1/708. Complete paraplegia due to multiple intracerebral and spinal cavernomas.We report on a 29-year-old male patient with multiple intracerebral and spinal cavernomas. Bleeding in the thoracic cord at admission and additional bleeding which occurred 12 days later in the cervical cord resulted in complete paraplegia below thoracic level 4 (Th4). Four years earlier multiple cerebral cavernomas had been diagnosed by magnetic resonance imaging (MRI). Based upon reported cases in the literature multiple intracerebral and spinal cavernomas are exceptional. Additionally, the clinical presentation in our case is uncommon.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/708. Successfully treated central nervous system Burkitt's lymphoma with minimal extraneural disease in a child.Burkitt's lymphoma, the most common childhood non-Hodgkin's lymphoma, usually presents with abdominal tumors. central nervous system (CNS) involvement in Burkitt's lymphoma is mostly secondary to advanced systemic disease, rarely with brain parenchymal lesions, and was previously recalcitrant to treatment. We report an unusual case of CNS Burkitt's lymphoma with minimal extraneural disease. This 10-year-old immunocompetent boy presented with increased intracranial pressure and seizures and was found to have multifocal intracerebral lesions on brain imaging studies. cerebrospinal fluid studies confirmed the presence of Burkitt's lymphoma cells. Abdominal computed tomography showed bilateral nephromegaly with left intrarenal lesions that disappeared after three doses of intravenous dexamethasone. The patient was treated for 6.5 months according to the LMB 89 group C protocol of the French Pediatric Oncology Society. The response was brisk and complete. The patient has been disease free for more than 4 years, and is believed to be cured.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
3/708. Early glial tumor metastases through a ventriculo-atrial shunt.A girl of 12 with a pontine polymorphous astrocytoma diffusely metastasizing to the spinal cord leptomeninges suddenly died 20 hours after the insertion of a right ventriculo-atrial shunt. Many severely damaged glial neoplastic cells were found in the blood vessels of the lung and liver.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
4/708. dementia following treatment of brain tumors with radiotherapy administered alone or in combination with nitrosourea-based chemotherapy: a clinical and pathological study.A retrospective clinical and pathological study of 4 patients who developed the syndrome of radiation induced dementia was performed. All patients fulfilled the following criteria: (1) a history of supratentorial irradiation; (2) no evidence of symptomatic recurrent tumor; (3) no other cause of progressive cerebral dysfunction and dementia. The clinical picture consisted of a progressive "subcortical" dementia occurring 3-12 months after a course of cerebral radiotherapy. Examination revealed early bilateral corticospinal tract involvement in all patients and dopa-resistant Parkinsonian syndrome in two. On CT scan and MRI of the brain, the main features consisted of progressive enlargement of the ventricles associated with a diffuse hypodensity/hyperintensity of the white matter best seen on T2 weighted images on MRI. The course was progressive over 8-48 months in 3 patients while one patient had stabilization of his condition for about 28 years. Treatment with corticosteroids or shunting did not produce sustained improvement and all patients eventually died. Pathological examination revealed diffuse white matter pallor with sparing of the arcuate fibers in all patients. Despite a common pattern on gross examination, microscopic studies revealed a variety of lesions that took two basic forms: (1) a diffuse axonal and myelin loss in the white matter associated with tissue necrosis, particularly multiple small foci of necrosis disseminated in the white matter which appeared different from the usual "radionecrosis"; (2) diffuse spongiosis of the white matter characterized by the presence of vacuoles that displaced the normally-stained myelin sheets and axons. Despite a rather stereotyped clinical and radiological course, the pathological substratum of radiation-induced dementia is not uniform. Whether the different types of white matter lesions represent the spectrum of a single pathological process or indicate that the pathogenesis of this syndrome is multifactorial with different target cells, remains to be seen.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
5/708. movement disorders following nonfunctional neurosurgery.OBJECT: knowledge is scarce about movement disorders that follow neurosurgical operations other than functional stereotactic surgery. The cases of 14 patients who suffered from movement disorders secondary to craniocerebral or spinal surgery are analyzed. None of these patients was initially treated by any of the authors. methods: Twelve patients underwent surgery for cerebral diseases. Nine of these patients harbored tumors and three patients had neurovascular disorders. Two patients underwent spinal surgery for cervicothoracic ependymoma or for multiple cervical disc herniations. Twelve of the 14 patients had immediate postoperative side effects such as hemiparesis, ataxia, and somnolence. In all but two patients, movement disorders became manifest only after a delay. Dystonic movement disorders developed in eight patients, unilateral tremors in three patients, unilateral facial myokymia in one patient, and hemichorea-hemiballism in two patients. The mean delay of onset for tremor was 5 weeks and that for dystonic movement disorders was 5.5 months. movement disorders were transient in three patients; however, they were persistent in 11 patients at a mean follow-up period of 5 years. These movement disorders caused marked persistent disability in four patients. Lesions of the contralateral striatum were identified in patients with dystonic syndromes and lesions of the dentatothalamic outflow in patients with tremors. In three patients who had postoperative basal ganglia lesions after partial removal of astrocytomas, tumor regrowth was later documented. Medical treatment in patients with persistent movement disorders rendered only limited benefit. Two patients improved with botulin injections. In one patient postoperative hemidystonia was alleviated by contralateral thalamotomy. CONCLUSIONS: Dystonic syndromes and tremors are the most common movement disorders that occur after craniocerebral and spinal surgery. Postoperative movement disorders can lead to various degrees of functional disability. The pathoanatomical correlations are similar to those described in other patients with secondary movement disorders.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
6/708. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases.A series of 35 primary cerebral neuroblastoma is reported. These rare tumours occur most often in children in the first half of the first decade. Grossly the tumors are often massive, discrete, lobular, firm and cystic. Histologically three variants, largely determined by the extent and distribution of the fibrous connective tissue stroma, are recognized: (1) a classical variant, which most resembles the peripheral neuroblastoma and is characterized by a high frequency of Homer Wright rosettes and a relatively high frequency of ganglionic differentiation; (2) a desmoplastic variant, which is characterized by an intense connective tissue stroma; and (3) a transitional variant, in which both the classical and the desmoplastic features may be present within the same case, either concurrently or consecutively. Both the desmoplastic and the transitional forms are less likely to exhibit differentiation to mature ganglion cells, but the importance of identifying the primitive cell elements as neuroblasts is emphasized. With rare exceptions, this can be established only by specific silver impregnations on frozen material. Occasionally the direction of growth may be largely leptomeningeal. Seven illustrative clinical histories with pathological correlations are described. The over-all clinical behaviour of these tumours is that of malignant neuroepithelial neoplasms, characterized by a high recurrence rate. recurrence may, however, be a late development, in some cases occurring five or seven years after apparently successful surgical removal. The tumour shows shows a high incidence of metastatic spread, almost 40 per cent of the cases examined at autopsy having disseminated in the cerebrospinal pathways. Exceptionally, extraneural metastases may also develop. However, long post-operative survival occasionally occurs, and the subsequent clinical course is not always predictable in the individual case. The differential diagnosis is briefly discussed. The cellular nature of the tumour and its biological behaviour recall those of the cerebellar medulloblastoma. Post-operative radiation to the entire neuraxis should be considered for these neoplasms.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
7/708. Neurofibromatosis type 2 with multiple primary brain tumors in monozygotic twins.BACKGROUND: Although monozygotic twins with neurofibromatosis complicated by brain tumors rarely have been reported, none of them fulfilled the diagnostic criteria for neurofibromatosis type 2 (NF2). METHOD: We describe here the first pair of monozygotic twins with NF2, and the result of the molecular analysis of their NF2 gene. RESULTS: One of the brothers (Case 1) developed tetraparesis and cerebellar truncal ataxia at age 12. He had no skin lesions. Radiological examinations revealed, at one time or another, bilateral vestibular schwannomas, a foramen magnum meningioma, five supratentorial meningiomas, and multiple spinal cord tumors. He underwent three operations over a 10-year period to remove tumors. The patient is now 23 years old and is in college. Although asymptomatic when examined at age 12, CT scan revealed that his brother (Case 2) also had multiple brain tumors, including meningiomas, schwannomas, and multiple spinal tumors. Tumors were removed in eight operations over a 10-year period. The patient is now deaf and confined to a wheelchair. An identical nonsense mutation caused by a C to T transition (C169) in a CpG dinucleotide of the NF2 gene was identified in both patients. CONCLUSION: These results led us to speculate that dissimilarities with respect to time of appearance, distribution, and extent of symptoms and tumors between the twins were dependent on the influence of other genetic factors.- - - - - - - - - - ranking = 0.33333333333333keywords = spinal (Clic here for more details about this article) |
8/708. Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report.Extracranial metastasis of an intracranial anaplastic ganglioglioma through a ventriculoperitoneal shunt is reported. A 53-year-old woman was treated by surgery, radiation and chemotherapy and died 2 years later. At autopsy, multiple metastatic lesions were found in the spinal cord, and the abdominal and pleural cavities. Histologically, all the metastatic lesions were composed of atypical cells which resembled primitive glial elements found in intracranial anaplastic ganglioglioma, suggesting that anaplastic glial elements have a metastatic potential. Extracranial metastasis of ganglioglioma is a rare occurrence; however, the spread of glial elements through the shunt further suggests that caution is required in therapy and indicates a need for protective filters in the shunt system.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
9/708. Treatment of AIDS-related primary central nervous system lymphoma with zidovudine, ganciclovir, and interleukin 2.AIDS-related primary central nervous system lymphoma (AIDS PCNSL) is a rapidly fatal disease. Conventional therapeutic modalities offer little and new approaches are needed. Previous work has shown that zidovudine (AZT) in combination with other agents is active in retroviral lymphomas. Epstein-Barr virus (EBV) is detected in tumor tissue and cerebrospinal fluid of AIDS PCNSL patients. In a preliminary in vitro study we found that an Epstein-Barr virus-positive B cell line underwent apoptosis on coculture with AZT. This effect was accentuated by the addition of ganciclovir (GCV). We treated five patients with AIDS PCNSL with a regimen consisting of parenteral zidovudine (1.6 g twice daily), ganciclovir (5 mg/kg twice daily), and interleukin 2 (2 million units twice daily). Four of five had an excellent response. Two patients are alive and free of disease 22 and 13 months later; another responded on two separate occasions, 5 months apart, and the last patient responded with a 70-80% regression of tumor but could not be maintained on therapy owing to myelosuppression. We conclude that parenteral zidovudine, ganciclovir, and interleukin 2 is an active combination for AIDS-related central nervous system lymphoma.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
10/708. Diffuse neonatal hemangiomatosis with extensive involvement of the brain and cervical spinal cord.BACKGROUND: Diffuse neonatal hemangiomatosis (DNH) is a rare disorder first recognized at birth or during the neonatal period. DNH is characterized by numerous cutaneous and visceral hemangiomas involving three or more organ systems. MATERIALS AND methods: Although the skin and liver are most frequently affected, we present a case of DNH demonstrating an unusual predilection for the central nervous system (CNS). RESULTS AND CONCLUSION: We report the imaging findings in a patient with this disorder, paying particular attention to the features seen on cranial sonography and spinal MR imaging.- - - - - - - - - - ranking = 0.83333333333333keywords = spinal (Clic here for more details about this article) |
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