1/86. Cervical spine abnormalities in down syndrome.down syndrome (DS) is the most common of the chromosomal disorders and manifests abnormalities in several organ systems. While mental retardation, skull and brain anomalies, and the development of Alzheimer-type neuropathological changes in patients greater than age 40 years are well recognized by neurologists and neuropathologists, less appreciated are the various cervical spine abnormalities that can occur. Widening of the anterior atlanto-odontoid distance (AAOD) and atlantooccipital instability occur in up to 21% and 63% of DS patients, respectively, but neurologic complaints are uncommon and rarely are severe enough to contribute to the patient's demise. We present a case of 49-year-old DS patient whose triplegia, subacute progressive respiratory failure, and death could be attributed to severe degenerative joint disease of the cervical spine with osteophyte formation and severe spinal canal stenosis. We provide the first detailed correlation study between pre-mortem magnetic resonance imaging (MRI) and extensive autoptic dissection in an adult DS patient with cervical spine abnormalities, as well as a review of the literature.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/86. Surgical decompression and radiation therapy in epidural metastasis from cervical cancer.spinal cord compression by epidural metastasis is considered an exceptional complication in patients with cervical carcinoma. We report three patients treated for a cervical carcinoma who developed epidural metastasis with spinal cord compression at 9, 25 and 48 months after primary treatment of the uterine malignancy. All patients had poorly-differentiated adenocarcinomas with lymphovascular space invasion, and two had lymph node metastasis. All patients underwent emergency decompressive laminectomy followed by radiotherapy and a partial recovery of the neurological function was achieved. In two patients the spinal cord was the only site of recurrent disease, whereas the other had lung and brain metastasis at the time of epidural involvement diagnosis. All three patients, however, died of disseminated disease. Surgical decompression followed by radiation therapy may result in a complete preservation of the neurologic functions in patients with spinal cord compression secondary to metastatic carcinoma of the uterine cervix. Considering the propensity for disseminated disease, long term survival might be achieved only with the use of effective chemotherapy.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/86. Simultaneous development of a pineal tumor and an intradural spinal mass during remission of acute lymphocytic leukemia.A small percentage of children with acute lymphocytic leukemia experience relapse in the central nervous system in spite of prophylaxis. Diffuse leptomeningeal infiltration is common but an intracranial leukemic mass or spinal cord involvement is a rare manifestation. We report a child with acute lymphocytic leukemia who simultaneously developed a pineal tumor and an intradural spinal cord mass as her first relapse. She was successfully managed by comprehensive combined treatment including peripheral blood stem cell transplantation. She remains in continuous complete remission for more than 5 years without further evidence of neurological sequelae.- - - - - - - - - - ranking = 61.228487457432keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/86. Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review.Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome, characterised by large or numerous congenital pigmented nevi and excessive proliferation of melanin-containing cells in the leptomeninges. We report the MR findings in the brain and spine of a child with NCM who underwent neurosurgical treatment and was followed up for 8 years. The findings in this child (small hyperintense collections of melanocytes in both temporal lobes, mild meningeal enhancement along the spine and the development of an extensive subarachnoid CSF accumulation with cord compression and syringomyelia) are believed to be exceptionally rare.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/86. Malignant spread of haemangioblastoma: report on two cases.Two cases are described in which, after successful removal of a cerebellar haemangioblastoma followed by several years of freedom from symptoms, there developed a progressive spinal cord compression, leading to death. At necropsy the spinal cords in both cases and the brainstem in one case, were irregularly plastered with haemangioblastoma. Although there was no doubt that malignant spread had occurred from one or more primary tumours, the histology of the tumour tissue was in no way different from that of conventional haemangioblastoma.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/86. Primary intradural extramedullary ependymoma: case report and review of the literature.STUDY DESIGN: The authors report the ninth case in the literature of a primary intradural extramedullary ependymoma of the spinal cord. OBJECTIVE: To discuss surgical treatment and the physiopathologic hypothesis of this localization on the basis of the results of the present study and a review of the literature. SUMMARY OF BACKGROUND DATA: ependymoma is a glial tumor known to arise in the central nervous system. Intradural extramedullary location of this neoplasm has been exceptionally described previously. methods: A 43-year-old woman was admitted to the authors' institution with an history of progressive paraplegia. neurologic examination showed sensory loss below T1 and bladder disturbances. magnetic resonance imaging revealed an enhanced thoracic intradural extramedullary tumor, extending from T1-T8. No other lesion in the central nervous system was found. Emergency surgical resection was performed. RESULTS: Surgery gave confirmation of an encapsulated extramedullary tumor without attachment to the spinal cord or to the dura mater. Total removal was achieved under microscope. The postoperative course was uneventful, with complete neurologic recovery 3 months later. The patient has been well for 24 months of follow-up evaluation, without evidence of recurrence on magnetic resonance images. Histologic examination revealed the tumor as a benign ependymoma. CONCLUSION: The encapsulated feature, the lack of attachment to the central nervous system, and the absence of other neoplastic processes within the brain or the spinal cord suggested that this lesion is a primary tumor developed from ectopic ependymal cells.- - - - - - - - - - ranking = 184.68546237229keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
7/86. Extradural spinal cavernous haemangioma: case report and review of the literature.Cavernous haemangiomas (cavernomas) are uncommon vascular malformations of the central nervous system (CNS). They occur in both sporadic and familial forms and may involve any site in the CNS. Spinal cavernomas are less common than intracerebral lesion s, and examples in the spinal epidural space are rare. A case of a solitary sporadic spinal extradural cavernoma in a 41 year old male which presented as progressive lower limb numbness and weakness is reported. The literature regarding spinal cavernomas is reviewed and the symptomatology, diagnostic evaluation, pathology, management and prognosis of these lesions are discussed.- - - - - - - - - - ranking = 61.228487457432keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/86. Epidermal naevus and segmental hypermelanosis associated with an intraspinal mass: overlap between different mosaic neuroectodermal syndromes.The epidermal naevus syndrome is a neurocutaneous syndrome characterised by the association of epidermal naevi with central nervous system, skeletal, ocular or cardiovascular abnormalities. We report on a 1.75-year-old boy who presented with spastic diparesis, a partial paresis of the left forearm and macrocephaly. He had a large epidermal naevus along the cervical spine and a segmental hypermelanosis. MRI studies revealed a large intraspinal mass extending from the lower cervical to the upper thoracic spine. The condition of our patient demonstrates the overlap of the epidermal naevus syndrome with well-defined mosaic neuroectodermal phenotypes such as encephalocraniocutaneous lipomatosis, Feuerstein-Mims syndrome and proteus syndrome. Conclusion: we recommend evaluation of all patients with large epidermal naevi, especially in the head and neck region, for the presence of central nervous system abnormalities or neoplasms.- - - - - - - - - - ranking = 122.45697491486keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/86. Metastatic carcinoid presenting as a spinal tumor.A case of carcinoid tumor metastatic to the thoracic spine with associated myelopathy is described. Multiple posterior explorations were singularly unsuccessful in locating and indentifying the metastasis, but an anterior transthoracic exposure allowed identification of the tumor, removal of the osteoblastic epidural mass, and fusion of the spine with a rib graft. The patient's myelopathy improved steadily following surgery without evidence of tumor recurrence or regression of neurologic status. The reasons for the relative absence of central nervous system carcinoid metastases are not known.- - - - - - - - - - ranking = 61.228487457432keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/86. Cervicomedullary junction compression caused by vertebral artery dolichoectasia and requiring surgical treatment. Case report.A case of progressive brainstem syndrome secondary to vertebral artery (VA) dolichoectasia is reported. The patient presented with partial bilateral abduction paralysis, which progressed to quadriparesis, ataxia, and areflexia. The initial diagnosis was stroke, but because of the patient's deterioration, a diagnosis of Miller-Fisher syndrome was made. Neuroimages obtained at that time revealed an ectatic left VA with minimal cervicomedullary compression. The patient continued to deteriorate despite medical management. Follow-up imaging demonstrated worsened cervicomedullary compression. An emergency posterior fossa neurovascular decompression was performed using a Gore-Tex sling and resulted in mild neurological improvement. This case emphasizes that early recognition and surgical intervention to prevent progressive neurological sequelae are crucial in symptomatic VA dolichoectasia.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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