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)

Special problems of neuroblastoma sympathicum are being demonstrated by means of 10 cases. Although in the past 20 years therapy ahs been improved considerably, the 2-year survival rate in 35--40% was hardly increased. Three children are specially presented: case I with myoclonus encephalopathy, case II with spontaneous regression and case III, where therapy was carried through by radical surgery. Various possible causes of myoclonus encephalopathy are being discussed and, regarding prognosis, assessed favourably. Several samples of excision should be required, since the tumor substance varies histologically. It is further reported about immunological phenomena. We point out especially that, if a tumor is manifested in a child under one year of age, the prospect is very good. The tumor should be excised by radical surgery, and, if vital structures are included, a resection en bloc might be necessary, with re-implantation of important blood vessels. Favourable factors with regard to prognosis are being counted up. Therapy should be carried out individually, depending on a low risk or high risk case.
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PMID:[Neuroblastoma sympathicum problem: our own experiences with 10 neuroblastoma patients]. 39 85

A case of rhythmic myoclonus of the quadriceps related to involvement of the crural nerve by a schwann-cell sarcoma of the thigh is reported. The rhythm of involuntary contractions varies between 35 and 300 per minute. The myoclonic jerks occur rhythmically in the quadriceps only during full extension of the knee. The jerks are inhibited during lengthening of the quadriceps by active or passive flexion of the knee. Clonazepam is very effective in controlling the myoclonus. It is suggested that the myoclonus is the consequence of a response of alpha-motoneurons of the spinal cord to the excitation of Ia fibers of the crural nerve by the tumor. This activation appears to be enhanced by descending excitation pathway and inhibited by activation of Golgi tendon organs of the quadriceps.
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PMID:[Rhythmic quadriceps myoclonia related to sarcomatous involvement of the crural nerve]. 91 62

Case reports of 28 neuroblastoma patients who had opso-myoclonus as their presenting feature are reviewed. As compared with the 30%-34% two-year survival rate for the overall population of patients with neuroblastoma, those who exhibited the opso-myoclonus/neuroblastoma combination had a tumor-free two-year survival rate of 89.3%. This excellent prognosis may be explained partially by earlier diagnosis and a higher percentage (71% vs. 33%) of patients with Stage I, II, and IV-S disease in the opso-myoclonus sub-group. However, these factors are not, of themselves, sufficient to explain totally the differences in survival rate since five of seven patients with Stage III-IV disease also exhibited long-term survival. This raises the question as to whether the neurologic dysfunction in these patients is pathogenetically related to an unknown factor (possibly autoimmune) which also controls growth and spread of the tumor.
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PMID:Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. 125 9

The association between Beckwith-Wiedemann syndrome and hepatoblastoma is well established and relatively commonplace. The occurrence of opsoclonus-myoclonus syndrome in individuals with occult neoplasia is also well documented. However, the development of opsoclonus-myoclonus syndrome in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma has not been reported previously. The list of underlying causes of opsoclonus-myoclonus syndrome should be expanded to include hepatoblastoma, particularly in any child with features suggestive of Beckwith-Wiedemann syndrome.
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PMID:Opsoclonus-myoclonus with Beckwith-Wiedemann syndrome and hepatoblastoma. 131 77

Paraneoplastic syndromes are complexes of symptoms and signs that occur in association with cancer and that are unexplained by the known anatomic and physiologic characteristics of the tumor. Many of these syndromes are neurologic in nature or have consequences for the central or peripheral nervous system. These syndromes have been well characterized in adults. With the exception of opsoclonus-myoclonus, little has been written about the occurrence of such syndromes in children. This review looks at published reports of paraneoplastic syndromes in children and concludes that paraneoplastic syndromes in childhood differ from those seen in adulthood because of differences in both the host and the kinds of neoplasms most prevalent in each age group. Paraneoplastic syndromes may be underreported in childhood because of the difficulty in eliciting specific neurologic complaints from children and because a thorough neurologic examination is often not undertaken as a matter of routine.
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PMID:Nervous system dysfunction in children with paraneoplastic syndromes. 163 46

An 87-year-old male presented with rapidly progressive dementia, disorder of consciousness and myoclonus, suggesting Creutzfeldt-Jakob disease, but with nonspecific EEG and negative CT findings. Autopsy revealed a malignant angiotropic lymphoma with accumulation of tumor cells within small vessels of the brain and visceral organs. Immunohistochemistry disclosed a large cell lymphoma of B cell type.
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PMID:Angiotropic lymphoma (malignant angioendotheliomatosis) presenting with rapidly progressive dementia. 178 63

Substance P-like and somatostatin-like immunoreactivities (SPLI and SLI) were determined in ventricular fluid of patients with chronic pain syndromes and in a comparison group with multiple sclerosis, essential tremor, epilepsy and postanoxic myoclonus. Concentrations of SPLI and SLI were non-significantly decreased by 40% and 33% in chronic pain patients as compared with control patients without pain. There were no differences apparent between subgroups of pain patients (deafferentation pain, neoplasia-induced pain, thalamic pain). High pressure liquid chromatography combined with radioimmunoassay showed marked heterogeneity of SPLI and SLI.
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PMID:Substance P-like immunoreactivity and somatostatin-like immunoreactivity in the ventricular fluid of patients with chronic pain syndromes. 183 80

The combination of opsoclonus, myoclonus, and ataxia in small children suggests the presence of an occult neuroblastoma, and simple laboratory tests rather than sophisticated neuroradiological procedures may point directly to that diagnosis. We described an 18-month-old boy who presented with opsomyoclonus. A small neuroblastoma arising from the left adrenal gland was found by abdominal CT scan and I-131 M. I. B. G. (Metaiodobenzylguanidine) scan. Three and a half years after tumor removal, moderate mental retardation and ataxia persisted without tumor recurrence.
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PMID:[Occult neuroblastoma presenting with opsomyoclonus. A case report]. 193 30

Paraneoplastic syndromes are heterogeneous in their clinical presentations and their associations with particular tumor types and are an important part of the differential diagnosis of neurologic dysfunction in patients with or without a known neoplasm. Patients presenting with one of the more distinctive syndromes, such as subacute cerebellar degeneration, opsoclonus-myoclonus, and the Lambert-Eaton syndrome, should undergo a careful evaluation for the presence of an occult malignancy. The importance of looking for a monoclonal gammopathy in patients with certain polyneuropathies and motor neuron syndromes is also becoming clear. At this time, an autoimmune pathogenesis has been clearly demonstrated only for the Lambert-Eaton syndrome. Specific autoantibodies in other syndromes appear to be valuable diagnostic markers for the presence of an underlying malignancy, but the actual role of these antibodies in producing tissue damage and clinical disease is still unknown.
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PMID:The remote effects of cancer on the nervous system. 254 61

Focal cortical myoclonus is rare. Obvious causes include tumor or atrophy involving the motor strip, but in some cases no cause is apparent. We present 4 patients who started to have focal myoclonus in childhood. All had focal motor seizures as well, and one had recurrent focal motor status epilepticus. All 4 had a mild progressive hemiparesis. Electrographic investigations showed focal epileptic discharges in the contralateral rolandic areas. Radiological studies were unrevealing, but magnetic resonance showed rolandic lesions in 3 patients. At surgery, abnormally wide gyri were found in the distribution demonstrated by magnetic resonance. The pathological substrate was focal cortical dysplasia. All patients have improved considerably following surgery. These findings suggest that focal myoclonus may be due to a rolandic neuronal migration disorder. Visualization of these lesions by magnetic resonance permits development of a surgical strategy leading to optimal treatment of these medically intractable epileptic disorders.
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PMID:Focal cortical myoclonus and rolandic cortical dysplasia: clarification by magnetic resonance imaging. 313 90


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