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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two new cases of infantile myoclonic encephalopathy are reported and a survey of literature is given. The disease is characterized by generalised myoclonic jerks in all striated muscles, by cerebellar ataxia and by fast, jerking, mostly conjugated irregular eye movements (opsoclonus). The disease develops mostly during late infancy and early childhood. The pathogenesis is unknown, probably it is caused by immunological reactions to various agents. Treatment with ACTH or corticosteroids leads to rapid remission of the initial neurological symptoms, but it is suggested that therapy does not prevent frequent sequelae of psychomotor retardation and speech distubances. Remarkably, there is the high coincidence of infantile myoclonic encephalopathy and neuroblastoma. Therefore it is necessary to keep in mind the possibility of a causative neuroblastoma in all children with myoclonic encephalopathy and to control repeatly radiological findings and urin-excretion of catecholamines as well as their metabolic products.
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PMID:[Myoclonic encephalopathy in childhood (author's transl)]. 19 87

We describe 7 children with myoclonic encephalopathy of infants (MEI). MEI is a clinical entity characterized by an acute or subacute onset of polymyoclonia, cerebellar ataxia and opsoclonus ("dancing eyes"). It occurs either spontaneously, following an infectiuos illness or in association with an occult neuroblastoma. It is likely that immunological factors play a role in the pathogenesis. Steroid therapy resulted in rapid dramatic improvement of the neurological symptoms in 4 cases. However, this initial response did not correlate with the eventual outcome. We reviewed the literature to compare 45 reported cases of MEI associated with a neuroblastoma with 48 children without such a tumor to identify possible differences in clinical presentation, response to steroid medication and long-term prognosis of the neurological syndrome. In this respect we found no differences. Impairment of motor, verbal or intellectual performance were reported in at least half the cases. Although an immediate and marked response to steroids occurs in many cases of both groups, it remains unclear whether the long-term outcome is favourably influenced by this medication. The two-year-survival rate (90%) in the neuroblastoma group and the percentage of mediastinal localisation of the tumor (49%) are much higher compared with neuroblastomas without MEI. The reasons for these remarkable differences are not known. Diagnostic, therapeutic and prognostic implications justify the separation of MEI from the more common and benign syndrome known as acute cerebellar ataxia of childhood.
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PMID:Myoclonic encephalopathy of infants or "dancing eyes syndrome". Report of 7 cases with long-term follow-up and review of the literature (cases with and without neuroblastoma). 22 13

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

The neuropathological basis for myoclonic encephalopathy associated with neuroblastoma has never been demonstrated. In our 6-year-old patient, the brain changes were restricted to the cerebellum and consisted of demyelination, gliosis, and loss of Purkinje's cells.
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PMID:Neuropathologic findings in a patient with neuroblastoma and myoclonic encephalopathy. 42 Jun 8

A girl developed subacute sclerosing panencephalitis (SSPE). Eight years earlier she had had measles infection contracted shortly after cytotoxic treatment and radiotherapy for a spinal neuroblastoma. The case illustrates that typical SSPE, like immunosuppressive measles encephalopathy, can arise after drug-induced immunosuppression, and supports the view that these diseases probably represent opposite ends of a spectrum induced by measles virus infection in an individual with some form of immunological deficiency.
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PMID:Subacute sclerosing panencephalitis after drug-induced immunosuppression. 50 19

The case of five-year old boy is reported who at the age of 18 months had successfully been operated upon for neuroblastoma and who had subsequently signs of cerebellar encephalopathy. The paraneoplastic conditions of childhood are discussed in connection with the reported case. Opsoclonus was not observed in the patient, and symptoms showed rapid improvement on methotrexate, carmustine and CCNU treatment. Six months later the child was free of neurological disturbances and only displayed a slight mental retardation (IQ: 88). Cytological alterations observed in the CSF during the cerebellar encephalopathy are described in detail. At present, 41 months after the operation the child is well and free of symptoms.
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PMID:Paraneoplastic syndrome in childhood. 60 50

A case of measles infection without a rash, which was followed by a severe encephalopathy after two months, is described in a 2 1/2 year old boy. At the age of 8 months he had been irradiated for an inoperable intrathoracic neuroblastoma, and at the time of exposure to measles he was being treated with cyclophosphamide and vincristine. This case closely resembles other cases recently described and termed immunosuppressive measles encephalopathy. The syndrome is believed to represent the effect of measles virus in patients with deficient cellular immunity induced by antineoplastic treatment. The importance of protecting children on immunosuppressive treatment for contracting measles is stressed.
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PMID:Immunosuppressive measles encephalopathy. 62 62

Frequently the first clinical sign of neuroblastoma is not caused by local or metastatic tumor growth but is a paraneoplastic symptom (PNS). Such PNS are fever, diarrhea, hypertension, weakness of muscles, Horner's syndrome and myoclonic encephalopathy. Certain PNS disappear with tumor removal, other do not. The clinical importance of PNS is the prognostic and especially diagnostic value. The pathogenetic relations between tumor and PNS as discussed in the literature are interesting but mostly speculative. Effects of Catecholamines and/or immunologic reactions are thought to be the most probable cause of PNS.--The article is based on current literature; in addition, two short case histories are presented.
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PMID:[Paraneoplastic symptoms of neuroblastoma (author's transl)]. 77 98

Five patients with an unusual encephalopathy, possible secondary to measles virus infection, are described. Features common to these patients are: an existing chronic disease, neurologic deterioration 2 1/2 to 6 months after a measles infection, and death several weeks later. These events occurred when the chronic disease (e.g. leukemia or neuroblastoma) was in remission. That the measles virus was the causative agent is suggested only by finding in brain and extracranial tissues intracytoplasmic and intranuclear inclusions which contained measleslike particles. Additional clinical features seen in each of the five patients were: seizures, hypertension, and the inappropriate secretion of antidiuretic hormone.
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PMID:Encephalopathy following measles infection in children with chronic illness. 127 Nov 91

Patients with aluminum-induced encephalopathy syndromes have been shown to have a high level of aluminum concentration in the brain. In the present study, the effects of aluminum were studied in mouse neuroblastoma cells (N-2A) grown in medium supplemented with aluminum (100 microM). It was found that aluminum enhanced neurite growth within 2 days of exposure. The mean total length of neurites in the control after 14 days in culture was 29.8 +/- 4.7 microns, whereas the neurite length of cells pre-exposed to aluminum for 2 days and then maintained in normal media for an additional 12 days was 56.4 +/- 8.9 microns. Further, the duration of exposure did not significantly promote a greater neurite response. The neurite length of cells exposed to aluminum for 14 days (60.7 +/- 9.6 microns) was not statistically different from that of cells exposed to aluminum for 2 days. Using morin stain, intracellular aluminum was detected within 24 h of exposure in the majority of aluminum-exposed cells. Intracellular aluminum did not disappear from those cells even after they were grown for 12 days in control medium. Our finding suggests that a brief exposure (2 days) to low level aluminum (100 microM) is sufficient to cause long-lasting effects on the morphology of neuroblastoma cells in culture. Such neurite behavior associated with aluminum exposure may suggest a morphological basis for the dementia seen in aluminum encephalopathy.
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PMID:Enhanced neurite growth in cultured neuroblastoma cells exposed to aluminum. 128 Jul 92


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