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

Neuroblastoma, which is primarily an extracranial disease, has remote manifestations which can be confusing. Neurologic signs and symptoms such as opsoclonus (dancing eyes), ataxia, blindness, and cord paralysis may precede by several months the manifestations of the primary tumor in a distant site such as the abdomen. Descriptions of nine cases demonstrate the various modes of presentation and some of the unusual aspects of this disease. The recent advent of computed tomography requires a reevaluation of the neuroradiologic approach to the evaluation of neuroblastoma.
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PMID:Remote effects of neuroblastoma. 9 12

Two malignant canine tumours of peripheral neuronal tissue are described. A mediastinal neuroblastoma was found in a 15-mth-old Boston Terrier with dyspnoea. The tumour compressed adjacent lung and invaded the cervical spinal cord. A retroperitoneal ganglioneuroma was found in a 2-yr-old Labrador with posterior ataxia and atrophy of the muscles of the hind legs. This tumour surrounded adjacent adjacent soft tissues and invaded the overlying vertebrae.
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PMID:Neuroblastoma in the dog. 119 54

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

Acute cerebellar encephalopathy (ACE)--ataxia often associated with opsoclonus, polymyoclonus, and irritability--may be associated with neuroblastoma and should be suspected in a child who presents with ACE. The survival in ten children with ACE associated with neuroblastoma was 100%. Most of the tumors were ganglioneuroblastomas. The abdomen was the most common location for the tumor in this study although it also can be found in the mediastinum. All patients with this syndrome had a localized tumor. With ACTH therapy, ACE may resolve early postoperatively, but it tends to recur in most patients, continuing for as long as 3 years after the initial operation. Treatment with ACTH or prednisone modifies and rapidly clears the symptoms during the acute episode of recurrent ACE as well as helps to resolve subsequent neurologic sequelae. Significant neurologic sequelae persisted in seven of ten patients and included deficits in cognition or intellect, hyperactivity, impulsivity, emotional lability, and mild motor deficits. Seizures were seen in one patient.
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PMID:Clinical outcome in children with acute cerebellar encephalopathy and neuroblastoma. 272 81

A case of a neuroblastoma occurring in the cerebellum of a 73-year-old man is reported. The patient presented with progressive truncal ataxia and was found to have an enhancing tumor mass in the cerebellar vermis. By light microscopy, the tumor was a small cell neoplasm and was similar to medulloblastoma, with areas showing structures suggestive of Homer-Wright pseudorosettes. By electron microscopy and immunoperoxidase techniques, however, the tumor showed convincing evidence of neuronal differentiation. The absence of previous reports of this tumor in the posterior fossa of adults suggests that immunoperoxidase techniques and/or electron microscopy of such small cell tumors may be required for accurate diagnosis.
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PMID:Posterior fossa neuroblastoma occurring in an elderly man. 334 12

Kinsbourne's syndrome (ataxia--opsoclonus--myoclonus) is described. The characteristics of this syndrome are: infections of the upper respiratory tract and enteric system and behavioural disturbances leading to the typical symptomatological triad. Various tests were carried out to determine the presence of neuroblastoma which is often associated (46%) to this syndrome. These tests resulted negative. Differential diagnosis was considered in relation to other conditions which present a characteristic symptomatology similar to Kinsbourne's syndrome. Synthetic ACTH (Synacthen) treatment produced a regression of the clinical symptoms. Based on the examinations carried out, which always resulted negative even after 36 months, and on the drug dependence, the present case was diagnosed as primitive opsoclonus.
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PMID:[Opsoclonic encephalopathy in childhood (Kinsbourne syndrome)]. 383 5

A case of opsoclonus myoclonus ataxia shown not to be due to neuroblastoma was biologically and virologically studied. The presence of interferon was found in the patient's CSF. Its implication in the etiology of cerebellar ataxia is discussed.
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PMID:[Presence of interferon in cerebrospinal fluid in nontumoral opsomyoclonic ataxia]. 619 88

The ataxia-opsoclonus-myoclonus syndrome that was well individualized by Kinsbourne is mostly observed in young children (less than three years old in 90 percent of the cases). From six personal cases, and from a review of ninety cases of the literature, the clinical and etiological features, as well as the evolution of the syndrome, are studied. Prodromes (infectious and digestive manifestations) and comportmental changes usually precede the sudden onset of the clinical triad. Neurologic complementary investigations are typically normal during the acute phase. The frequent association (46 percent of the cases) of this syndrome to a neuroblastoma (usually thoracic) makes it very particular from the etiological point of view. The evolution is identical whatever the type ("isolated" or "tumoral"). Corticotherapy (ACTH or corticoids) is efficient in 60 percent of the cases. But recurrences and cerebral sequelae (mental deficiency and speech disorders) are frequent.
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PMID:[Ataxia-opsoclonus-myoclonus syndrome]. 626 94

Metastasis to the brain or spinal cord parenchyma is extremely rare in cases of neuroblastoma. We present a 3-year-7-month-old boy with neuroblastoma, stage IV, with intraspinal metastasis. He had no neurologic manifestation except incontinentia urinae and ataxia at the terminal stage. His cranial computed tomography scan showed high density areas in both cerebellar hemispheres which seemed to be distant metastatic masses. At autopsy, the metastases in the cerebellum were found to be due to dural infiltration but in the spinal cord parenchyma of the lumbar spine metastases were detected macroscopically. There were multiple distant metastatic areas near the roots, anterior and posterior. The neuroblastoma seemed to have spread along the roots by direct invasion from the meninges. In the future, the number of patients with metastasis into the brain or spinal cord parenchyma will increase because patients with progressive disease could live for a long time as a result of intensive chemotherapy. Observation of these cases will be helpful to clarify the routes of metastasis to these areas.
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PMID:Intraspinal metastasis of neuroblastoma--report of a case detected at autopsy. 664 87

We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.
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PMID:Long-term outcome in children with opsoclonus-myoclonus and ataxia and coincident neuroblastoma. 775 22


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