Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

A rare case of the choroid plexus papilloma originating from the extraventricle was reported. A 48-hear-old woman began not to walk well about two years ago. Then, she was complained of vertigo, hearing disturbance of the right ear and nausea. These symptoms gradually increased. On admission she had papilledema, Brun's nystagmus, hearing disturbance of right ear and cerebellar ataxia. Cerebral angiogram and CT scan showed dilated ventricles and a large tumor in the posterior fossa. At operation, tumor was found in extramedullary space at the right cerebello-pontine angle and extended to the foramen magnum. This was removed totally. Histological examination revealed choroid plexus papilloma. Her postoperative course was satisfactory and shunting procedure was not necessary. According to the electron microscopic findings, the ultrastructure of tumor was similar to that of normal choroid plexus. We could not clear the morphological features which were considered essential for overproduction of CSF in the tumor.
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PMID:[A case of choroid plexus papilloma at the right cerebello-pontine angle (author's transl)]. 30 61

A 63-year-old man developed gradually progressive bilateral loss of vision, cerebellar ataxia, and downbeat nystagmus. Visual acuity was 20/400 OD and 20/200 OS, with cecocentral scotomas OU. Fundus examination showed bilateral optic atrophy and a vitreous cellular reaction. MRI of the brain was normal. CSF protein was elevated, with increased IgG levels but no malignant cells. Biopsy of a pulmonary lymph node showed undifferentiated small cell carcinoma. Neoplastic cells were positive for neuron-specific enolase. Serum contained IgG, which reacted with neuronal and glial cytoplasm and processes. IgG reactivity with systemic tissues and the patient's tumor was not different from that observed with control sera. Paraneoplastic optic neuropathy should be considered in patients with unexplained visual loss and malignancy, and our observations suggest a possible immunologic basis for this condition.
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PMID:Optic neuropathy: a rare paraneoplastic syndrome. 132 6

Three cases in which the signs and symptoms of Chiari I malformation were induced and worsened after the occurrence of supratentorial mass lesions are reported. The symptoms improved markedly after removal of the supratentorial lesions in these cases. In the first case, a meningioma in the right parietal region coexisted with Chiari I malformation. Although the cerebellar ataxia and nystagmus disappeared after tumor removal, decompressive surgery for the malformation was performed because of upper cervical nerve symptoms and the patient recovered completely. In the second case, the symptoms first occurred after a car accident, and a computed tomographic scan revealed not only a subdural hematoma, but also tonsillar herniation due to Chiari I malformation. After evacuation of the hematoma and decompressive surgery on the craniospinal junction, the symptoms disappeared. In the third case, the symptoms of malformation developed gradually with depression and gait disturbance. A meningioma in the left frontal region coexisted with Chiari I malformation. Although the symptoms improved remarkably after tumor removal, decompressive surgery on the craniospinal junction was performed because of upper cervical nerve symptoms, and the patient recovered completely. These clinical findings indicate that a supratentorial mass lesion may provoke the symptoms of Chiari I malformation in the aged.
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PMID:Is Chiari I malformation in the aged initiated by mechanical factors? Report of three cases. 169 48

A 50-year-old female was admitted because of nausea, vomiting, and cerebellar ataxia. Computed tomography scan revealed an enhanced mass accompanied with a cyst in the right cerebellar hemisphere. The mass situated in the subcortical region was removed. Histologically, highly vascular tumor cells lined the cavities. Postoperative radio- and chemotherapy were administered and the clinical symptoms improved gradually. Two months later, the patient complained of dyspnea. Chest X-ray on second admission demonstrated cardiomegaly. Hemorrhagic pericardial effusion amounting to 1000 ml was aspirated by pericardial puncture. Papillary clusters of tumor cells were demonstrated in the pericardial effusion. The patient died of cardiac failure. At necropsy solid tumors were located in the heart, lung, left inguinal region, and cerebellum. Histological diagnosis was mesothelioma arising from the heart. Primary pericardial mesotheliomas are rare; approximately 106 cases have been reported. Pericardial mesothelioma frequently spreads to the adjacent pleura and mediastinum, but distant metastases are extremely rare because patients with pericardial mesothelioma tend to die early due to cardiac failure or cardiac tamponade.
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PMID:[Brain metastasis from primary pericardial mesothelioma. Case report]. 170 70

Performance on an eye-hand tracking task and a multi-joint reaching movement to a visual target was studied in a patient with stable cerebellar ataxia and in control subjects. The patient subsequently died and a full neuropathological examination was performed. The neuropathological findings were similar to those seen in patients with paraneoplastic cerebellar degeneration, but no tumor was found at autopsy eight years after onset of the patient's cerebellar syndrome. A severe cerebellar cortical degeneration with complete Purkinje cell loss was demonstrated, whereas cerebellar nuclei and brainstem structures showed no neuronal loss. Tracking performance by the patient was characterized by abnormally large numbers of high velocity movements and hand direction reversals, and by excessive lagging of the hand behind the target in time. In the multi-joint reaching movement, the patient showed a delay in movement onset at the elbow joint compared to movement onset at the shoulder joint. The velocity profile of the movement at the shoulder joint was abnormal. The duration of the acceleration phase was poorly correlated with both peak angular velocity and the duration of the deceleration phase. One of the most striking findings was the inability of the patient to consistently produce the same movement direction from trial to trial while reaching to the same target. Our data suggests that the cerebellar cortex is involved in multiple aspects of motor control including visuomotor integration mechanisms.
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PMID:Multi-joint reaching movements and eye-hand tracking in cerebellar incoordination: investigation of a patient with complete loss of Purkinje cells. 178 14

A 44-year-old male was admitted for diplopia, headache and gait disturbance. Neurological examination revealed the meningeal irritation sign, the Vth, VII, VIth, IXth and the Xth nerve palsies and cerebellar ataxia of all four limbs. Laboratory studies carried out on the day of admission demonstrated the elevation of serum levels of both tumor markers, carbohydrate 19-9 (CA19-9) and carcinoembryonic antigen (CEA). The estimated serum concentrations of CA19-9 and CEA were 39.4 U/ml and 81.1 ng/ml, respectively. The cerebrospinal fluid (CSF) contained 9 cells/mm3 which consisted of the mixture of malignant cells with appearance strongly suggestive of poorly differentiated adenocarcinoma and of hemosiderinphagocytizing macrophages. Detection of both CA19-9 and CEA in the tumor cells in the CSF was undertaken by the avidin biotinylated peroxidase complex method by use of monoclonal antibodies against each marker. As the results the following findings were obtained. Namely, although all the tumor cells isolated from the CSF were found to contain both tumor markers, CA19-9 and CEA, there was found a difference in the concentration ratio in CSF vs serum between the markers. The estimated concentration of CA19-9 (61.4 U/ml) in CSF was approximately 1.6 times higher than that in serum. On the other hand, the CSF concentration of CEA remained constantly very low (1.1 ng/ml) as compared with that in the serum throughout the whole course of the disease. In response to intrathecal administration of cytarabine and brain irradiation, the CSF cytological findings became normal and concomitant decrease in the concentration of CA19-9 was demonstrated.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of leptomeningeal carcinomatosis: demonstration of CA19-9 and CEA positive malignant cells in the CSF and particular elevation of CA19-9 level in the CSF]. 206 Feb 42

Five children with subacute or acute onset of cerebellar ataxia and opsoclonus are described. Two had cerebrospinal fluid pleocytosis at the onset of ataxia and were initially thought to have acute parainfectious cerebellar ataxia of childhood. All were found to have tumors of neural crest origin (two neuroblastomas, three ganglioneuroblastomas). Tumors were small and only found by computed tomographic techniques. Urinary catecholamine metabolites were elevated in only two of the patients. Four of the five failed to improve neurologically with resection of the tumor. All four have had a steroid-sensitive chronic ataxic syndrome that worsens with acute nonspecific illnesses and has resulted in long-term deficits, particularly in speech and gross motor function. This is a metabolic encephalopathy associated with permanent residual neurologic deficits but without visible lesions on neuroimaging studies. We stress the frequency of cerebrospinal fluid pleocytosis in patients with tumor-associated opsoclonus and the clinical difficulty in separating tumor-associated cases from those due to other causes [corrected].
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PMID:Opsoclonus-ataxia due to childhood neural crest tumors: a chronic neurologic syndrome. 234 82

Ataxia-telangiectasia, an inherited disorder characterized by progressive cerebellar ataxia and telangiectasias, is often associated with primary immunodeficiency and high incidence of malignancies, mostly of the lymphoreticular type. Endodermal sinus tumor is a rare germ cell tumor of the ovary characterized by an extremely rapid growth and poor prognosis. Both these diseases are associated with an abnormal production of alpha-fetoprotein. Primary tumors of the ovary in patients with ataxia-telangiectasia are extremely rare and the association of an endodermal sinus tumor and ataxia-telangiectasia has never been reported in the literature. This case report serves to focus on the particular problems encountered in the diagnosis and management of two diseases both characterized by the same serum marker.
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PMID:Ataxia-telangiectasia and endodermal sinus tumor of the ovary: report of a case. 244 92

Neurological disorders associated with a malignant neoplasm, which is not caused by a direct effect such as metastasis, infiltration or compression, is called carcinomatous neuromyopathy. Subacute cerebellar degeneration recognized in this category is characterized by acutely or subacutely progressive cerebellar ataxia and widespread loss of Purkinje cells. There have been several reports of subacute cerebellar degeneration in lung carcinoma, ovarian carcinoma and Hodgkin's disease, but rare in urogenital malignancies. We present a patient with neurological disorder considered subacute cerebellar degeneration associated with HCG-beta positive seminoma. A 29-year-old man noticed a left intrascrotal mass in the summer of 1984. The mass began to grow in April, 1985 and diplopia, gait disturbance and dysarthria appeared late in May. He consulted our hospital on July 20, 1985. Serum human chorionic gonadotropin (HCG)-beta was elevated to 200 ng/ml but alpha-fetoprotein and carcinoembryonic antigen were normal. Left high orchiectomy was performed and the tumor was diagnosed histologically as typical seminoma. Bulky metastatic tumor was recognized in retroperitoneum on abdominal CT but brain CT was normal. VAB VI chemotherapy was performed. The retroperitoneal metastatic tumor disappeared and HCG-beta was normalized and complete remission achieved, but cerebellar symptoms still remain 14 months after remission. This case is considered to be subacute cerebellar degeneration associated with seminoma and is the second case with testicular carcinoma reported.
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PMID:[Subacute cerebellar degeneration with HCG-beta positive seminoma of the testis]. 245 60


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