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 5-year-old boy is reported with spinal myoclonus caused by cervical astrocytoma due to neurofibromatosis. The essential clinical and electromyographical signs of spinal myoclonus are the following: (1) Continuous rhythmical appearance in muscles innervated by the affected segments of the spinal cord. (2) Synchronous myoclonus in muscles innervated by the same spinal segments of one body side, asynchronous myoclonus in muscles of different segments as well as in contralateral muscles of the same segments. (3) Usually stable frequency, which may increase to a manifold under the influence of different stimuli. (4) Accentuation of the myoclonus under mental distress, disappearance during deeper sleep. The pathophysiological basis of spinal myoclonus might be a disinhibition in the area of the spinal formatio reticularis. Supraspinal stimuli can influence the myoclonus.
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PMID:Sinal myoclonus. 9 51

We describe a child with a cerebellar astrocytoma who presented with paroxysmal segmental rhythmic myoclonus. The movement disorder was characterized by focal onset in the left eyelid followed by a sequential march of clinical events. There were no clinical or laboratory findings to suggest brainstem infiltration or cerebral involvement. Marked clinical improvement followed tumour resection. We suggest that the cerebellar lesion was primarily responsible for the movement disorder.
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PMID:Involuntary movements with cerebellar tumour. 366 73

A 10-year-old boy underwent a posterior fossa craniectomy for removal of a grade 2 cerebellar astrocytoma. Dural closure was achieved by the placement of a dural graft. Eight years later the patient developed dementia and myoclonus. Electroencephalography demonstrated generalized slow activity that evolved into a pattern of periodic triphasic waves. Computed tomography scan and magnetic resonance imaging were unremarkable. Brain biopsy confirmed spongiform encephalopathy of the Creutzfeldt-Jakob type. In the light of previous reports of four similar occurrences, and of our own experience with two further cases of this disease, we believe that the cadaveric dura was the source of transmission of Creutzfeld-Jakob disease in our patient. The authors remark the importance of the awareness of this late complication of dural substitutes, both for the diagnosis of possible future cases and for taking preventive measures to stop the spread of the disease.
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PMID:Pediatric Creutzfeldt-Jakob disease: probable transmission by a dural graft. 840 7

A 45-year-old woman developed a rare case of palatal myoclonus with no other neurological signs after undergoing extirpation of a small cerebellar low-grade astrocytoma that was located in the right dentate nucleus. The palatal myoclonus has persisted for 4 years after the operation. Magnetic resonance T2-weighted imaging revealed a high-intensity lesion in the left inferior olive. Palatal myoclonus associated with the removal of cerebellar tumors is unusual but may easily be overlooked.
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PMID:Palatal myoclonus induced by extirpation of a cerebellar astrocytoma. Case report. 960 9

An old antimalarial drug quinacrine is one of the compounds that can inhibit the accumulation of pathogenic prion protein in cultured neuroblastoma cells. Here we report the result of a clinical trial of quinacrine administration to a patient with Creutzfeldt-Jakob disease (CJD). A 37-year-old woman was admitted to our hospital for ataxic gait, hallucination and dementia in August 2001. As a past history she had received an operation for cerebellar astrocytoma and had a cadaveric dura mater graft at 14 years old. After hospitalization she showed signs of anxiety, stimulus-sensitive myoclonus, decorticate rigidity, then deteriorated pursuit eye movement and voluntary limb movement. The ethics committee of Dokkyo University hospital approved this treatment, and the family of the subject was fully informed as to the nature and purpose of the treatment and gave their consent. One week after the administration of 300 mg/day of quinacrine, voluntary limb movement and pursuit eye movement appeared. There was PSD on EEG, but one week after administration of the quinacrine, PSD disappeared, and a theta wave was recognized as the basic activity on the EEG. But her symptoms deteriorated again after 3 weeks, and PSD appeared. Liver dysfunction developed 2 months after administration, and the administration of quinacrine had to be stopped. Quinacrine was administrated to a CJD patient who received a cadaveric dura mater graft and developed CJD 23 years later. The symptoms of the patient were transiently improved with the disappearance of PSD on EEG. Although the mechanism of quinacrine for the central nervous system is still unclear, this case suggests that quinacrine may be clinically one possible drug for treating CJD.
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PMID:[Quinacrine administration to a patient with Creutzfeldt-Jakob disease who received a cadaveric dura mater graft--an EEG evaluation]. 1458 66

During the 3rd International Symposium on Dietary Therapies held in Chicago, Illinois, there was a first-ever, half-day session devoted to the management of adults with epilepsy and other disorders with dietary treatments. Speakers from 3 different continents shared their successes, challenges, and future directions in their management of these patients. Diets used to treat adults included the classic ketogenic diet, the modified Atkins diet, and a low glycemic index treatment. The utility of dietary therapies was demonstrated not only in patients with epilepsy but also patients with propriospinal myoclonus, astrocytoma, type 2 diabetes, obesity, hyperlipidemia, and metabolic disorder. The session provided evidence that dietary therapies are safe and effective in adults.
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PMID:Worldwide dietary therapies for adults with epilepsy and other disorders. 2367 Feb 44

Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration seen after interruption of the dentato-rubro-olivary tract also known as the Guillain-Mollaret triangle (GMT). It is associated with hypertrophic changes of the inferior olive. Commonly reported in adults, this lesion presents with ataxia and oculopalatal myoclonus. Up to date, few cases have been published in the literature that refer to pediatric cases. This diagnosis is particularly important in the setting of brainstem tumor surgery as it should not be confused for tumor recurrence or metastasis, in turn avoiding unwarranted surgical intervention. We present the case of a 15-year-old male who underwent resection of a left superior cerebellar peduncle (SCP) pilocytic astrocytoma. On follow-up, magnetic resonance imaging (MRI) demonstrated evidence of mild residual tumor as well as progressive engorgement of the inferior olivary nucleus (ION). The patient was clinically asymptomatic and has since been observed expectantly without any issues. We were able to pinpoint the most probable location of injury in our patient's GMT. HOD remains a somewhat obscure entity. Its presentation may be early and not accompanied by significant neurologic findings, in contrast to what has been previously reported. Particularly in neoplastic cases, it may represent a diagnostic challenge and could be easily confused for tumor recurrence. A multidisciplinary approach for this entity, as with other pathologies, is of particular importance. Its proper recognition will result in the best outcomes for the patient.
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PMID:Temporal Evolution of Hypertrophic Olivary Degeneration in a Pediatric Patient: A Case Report and Review of Literature. 3324 Jul 5