1/167. The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. methods: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/167. Respiratory sinus arrhythmia of brainstem lesions.In this pilot study we investigated the hypothesis that intrinsic and extrinsic brainstem lesions situated within the pontomedullary region would effect the integrity of respiratory sinus arrhythmia. The study sample consisted of three patients with anatomic brainstem abnormalities associated with isolated Chiari I malformation, Chiari II malformation with syringobulbia, and achondroplasia with cervicomedullary compression. They were compared to an age- and sex-matched control group of nine patients. Each subject's electrocardiogram was recorded in a quiet room and digitized by a personal computer during five 1-minute periods. R-R intervals within each 1-minute period were converted to heart rate in 120 successive 0.5-second intervals. The resultant heartrate time series was converted to its underlying frequency composition by a fast Fourier transform and averaged across minutes. Respiratory sinus arrhythmia was defined as the variability in the time series over a frequency range (0.096 to 0.48 Hz) corresponding to a range of respiratory rates from 6 to 30 breaths per minute. Analysis revealed a significant reduction in respiratory sinus arrhythmia (P < .05), defined as the summated area under the curve, with a mean for controls of 35.42 /-28.13 SD and for subjects of 17.20 /-11.50 SD. There was a gradient of abnormality noted, with the mildest deviation in respiratory sinus arrhythmia for the patient with isolated Chiari I malformation and maximum deviation seen in the patient with extrinsic cervicomedullary compression.- - - - - - - - - - ranking = 3keywords = malformation (Clic here for more details about this article) |
3/167. Idiopathic localized hydromyelia: dilatation of the central canal of the spinal cord of probable congenital origin.Three adult patients are reported with asymptomatic localized widening of the central canal of the spinal cord. These patients were followed for a period of 24 years by imaging and/or clinical history and physical examination without evidence of signs or symptoms related to the spinal cord. This condition probably represents persistence into adult life of a fetal configuration of the central canal of the spinal cord. This process may be termed "idiopathic localized hydromyelia" to distinguish it from syringomyelia secondary to such causes as Chiari malformation, trauma, infection, or neoplasm.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
4/167. magnetic resonance imaging in five patients with a tumefactive demyelinating lesion in the central nervous system.Five patients with a tumefactive lesion were clinically followed from 1992 to 1993. Four patients were female; age ranged from 32 to 57 years, the duration of symptoms varied from 3 days to 3 years. Neurological examination disclosed dementia in two patients, aphasia in three, hemiparesis in four, hemihypoaesthesia in three, optical neuritis in two, tetraparesis with sensitive level and neurogenic bladder in one. MRI disclosed lesions with a hypersignal on images assessed at T2 and hyposignal at T1, and gadolinium heterogeneous enhancement; these lesions were located in the: a) temporooccipital region bilaterally and brain stem, b) frontoparietal white matter, c) basal ganglia, bilateral white matter and brain stem, d) left parietal region, e) cervical spinal cord, with enlargement of this region. Cerebral biopsy was performed in three patients; acute and subacute demyelinating disease was diagnosed by histological examination. Two patients had an evolutive diagnosis; exclusion of other pathologies and clinical and radiological improvement after corticotherapy, pointed to an inflammatory disease.- - - - - - - - - - ranking = 10.635929992514keywords = nervous system (Clic here for more details about this article) |
5/167. Progressive necrotic myelopathy: clinical course in 9 patients.OBJECTIVE: To review the clinical, laboratory, and radiological findings of 9 patients who had progressive idiopathic myelopathy with evidence of spinal cord necrosis. DESIGN AND methods: We reviewed personally examined cases of myelopathy that fulfilled the following criteria: (1) regional loss of reflexes, flaccidity, and muscle atrophy; (2) magnetic resonance imaging showing a shrunken or cavitated cord without evidence of arteriovenous malformation; (3) electromyogram showing denervation over several contiguous spinal cord sgements with preservation of sensory potentials in some cases; and (4) the absence of evidence of systemic disease or neoplasm. RESULTS: The illness began in these patients after the age of 40 years, with prominent burning or tingling limb pain, occasionally with radicular features or with less well-defined back, neck, or abdominal pain. Leg or infrequently arm weakness appeared concurrently or soon after the onset of pain. The most distinctive feature was a saltatory progression of symptoms, punctuated by both acute and subacute worsenings approximately every 3 to 9 months, culminating in paraplegia or tetraplegia. The distinguishing clinical findings, together indicative of destruction of gray matter elements of the cord, were limb atrophy, persistent areflexia, and flaccidity. The concentration of cerebrospinal fluid protein was typically elevated between 500 g/L and 1000 g/L, without oligoclonal bands, accompanied infrequently by pleocytosis. magnetic resonance imaging showed features suggesting cord necrosis, specifically swelling, T2-weighted hyperintensity, and gadolinium enhancement over several spinal cord segments, succeeded months later by atrophy in the same regions. necrosis of the cord was found in biopsy material from one patient and postmortem pathology in another case, but inflammation and blood vessel abnormalities were absent. Only 2 patients had prolonged visual evoked responses. The disease progressed despite immune-modulating treatments although several patients had brief epochs of limited improvement. CONCLUSIONS: The saltatory course, prolonged visual evoked responses in 2 patients, and a cranial abnormality on magnetic resonance imaging in another, raised the possibility of a link to multiple sclerosis. However, the normal cranial magnetic resonance imaging scans in 6 other patients, uniformly absent oligoclonal bands, and poor response to treatment were atypical for multiple sclerosis. On the basis of shared clinical and laboratory features, idiopathic progressive necrotic myelopathy is indistinguishable from a limited form of Devic disease.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
6/167. Cervical epidural pseudotumor and multifocal fibrosclerosis. Case report and review of the literature.The authors present the case of a 45-year-old man suffering from progressive quadriplegia due to an expansive C3-T2 epidural mass. Neuropathological examination demonstrated pseudotumor tissue. The patient had had an orbital pseudotumor 5 years before admission, and other systemic manifestations of an idiopathic inflammatory disease were discovered. This case is extremely rare. Nine cases of multifocal fibrosclerosis with central nervous system involvement are described in the literature. To the authors' knowledge, this is the first description of a cervical epidural pseudotumor. Modern imaging has made the diagnosis of such an entity possible, and it is important for the neurosurgeon to consider this syndrome because the combination of surgery and systemic medical therapy can ensure a long-term survival with good quality of life.- - - - - - - - - - ranking = 2.6589824981284keywords = nervous system (Clic here for more details about this article) |
7/167. Transganglionic gracile response following limb amputation in man.Gracile neuroaxonal dystrophy (nad) is an distinctive morphological alteration of central projecting axon terminals of dorsal root ganglion neurons. Experimentally, lower limb amputation has been shown to accelerate the formation of gracile nad, suggesting that the transganglionic response to peripheral axotomy may play a role in its development. To determine if a similar response occurs in the human sensory nervous system following peripheral nerve injury, we have performed postmortem histopathological examinations of the dorsal column nuclei of three patients (aged 15, 55, and 77 years old); all of whom had undergone accidental or therapeutic unilateral limb amputation (1 year, 38 years, and 1 year 8 months prior to death, respectively). In a 15-year-old man who underwent therapeutic leg amputation, the gracile nuclei on the transected side revealed reactive gliosis and many small axonal spheroids. The spheroids and fine neurites were immunolabelled with antibodies for growth-associated protein-43, ubiquitin and neuropeptide y (NPY). Neither routine histological nor immunohistochemical methods demonstrated comparable changes in the contralateral gracile nucleus. In a 77-year-old man who underwent leg amputation, the gracile nucleus on the amputated side was gliotic and showed several NPY and ubiquitin-immunoreactive spheroids, which were not seen in the contralateral non-transected side. A 55-year-old man with a history of accidental arm amputation showed well-developed nad in the cuneate nucleus only on the transected side. This study clearly demonstrates the occurrence of transganglionic response to limb amputation in human dorsal column nuclei. The extent of the regenerative and/or degenerative responses may vary depending on the age of the patient and the time interval following the peripheral axotomy.- - - - - - - - - - ranking = 2.6589824981284keywords = nervous system (Clic here for more details about this article) |
8/167. Extramedullary hemangioblastoma of the conus medullaris.BACKGROUND: We report the case of an extramedullary pathologically proven hemangioblastoma of the conus medullaris. As spinal dural arteriovenous fistulas most commonly present with a conus medullaris syndrome, our presentation of the MRI, myelographic, and angiographic findings of this unique lesion may be useful in differentiating these two entities. CLINICAL MATERIAL: We report the case of a 57 year old woman with a two year history of progressive low back and right lower extremity pain and weakness. Spinal MRI and myelography demonstrated serpiginous vasculature on the dorsum of the spinal cord consistent with either a vascular tumor or malformation. Selective spinal angiography was thus undertaken by the neuroendovascular team which revealed a tumor nodule consistent with vascular tumor. T12-L1 laminectomy was performed and a 6 mm vascularized tumor was found in the intradural extramedullary compartment adjacent to the conus medullaris. The tumor was completely removed and pathological analysis was consistent with hemangioblastoma. CONCLUSION: This report documents a unique location for extramedullary spinal hemangioblastomas. Although both MRI and myelography are helpful in studying these lesions, angiography remains the gold standard in differentiating between vascular tumor and malformation. We suggest that the angiography be performed by a neurointerventional team to facilitate embolization, should this be warranted.- - - - - - - - - - ranking = 2keywords = malformation (Clic here for more details about this article) |
9/167. Spinal AVM, epidermal nevus, and rhabdomyosarcoma: A rare neurocutaneous syndrome?A 26-year-old man with a history of an embryonal rhabdomyosarcoma arising from the urachus and a large, right-sided, epidermal nevus presented with a rapidly evolving tetraparesis. Investigations confirmed an intramedullary hemorrhage of the cervical spinal cord and an extensive arteriovenous malformation (AVM). An association between his nevus, rhabdomyosarcoma, and spinal AVM is hypothesized.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
10/167. 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 = 1keywords = malformation (Clic here for more details about this article) |
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