bursts failed to show a reliable EEG-EMG correlation. However, the facial reflex myoclonus might have originated from the left rolandicopercular cortex, as it was triggered by complex language activities. Findings in this case are compared with those reported for other forms of reflex seizure and myoclonus.
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PMID:Facial reflex myoclonus induced by language: a neuropsychological and neurophysiological study. 1043 Dec 91

A 60-year-old hypertensive woman had a pontine hemorrhage that caused slight right hemiplegia, deep sensory disturbance on her right side and dysarthria. Three months after the stroke, she was transferred to our hospital for rehabilitation. Approximately 6 months later, she gradually began to complain of the visual oscillation. Continual, unceasing conjugate vertical/rotatory eye movements were observed. Fixation was momentary at best because of an inability to dampen the spontaneous eye movements. Electrooculography (EOG) showed bilateral vertical/rotatory sinusoidal eye movements of 2.5 Hz frequency and 10- to 35-degree amplitude. Both vertical and horizontal optokinetic nystagmus were absent. Caloric stimulation did not evoke any responses bilaterally. There were no rhythmical movements at similar frequencies in other parts of the body such as palatal myoclonus. MRI revealed not only hematoma mainly at the dorsal pontine tegmentum but also hypertrophy of the inferior olive nucleus, suggesting disruption of the central tegmental tract. Lesions of this tract may be one cause of pendular nystagmus. Several drug therapies were investigated for the nystagmus. There was no response to baclofen 15 mg. Trihexyphenidyl 4 mg was discontinued because of drug-induced hallucinations. Tiapride 600 mg and phenobarbital 90 mg were each slightly effective in reducing both frequency and amplitude of nystagmus. Treatment with clonazepam 1 mg resulted in the striking disappearance of nystagmus. She was aware of this and no longer experienced oscillopsia. Despite the visual benefit, however, the patient did not wish to continue this drug because of drowsiness and muscle relaxation. The potential long-term therapeutic application of clonazepam should be further investigated. To our knowledge, there have been no reports of successful treatment in acquired pendular nystagmus with clonazepam. Therefore, based on this favorable experience, it is suggested that clonazepam should be added to the list of potential therapies for pendular nystagmus.
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PMID:[Acquired pendular nystagmus after pontine hemorrhage]. 1065 2

Despite an initial sedative effect, alcohol disrupts sleep persistently and should not be used as a sleeping aid. Nocturnal withdrawal symptoms may lead to an increased duration of wakefulness, and to tachycardia and sweating in the second half of the night. It is not known by which mechanism alcohol affects sleep; however, effects do not appear to depend on the stimulation of benzodiazepine receptors or the antagonism at adenosine receptors. Alcohol can exacerbate primary sleep disturbances such as sleep apnea and nocturnal myoclonus, and thereby contribute to excessive daytime sleepiness. The sleep of alcoholic patients is characterized by increased sleep latency, and reduced sleep efficiency, total sleep time, slow wave sleep and non-REM sleep. Even during abstinence, the changes in sleep architecture can persist for months or years, and might contribute to a relapse into alcoholism. The use of benzodiazepines or other hypnotics to treat alcohol-related sleep disturbances is not recommended.
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PMID:[Alcohol and sleep disorders]. 1080 84

We report a 15-month-old female infant who had recurrent tongue biting due to hypnic myoclonia. She began to bite her tongue and bleed when she was 9 months old. The tongue biting was accompanied by generalized myoclonus and was seen only during drowsiness. On admission at 15 months of age, she was observed by video-EEG monitoring. There were hypnic jerks with powerful closure of the mouth and severe screaming, but no accompanying evidence of epileptic discharges. The tongue biting with hypnic myoclonia disappeared spontaneously at 22 months of age, and was considered to be an age-dependent phenomenon related to the maturation of the brain and to the development of oral functions.
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PMID:[Recurrent tongue biting due to hypnic myoclonia in infancy]. 1091 78

Mental status changes are second only to headaches as a cause of neurologic consultation in children with systemic cancer, but the literature on these patients is sparse. This study consisted of a review of the consultations because of changes in mental status in patients with pediatric cancer, with analysis of clinical presentation, etiology, underlying cancer, and neuroradiologic findings. Hematologic cancers were the underlying disorder in slightly more than one half of the patients. The majority of children suffered from iatrogenically induced encephalopathy, predominantly opioid-related. Several drugs were associated with depressed sensorium, but a pure metabolic deficit was rare. One third of the patients had multiple causative factors. Neuroimaging studies were particularly helpful in children with unexplained somnolence but were less useful if the patient had hallucinations. The absence of localizing signs in children with unexplained somnolence or stupor did not exclude the presence of structural disease. Altered mental status is frequently observed in children being actively treated for systemic cancer. The majority of children suffer from iatrogenically induced encephalopathy, predominantly opioid-related. The presence of hallucinations favors a toxic-metabolic dysfunction, especially if associated with myoclonus. Under those circumstances a neuroimaging study is usually unrewarding. If the main complaint is unexplained somnolence without hallucinations or delusions, the performance of a brain imaging study is mandatory, even in the absence of localizing signs.
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PMID:Mental status changes in children with systemic cancer. 1216 Sep 72


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