Encephalomyelitis due to Cryptococcus neoformans var gattii presenting as spinal tumour: case report and review of the literature.
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A 24 year old immunocompetent German resident is described who developed multifocal encephalomyelitis due to infection with Cryptococcus neoformans var gatti, commonly considered a disease of tropical regions. In the light of current knowledge on the epidemiology of C neoformans var gatti and the travel history of the patient it is assumed that the infection was acquired outside Europe. As exclusive intramedullary involvement is an outstandingly rare manifestation in spinal cryptococcosis, the particular diagnostic procedure and the therapeutic strategies are discussed (+info)
Low grade astrocytomas in the West of Scotland 1987-96: treatment, outcome, and cognitive functioning.
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BACKGROUND: Low grade astrocytomas are among the most common central nervous system tumours in children. AIMS: To identify risk factors for the development of persistent intellectual handicap. METHODS: The notes of 30 children with histologically proven low grade astrocytoma who presented during the period 1987-96 were reviewed. Thirteen of these children who were diagnosed with intracranial tumours between 1992 and 1996 underwent cognitive assessment one year after the completion of treatment. RESULTS: Low grade astrocytomas were found in the cerebellum (59%), thalamus (17%), cerebral hemispheres (10%), and the cervical spinal cord (9%). Where possible all patients were treated with gross total resection of the tumour. Symptomatic children with tumours judged to be inoperable underwent biopsy followed by radiotherapy (13%). Three patients developed progressive disease following surgery and underwent repeat surgery and radiotherapy. Survival at a median follow up of 75 months (range 30-131) is 97%. At one year after the completion of treatment persisting cognitive impairment was common. The strongest predictor of IQ scores was the duration of symptoms of increased intracranial pressure preoperatively. CONCLUSIONS: Although the overall survival rate of children with low grade astrocytoma is excellent, significant long term disability occurs. Early diagnosis is essential to reduce postoperative cognitive morbidity. (+info)
Neuroblastoma with symptomatic spinal cord compression at diagnosis: treatment and results with 76 cases.
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PURPOSE: To report on the treatment of patients with newly diagnosed neuroblastoma presenting with spinal cord compression (SCC). PATIENTS AND METHODS: Of 1,462 children with neuroblastoma registered between 1979 and 1998, 76 (5.2%) presented with signs/symptoms of SCC, including motor deficit in 75 patients (mild in 43, moderate in 22, severe [ie, paraplegia] in 10), pain in 47, sphincteric deficit in 30, and sensory loss in 11. Treatment of SCC consisted of radiotherapy in 11 patients, laminectomy in 32, and chemotherapy in 33. Laminectomy was more frequently performed in cases with favorable disease stages and in those with severe motor deficit, whereas chemotherapy was preferred in patients with advanced disease. RESULTS: Thirty-three patients achieved full neurologic recovery, 14 improved, 22 remained stable, and eight worsened, including three who become paraplegic. None of the 10 patients with grade 3 motor deficit, eight of whom were treated by laminectomy, recovered or improved. In the other 66 patients, the neurologic response to treatment was comparable for the three therapeutic modalities. All 11 patients treated by radiotherapy and 26 of 32 patients treated by laminectomy, but only two of 33 treated by chemotherapy, received additional therapy for SCC. Fifty-four of 76 patients are alive at time of the analysis, with follow-up of 4 to 209 months (median, 139 months). Twenty-six (44%) of 54 survivors have late sequelae, mainly scoliosis and sphincteric deficit. CONCLUSION: Radiotherapy, laminectomy, and chemotherapy showed comparable ability to relieve or improve SCC. However, patients treated with chemotherapy usually did not require additional therapy, whereas patients treated either with radiotherapy or laminectomy commonly did. No patient presenting with (or developing) severe motor deficit recovered or improved. Sequelae were documented in 44% of surviving patients. (+info)
MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings.
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BACKGROUND AND PURPOSE: To our knowledge, a detailed analysis of MR findings in spinal hemangioblastoma has not been conducted to date. Our purpose was to elucidate the MR features of this disease with special attention to tumor size, correlation with MR findings and clinical symptoms, and any differences between patients with and without von Hippel-Lindau disease (VHLD). METHODS: MR images in five patients with VHLD and seven patients without VHLD were reviewed retrospectively for spinal hemangioblastoma by two neuroradiologists. The MR findings were correlated with clinical symptoms and with angiographic and surgical findings. RESULTS: The MR features depended on the size of the spinal hemangioblastoma. Small (10 mm or less) hemangioblastomas were mostly isointense on T1-weighted images, hyperintense on T2-weighted images, and showed homogeneous enhancement. Larger hemangioblastomas tended to be hypointense or mixed hypo- and isointense on T1-weighted images, heterogeneous on T2-weighted images, and tended to show heterogeneous enhancement. Small hemangioblastomas were located at the surface of the spinal cord, most frequently along its posterior aspect. These were subpial in location at surgery and showed well-demarcated, intense enhancement. Symptomatic small hemangioblastomas had relatively large associated syringes, whereas asymptomatic ones did not. A hemangioblastoma larger than 24 mm was invariably accompanied by vascular flow voids. There was no difference in the MR findings between the two patient groups except for the multiplicity and higher percentage of small tumors in patients with VHLD. CONCLUSION: Knowledge of these MR features helps to differentiate spinal hemangioblastoma from other diseases that show enhancing nodules. (+info)
Intra-axial tumors of the medullocervical junction: diagnosis and microsurgical treatment.
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OBJECTIVE: To describe the clinical features, operative methods and postoperative management of the intra-axial tumors of medullocervical junction, and to make differential diagnosis for different subtypes. METHODS: Fifteen patients with intra-axial tumors of medullocervical junction were treated from August 1988 to June 1997. The diagnoses were confirmed by MRI and histological examinations. The tumors were divided into two subtypes according to the clinical features and the main body of the tumor. The distinctive points of the two subtypes and the appropriate surgical methods of different pathological type tumors were expounded. RESULTS: Tumors were totally removed in 10 patients and subtotally in 5. There was no death caused by operation. Postoperative complications included respiratory disturbance in six cases, upper digestive tract bleeding in one, depressed cough reflex in two, most of which recovered after proper treatment. On discharge, the nervous system status was improved in 11 cases, stable in 2 and worsened in 2. CONCLUSIONS: The intra-axial tumors of medullocervical junction can be divided into cervicomedullary and medullocervical subtypes. The MRI examination is decisive in the distinction of the diagnosis, and is important in the determination of the nature of the medullar cystic lesions and the guidance of the resection of tumor extent. The cervicomedullary tumors are more amenable to an aggressive surgical treatment, during which the surgeon should remove the tumors first in the cervical spinal cord area, then in the medullar area with the tumor resection expanding rostrally. It can make the operation safer to remove the tumors using appropriate techniques varied with pathological types of the tumors. Managing postoperative respiratory disturbances without delay is one of the important points in improving the therapeutic effect. (+info)
Radicular involvement and medullary invasion from a malignant mesothelioma.
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We present the case of a 57-year-old patient who had worked at a fiber-cement factory for 28 years. The patient developed an epithelioid-type pleural mesothelioma 5 years after retiring, after he was diagnosed with asbestosis. Only 5 months after the diagnosis of mesothelioma, a medullar section appeared to be totally invaded by a tumor in the medullar canal, thus causing paraplegia and affecting the bladder and anal sphincters. The patient underwent radiotherapy and chemotherapy, and achieved partial recovery, but died 9 months after the diagnosis. (+info)
Chromosomal abnormalities subdivide ependymal tumors into clinically relevant groups.
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Ependymoma occurs most frequently within the central nervous system of children and young adults. We determined relative chromosomal copy-number aberrations in 44 ependymomas using comparative genomic hybridization. The study included 24 intracranial and 20 spinal cord tumors from pediatric and adult patients. Frequent chromosomal aberrations in intracranial tumors were gain of 1q and losses on 6q, 9, and 13. Gain of 1q and loss on 9 were preferentially associated with histological grade 3 tumors. On the other hand, gain on chromosome 7 was recognized almost exclusively in spinal cord tumors, and was associated with various other chromosomal aberrations including frequent loss of 22q. We conclude that cytogenetic analysis of ependymomas may help to classify these tumors and provide leads concerning their initiation and progression. The relationship of these aberrations to patient outcome needs to be addressed. (+info)
Diffuse leptomeningeal malignant histiocytosis in the brain and spinal cord of a Tibetan Terrier.
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An 8-year-old male Tibetan Terrier showed prolonged astasia, complete paralysis, ticlike signs, and seizure and died 2 months after the onset of symptoms. Histopathologically, there was moderate to severe infiltration of pleomorphic histiocytic mononuclear cells bilaterally in the basiarachnoidal and ventricular areas of the brain. The spinal dura mater, arachnoidal space, and leptomeninges were also affected by infiltrative proliferation of these mononuclear cells. The infiltrating cells had the morphologic characteristics of histiocytes but exhibited moderate pleomorphism and atypia, with abundant mitotic figures. With immunohistochemistry and lectin histochemistry, most of the infiltrating cells were positive for lysozyme and lectin RCA-1 and negative for glial fibrillary acid protein, suggesting that they were of monocytic/histiocytic-origin. Positive proliferating cell nuclear antigen immunostaining demonstrated that most nuclei of the histiocytic cells were in the S phase of the cell cycle, consistent with a proliferating population of cells. Based on these findings, the case was diagnosed as diffuse leptomeningeal malignant histiocytosis. (+info)