1/101. A case of cervical myelopathy with developmental canal stenosis at the level of the atlas. A case report.The craniocervical junction is one of the most common sites of malformations. Only three cases of myelopathy due to hypoplasia of atlas have been reported previously. Among these malformations, the hypoplasia of atlas was first described by Wackenheim in 1974. Although developmental canal stenosis due to the hypoplasia of atlas seems to have a tendency of causing the cervical myelopathy, only three cases of cervical myelopathy due to this condition have been reported previously. A 77-year-old man with severe canal stenosis at the level of the atlas is reported. The clinical manifestations were 20-year history of progressive gait disturbance and paresis of both upper and lower extremities. The spinal cord was markedly compressed at the level of the atlas. The clinical manifestations improved after a resection of posterior arch of the atlas.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/101. Cervical subarachnoid hematoma of unknown origin: case report.OBJECTIVE AND IMPORTANCE: Spontaneous spinal subarachnoid hematoma is rare, having been reported in the English literature in only seven other cases. We describe the first case of spontaneous subarachnoid hematoma located in the cervical spinal cord of a 43-year-old man. The pathologic examination showed no apparent source of bleeding, but there was evidence of cervical spondylotic myelopathy. CLINICAL PRESENTATION: The patient presented with a 10-day history of severe neck pain, followed by the onset of quadriparesis that was more evident on the left side, urinary retention, and sensory loss below C5. His medical history included hypertension. magnetic resonance imaging showed a massive hemorrhage in the cervical spinal canal. INTERVENTION: A C4-C5 subarachnoid hematoma was removed. The patient died due to respiratory distress and uncontrollable hypotension on day 6 after surgery. Surgical exploration, neuroradiologic examinations, and autopsy showed no evidence of vascular malformations, tumors, or other possible sources of bleeding. CONCLUSION: After excluding more common causes of spontaneous subarachnoid hematoma in this patient, we suggest that chronic spinal cord compression (spondylotic myelopathy) and arterial hypertension in this patient may have caused the pathogenesis of this rare clinical entity. Experimental data supporting this hypothesis are discussed.- - - - - - - - - - ranking = 0.5keywords = malformation (Clic here for more details about this article) |
3/101. 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 = 1.0830861113347keywords = nervous system (Clic here for more details about this article) |
4/101. Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome.The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardio-facial syndrome. This study was a prospective evaluation of 41 consecutive patients with velocardiofacial syndrome, documented by fluorescence in situ hybridization, between March of 1994 and September of 1998. The 23 girls and 18 boys ranged in age from 0.5 to 15.2 years, with a mean age of 6.7 years. Nineteen patients were assessed with magnetic resonance imaging, 39 underwent lateral cephalometric radiography, and all patients were examined for neurologic deficits. Eight of 19 patients (42 percent) had anomalies of the craniovertebral junction, including Chiari type I malformations (n = 4), occipitalization of the atlas (n = 3), and narrowing of the foramen magnum (n = 1). One patient with Chiari malformation required suboccipital craniectomy with laminectomy and decompression. Fourteen of 41 patients (34 percent) had demonstrated neurologic deficits; 10 patients (24 percent) had velar paresis (6 unilateral and 4 bilateral). Chiari malformations, cervical spine anomalies, and neurologic deficits are common in velocardiofacial syndrome. Because these findings may influence the outcome of surgical intervention, routine assessment of patients with velocardiofacial syndrome should include careful orofacial examination, lateral cephalometric radiography, and magnetic resonance imaging of the craniovertebral junction.- - - - - - - - - - ranking = 4keywords = malformation (Clic here for more details about this article) |
5/101. Non-traumatic spinal extradural haematoma: magnetic resonance findings.Non-traumatic extradural spinal haematoma is an uncommon condition that is usually associated with a poor outcome. It may present acutely with signs and symptoms of major neurological dysfunction secondary to cord compression, or subacutely over a number of days or weeks with fluctuating symptoms. The exact aetiology of this condition is incompletely understood, but it is believed that the blood is venous in origin, as distinct from the arterial origin of intracranial extradural haematomas. Causes of non-traumatic extradural spinal haematoma include anticoagulation, vasculitis such as systemic lupus erythematosus (SLE), and spinal arteriovenous malformations. Conditions that may mimic an acute spinal haematoma include extradural abscess and extradural metastatic infiltration. It is important to make a diagnosis of extradural compression because surgery may offer the best hope in restoring neurological function in these patients. Imaging modalities used for the investigation of extradural haematomas include myelography, CT myelography (CTM) and MRI with or without gadolinium enhancement. The MR appearances of acute extradural abscess and extradural tumour can mimic an extradural haematoma. In subacute haematoma, owing to the magnetic properties of blood degradation products, MR is more specific in diagnosing and ageing of the haematoma.- - - - - - - - - - ranking = 0.5keywords = malformation (Clic here for more details about this article) |
6/101. 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 = 3.2492583340041keywords = nervous system (Clic here for more details about this article) |
7/101. 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 = 1.5830861113347keywords = nervous system, malformation (Clic here for more details about this article) |
8/101. Acute spontaneous spinal epidural haematoma in a child.Spontaneous spinal epidural haematomas rarely occur. patients tend to be in their sixties or seventies. Acute spontaneous spinal epidural haematomas in children without a predisposition for bleeding disorders, trauma, vascular malformations or anticoagulant therapy have seldom been described. We present a case of a 4-year-old girl with a spontaneous cervical epidural haematoma diagnosed with MR.- - - - - - - - - - ranking = 0.5keywords = malformation (Clic here for more details about this article) |
9/101. 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 = 2.1661722226694keywords = nervous system (Clic here for more details about this article) |
10/101. 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 = 1.0830861113347keywords = nervous system (Clic here for more details about this article) |
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