SUMMARY: Spinal intradural extramedullary capillary hemangiomas are extremely rare. We present the MR imaging and histologic findings in three patients with this abnormality. The three patients were men who had symptoms of either myelopathy (n = 2) or radiculopathy (n = 1). The tumors were well demarcated, 1.5-2.0 cm in diameter, and were located at the posterior or posterolateral portion of the thecal sac (one at the L1 level and the other two at the midthoracic level). On MR images, the tumor showed isointensity relative to the spinal cord on T1-weighted images, hyperintensity on T2-weighted images, and strong homogeneous enhancement on contrast-enhanced T1-weighted images in all three patients. In two patients, the dural tail sign was observed. Capillary hemangioma should be included in the differential diagnosis of a spinal intradural extramedullary tumor. (+info)
A rare case of intramedullary lipoma associated with cyst.
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Intramedullary lipomas are benign tumours of the spinal cord corresponding to 1% of all primitive intramedullary tumours. We report a rare case of "true" intramedullary lipoma associated with cyst. The patient underwent subtotal resection and the diagnosis was made by histopathological examination. There was postoperative neurological improvement. (+info)
Extramedullary astrocytoma of conus region : a short report.
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A 55 year old man presented with features of cauda equina syndrome. Magnetic resonance imaging (MRI) showed a well demarcated intradural extramedullary tumour at L2 vertebra. At surgery it was found to be well encapsulated and had no attachment to spinal cord or root. Histopathology including immunohistochemistry confirmed it to be a low grade astrocytoma. (+info)
Childhood organic neurological disease presenting as psychiatric disorder.
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Over a period of one year 12 children with complaints which had been diagnosed as due to a psychiatric disorder presented to a paediatric neurological unit where neurological disease was diagnosed. The group was characterized by behavioural symptoms such as deteriorating school performance, visual loss, and postural disturbance, which are unusual in children attending child psychiatric departments. It is suggested that where there is diagnostic uncertainty the presence of these physical symptoms calls for periodic neurological reassessment, and attention is drawn to the rare but serious disorders which may thus be diagnosed. Making an organic diagnosis, however, should not preclude psychosocial management of emotional reactions in these families. (+info)
Metastasizing neuroblastomas from taste buds in rats transgenic for the Simian virus 40 large T antigen under control of the probasin gene promoter.
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During establishment of a prostate cancer model in rats transgenic for the Simian virus 40 large T antigen, under control of the probasin gene promoter, with protein expression specific to the prostate, tongue, and spinal cord, undifferentiated small round cell tumors were frequently observed. Extensive examination of tongues of the transgenic rats, despite a macroscopically normal appearance, revealed the tumors to have come from taste buds of the papilla circumvallata and papilla foliata. The lesions were positive for the SV40 T antigen, PGP9.5 (ubiquitin C-terminal hydrolase), and synaptophysin, neuron and neuroendocrine markers. Morphologically and immunohistochemically, the tumors were diagnosed as neuroblastomas, considering the neuroepithelial origin. Histologically identical tumor cells in the spinal cord and lung were observed only in the rats with deeply invading tongue tumors, suggesting that metastasis from the tongue tumors had occurred. Castration or supplementation with testosterone propionate did not alter tumor development, indicating the tumors to be androgen-independent. These results clearly show that taste buds can give rise to metastasizing neuroblastomas. (+info)
Germline SDHD mutation in paraganglioma of the spinal cord.
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Hereditary paraganglioma of the head and neck is associated with germline mutations in the SDHD gene, which encodes a mitochondrial respiratory chain protein. Paragangliomas of the central nervous system are very rare, occur almost exclusively in the cauda equina of the spinal cord and are considered non-familial. In the present study, we screened 22 apparently sporadic paragangliomas of the cauda equina for SDHD mutations. One spinal paraganglioma and similar cerebellar tumours that developed 22 years later in the same patient contained a missense mutation at codon 12 (GGT-->AGT, Gly-->Ser) and a silent mutation at codon 68 (AGC-->AGT, Ser-->Ser). There was no family history of paragangliomas but DNA from white blood cells of this patient showed the same sequence alterations, indicating the presence of a germline mutation. All other cases of spinal paraganglioma had the wild-type SDHD sequence, except one case with a silent mutation at codon 68 (AGC-->AGT, Ser-->Ser). This is the first observation indicating that inherited SDHD mutations may occasionally cause the development of paragangliomas in the central nervous system. (+info)
Recognizing spinal cord emergencies.
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Physicians who work in primary care settings and emergency departments frequently evaluate patients with neck and back pain. Spinal cord emergencies are uncommon, but injury must be recognized early so that the diagnosis can be quickly confirmed and treatment can be instituted to possibly prevent permanent loss of function. The differential diagnosis includes spinal cord compression secondary to vertebral fracture or space-occupying lesion, spinal infection or abscess, vascular or hematologic damage, severe disc herniation and spinal stenosis. The most important information in the assessment of a possible spinal cord emergency comes from the history and the clinical evaluation. Physicians must look for "red flags"--key historical and clinical clues that increase the likelihood of a serious underlying disorder. In considering diagnostic tests, physicians should apply the principles outlined in an algorithm for the evaluation of low back pain prepared by the Agency for Healthcare Research and Quality (formerly the Agency for Health Care Policy and Research). Computed tomography and magnetic resonance imaging can clearly define anatomy, but these studies are costly and have a high false-positive rate. Referral of high-risk patients to a neurologist or spine specialist may be indicated. (+info)
Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990-1994.
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The Central Brain Tumor Registry of the United States (CBTRUS) obtained 5 years of incidence data (1990-1994)--including reports on all primary brain and CNS tumors--from 11 collaborating state cancer registries. Data were available for 20,765 tumors located in the brain, meninges, and other CNS sites, including the pituitary and pineal glands. The average annual incidence was estimated at 11.5 cases per 100,000 person-years. The higher incidence of tumors in male patients (12.1 per 100,000 person-years) than in female patients (11.0 per 100,000 person-years) was statistically significant (P < 0.05); the higher incidence in whites (11.6 per 100,000 person-years) compared with blacks (7.8 per 100,000 person-years) was statistically significant (P < 0.05). The most frequently reported histologies were meningiomas (24.0%) and glioblastomas (22.6%). Higher rates for glioblastomas, anaplastic astrocytomas, oligodendrogliomas, anaplastic oligodendrogliomas, ependymomas, mixed gliomas, astrocytomas not otherwise specified, medulloblastomas, lymphomas, and germ cell tumors in male than in female patients were statistically significant (P < 0.05), with relative risks (RR) ranging from 1.3 to 3.4. Meningiomas were the only tumors with a significant excess in females (RR = 0.5). We noted higher occurrence rates in whites than in blacks for the following histologies: diffuse astrocytomas, anaplastic astrocytomas, glioblastomas, oligodendrogliomas, ependymomas, mixed gliomas, astrocytomas NOS, medulloblastomas, nerve sheath tumors, hemangioblastomas, and germ cell tumors, with RRs ranging from 1.5 to 3.4. Racial differences in occurrence rates were not observed for predominately benign meningiomas or pituitary tumors. This study represents the largest compilation of data on primary brain and CNS tumors in the United States. Standard reporting definitions and practices must be universally adopted to improve the quality and use of cancer registry data. (+info)