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)

A case of Creutzfeldt-Jakob disease (CJD) with presenting Wernicke encephalopathy (WE)-like symptoms and severe insomnia is presented. An 80-year-old alcoholic man with a 6 month history of tremors, ataxia, memory loss and confabulation, developed profound insomnia, confusion, and delirium with vivid hallucinations. Polysomnography revealed a marked reduction of sleep time, with central-type sleep apnea. Neither myoclonus nor periodic synchronous discharge (PSD) was observed. An autopsy revealed diffuse spongiform changes and astrocytosis throughout the cerebral gray matter, with severe involvement of the mammillary bodies and thalamus. Prion protein (PrP) immunostaining was positive in kuru plaques in the cerebellum, PrP polymorphism at codon 129 was heterozygous Met/Val, and proteinase K resistant PrP (PrP(res)) was demonstrated by Western blotting. The lack of necrotizing lesions in the mammillary bodies, thalamus, and periaqueductal gray matter could rule out WE. The data suggest that the present case of CJD is consistent with PrP(res) type 2 (CJD M/V 2), but was unique in the lack of some typical CJD signs and the presence of signs of WE and sleep abnormalities.
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PMID:Wernicke encephalopathy-like symptoms as an early manifestation of Creutzfeldt-Jakob disease in a chronic alcoholic. 1037 Oct 84

The majority of cancer patients develop pain before death. This pain has been shown to be underdiagnosed and undertreated. Opioid use has increased in the past 20 years in both developing and developed countries. The changing pattern in opioid use has resulted in the emergence of neurotoxicity as a major side effect of the treatment of cancer pain. The syndrome of opioid-induced neurotoxicity (OIN) encompasses delirium, hallucinosis, myoclonus/seizures and hyperalgesia. Increased vigilance can lead to the timely diagnosis of OIN, and strategies for its treatment can be implemented with encouraging results. Identification and modification of risk factors for the development of OIN can help in its prevention and improve the quality of life in advanced cancer patients.
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PMID:Opioid use in cancer pain. Is a more liberal approach enhancing toxicity? 1052 40

We reported a 68-year-old female with Creutzfelt-Jakob disease (CJD) presenting hyperparathyroidism. She was suspected as Creutzfelt-Jakob like syndrome at her initial visit to our hospital because of progressive dementia and high level of serum calcium. Finally she was diagnosed as having CJD by the clinical symptoms including progressive dementia and myoclonus and the characteristic patterns of brain CT and electroencephalogram (EEG). CJD presents a variety of symptoms including progressive dementia, apathy and myoclonus. Hyperparathyroidism and toxicity of lithium, delirium and bismuth have been reported to induce similar symptoms of CJD, which are called Creutzfelt-Jakob like syndrome. Therefore, the diagnosis of CJD would be difficult in cases with CJD accompanied with Creutzfelt-Jakob like syndrome. It is rare for otolaryngologists to examine patients with dementia like CJD. However, we have to keep CJD in mind in the diagnosis of hyperparathyroidism.
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PMID:Creutzfelt-Jakob disease presenting hyperparathyroidism. 1080 21

The occurrence of undesirable side effects due to opioids (delirium, confusion, myoclonus, nausea, emesis) is one of the major complications in the management of pain, especially in chronic cancer pain states. Methadone, as an alternative to morphine, has been proposed in the control of opioid-induced toxicity. Methadone is a synthetic opioid, with mu and delta receptor activity, associated with the capacity to inhibit N-methyl-D-aspartate receptors. Questions have arisen concerning its equianalgesic ratio since its rediscovery over the past few years and are certainly related to its receptor interactions. Aspects of its pharmacology, indications, and switching modalities are discussed here. Opioid rotation is a new tool in the management of cancer pain, deserving more attention.
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PMID:Opioid switch to oral methadone in cancer pain. 1088 15

A 34-year-old pregnant patient, admitted with severe pre-eclampsia and the 'haemolysis, elevated liver enzymes, low platelets' (HELLP) syndrome at 31 weeks gestational age, was treated with magnesium sulphate for imminent eclampsia. The further management was aimed at prolonging gestation. During treatment, a delirium developed with myoclonus. As the foetal condition deteriorated, caesarean section was performed. Afterwards, the delirium proved to have been due to hypocalcaemia. After appropriate treatment the patient recovered. In pre-eclampsia, the calcium metabolism is disturbed. Hypermagnesaemia inhibits parathyroid hormone secretion and can therefore cause hypocalcaemia. In rare cases, this may cause a delirium, which is unusual in pre-eclampsia. If the clinical (neurological) picture in a pregnant woman with severe pre-eclampsia, who is receiving treatment with magnesium sulphate, is not fully understood, hypocalcaemia should be considered.
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PMID:[Hypocalcemic delirium due to magnesium sulphate therapy in a pregnant woman with pre-eclampsia]. 1235 87

Side effects can limit opioid dosage and reduce quality of life. The purpose of this systematic review was to assess the management of opioid side effects in the context of cancer pain management or, in the event that no evidence was available for cancer pain, for chronic noncancer pain. The side effects studied were constipation, pruritus, nausea and vomiting, myoclonus, sedation, respiratory depression, and delirium. Opioid rotation to manage side effects was also studied. For each side effect, we searched MEDLINE and the Cochrane Controlled Trials Register and identified 657 possible titles for inclusion. Of these, 67 studies met inclusion criteria for analysis. The lack of well-designed, randomized controlled trials and the heterogeneity of populations and study designs made the drawing of firm conclusions difficult and precluded performance of meta-analysis. The type, strength, and consistency of evidence for available interventions to manage opioid side effects vary from strong (eg, on the use of naloxone to reverse respiratory depression or constipation) to weak (eg, changing from the oral to epidural route of morphine administration to manage sedation). Well-designed trials in the specified populations are required to furnish clinicians with secure evidence on managing opioid side effects successfully.
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PMID:Management of opioid side effects in cancer-related and chronic noncancer pain: a systematic review. 1462 94

A 55-year-old woman with advanced ovarian cancer and severe pain developed hypoactive delirium after an increase in her opioid dosage. Myoclonus and delirium improved dramatically with the intravenous injection of the acetylcholinesterase inhibitor physostigmine, and this improvement was maintained during the administration of donepezil, an oral medication with similar pharmacodynamic properties. Evidence for a disorder of cholinergic neurotransmission in opioid-induced delirium is discussed, as is the rationale for treatment with acetylcholinesterase inhibitors and other cholinomimetic agents.
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PMID:Treatment of opioid-induced delirium with acetylcholinesterase inhibitors: a case report. 1503 35

Fever is sometimes associated with chill, myoclonus, delirium and convulsion. We previously reported EEG findings of febrile delirium, when we found that 18% of patients showed febrile myoclonus simultaneously with febrile delirium. The purpose of this study is to clarify the clinical features of febrile myoclonus and to investigate the relation to febrile convulsion. Myoclonic episodes were studied in 11 patients, aged 8 months to 11 years. EEG was recorded in eight patients. In the past history, febrile convulsion was noted in two patients and one of them also had febrile delirium. The age range of patients with febrile myoclonus was similar to those developing febrile convulsion except for one case. The duration of febrile myoclonus was usually from several to 30 min, but was longer than 2 h in four patients. Seventy-three percent of patients showed fear, surprise and shouting. EEG was abnormal in four patients and spike components were found in two patients. Myoclonic jerks were seen during the EEG recording in two patients and EEG findings were not concordant with epileptic myoclonic attack. Ten patients were followed for 1-2 years, and none had afebrile seizures. Febrile myoclonus is a benign symptom associated with fever. Mood change, fear or surprise and shouting with myoclonic jerks may suggest action of cytokine on the hypothalamus induced by infection. Febrile myoclonus, delirium and convulsion were seen in one patient in his first 3 years of life. These three symptoms seem to appear in children depending on their predisposition.
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PMID:A clinical study of febrile myoclonus in children. 1516 73

Parkinson's disease is associated with classical Parkinsonian features that respond to dopaminergic therapy. Neuropsychiatric sequelae include dementia, major depression, dysthymia, anxiety disorders, sleep disorders, and sexual disorders. Panic attacks are particularly common. With treatment, visual hallucinations, paranoid delusions, mania, or delirium may evolve. Psychosis is a key factor in nursing home placement, and depression is the most significant predictor of quality of life. Clozapine may be the safest treatment for psychotic features, but more research is needed to establish the efficacy of antidepressant treatments. Dementia with Lewy bodies, the second most common dementia in the elderly, may present in association with systematized delusions, depression, or RBD. Early evidence suggests the utility of rivastigmine, donepezil, low-dose olanzapine, and quetiapine in treating DLB. Parkinson-plus syndromes generally lack a good response to dopaminergic treatment and evidence additional features, including dysautonomia, cerebellar and pontine features, eye signs, and other movement disorders. MSA is associated with dysautonomia and RBD. SND (MSA-P) is associated with frontal cognitive impairments, but dementia, psychosis, and mood disorders have not been strikingly apparent unless additional pathological findings are present. In SDS (MSA-A), impotence is almost ubiquitous; urinary incontinence is frequent; depression is occasional, and sleep apnea should be treated to avoid sudden death during sleep. OPCA neuropsychiatric correlates await further definition. Progressive supranuclear palsy neuropsychiatric features include apathy, subcortical dementia, pathological emotionality, mild depression and anxiety, and lack of appreciable response to donepezil. CBD usually is recognized by early frontal dementia with ideomotor apraxia, often in the right upper extremity, attended later by poorly responsive unilateral Parkinsonism, with additional signs including cortical reflex myoclonus, limb dystonia, alien limb, oculomotor apraxia when asked to look horizontally, depression, personality changes, and, occasionally, Kluver-Bucy syndrome. The neuropsychiatry of FTDP-17 involves apraxia, executive impairment, personality changes, hyperorality, and occasional psychosis. Future research in these Parkinsonian disorders should target the characterization of neuropsychiatric sequelae and their treatment.
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PMID:The neuropsychiatry of Parkinson's disease and related disorders. 1555 Feb 93

We recently performed a post-mortem examination on a Japanese patient who had a prion protein gene mutation responsible for fatal familial insomnia (FFI). The patient initially developed cerebellar ataxia, but finally demonstrated insomnia, hyperkinetic delirium, autonomic signs and myoclonus in the late stage of the illness. Histological examination revealed marked neuronal loss in the thalamus and inferior olivary nucleus; however, prion protein (PrP) deposition was not proved in these lesions by immunohistochemistry. Instead, PrP deposition and spongiform change were both conspicuous within the cerebral cortex, whereas particular PrP deposition was also observed within the cerebellar cortex. The abnormal protease-resistant PrP (PrP(res)) molecules in the cerebral cortex of this case revealed PrP(res) type 2 pattern and were compatible with those of FFI cases, but the transmission study demonstrated that a pathogen in this case was different from that in a case with classical FFI. By inoculation with homogenate made from the cerebral cortex, the disease was transmitted to mice, and neuropathological features that were distinguishable from those previously reported were noted. These findings indicate the possibility that a discrete pathogen was involved in the disease in this case. We suggest that not only the genotype of the PrP gene and some other as yet unknown genetic factors, but also the variation in pathogen strains might be responsible for the varying clinical and pathological features of this disease.
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PMID:Fatal familial insomnia with an unusual prion protein deposition pattern: an autopsy report with an experimental transmission study. 1563 34


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