Dorsal thoracic arachnoid web and the "scalpel sign": a distinct clinical-radiologic entity.

Arachnoid webs are intradural extramedullary bands of arachnoid tissue that can extend to the pial surface of the spinal cord, causing a focal dorsal indentation of the cord. These webs tend to occur in the upper thoracic spine and may produce a characteristic deformity of the cord that we term the "scalpel sign." We describe 14 patients whose imaging studies demonstrated the scalpel sign. Ten of 13 patients who underwent MR imaging demonstrated T2WI cord signal-intensity changes, and 7 of these patients also demonstrated syringomyelia adjacent to the level of indentation. Seven patients underwent surgery, with 5 demonstrating an arachnoid web as the cause of the dorsal indentation demonstrated on preoperative imaging. Although the webs themselves are rarely demonstrated on imaging, we propose that the scalpel sign is a reliable indicator of their presence and should prompt consideration of surgical lysis, which is potentially curative.

Epidermoid cysts associated with thoracic meningocele.

Intraspinal tumours of cutaneous origin associated with various spinal dysraphisms have been well documented in the literature. However, the metachronous development of intra- and extra-medullary tumours in conjunction with dorsal meningocele is rare. The authors report a patient with a thoracic dorsal meningocele and congenital intradural extramedullary epidermoid tumour. The patient developed an intramedullary epidermoid growth 12 years later. Subtotal resection of the tumour predisposed to a later recurrence. Meningocele is not always an isolated clinical entity but the concurrent occult lesions are usually veiled by the more conspicuous surface anomaly. Thorough magnetic resonance imaging of the whole neural axis helps to identify associated pathologies. Delicate intradural exploration by a microsurgical approach is necessary to achieve appropriate treatment.

Fracture of anterior cervical plate implant--report of two cases.

Anterior cervical plate is a convenient and popular implant employed after corpectomy and bone grafting for traumatic or degenerative cervical spine disease. Although short-segment anterior cervical surgery may adequately be managed with anterior plate fixation alone, multilevel anterior cervical constructs exhibit a relatively high complication rate warranting a simultaneous posterior fusion. We report two patients undergoing two-level anterior corpectomy/fusion with a fixed anterior plate alone and exhibiting plate fracture in conjunction with pseudoarthrosis. The instrument failure was mainly attributed to pseudoarthrosis. However, improper contouring of the plate causing microstructural damage might create a weak point and contributed to this unusual hardware failure.

Simultaneous multiple hypertensive intracerebral haemorrhages.

BACKGROUND: Simultaneous occurrence of multiple intracerebral haemorrhages (ICHs) in different arterial territories is a rare clinical event which has been reported to be associated with cerebral amyloid angiopathy, venous sinus thrombosis, coagulopathy, vasculitis, haemorrhagic transformation of cerebral infarcts and multiple intracranial pathologies such as vascular anomalies or tumours. Although hypertension is the most common etiological factor for the development of spontaneous single intracerebral bleeding, its role in simultaneous multiple ICHs is not clear. METHODS: The authors have reviewed all patients with non-traumatic ICH admitted to Kaohsiung Medical University Hospital from 1993 to 2002. Ten hypertensive patients with simultaneous multiple ICHs were found. For the purpose of comparison, another 600 cases with solitary hypertensive ICH were also reviewed as a control group. Computerized tomographic scans and medical records concerning patients' histories, clinical presentations, locations of haematomas, associated risk factors, and outcome were analyzed. FINDINGS: The mean age and sex distribution were similar in both patient groups. Bilateral putaminal or thalamic haemorrhages were the most common combinations of simultaneous bleedings. As for the individual location of haematoma, there was a strong preponderance for the supratentorial space with the thalamus being the most preferable site. The duration of hypertension was longer and the percentage of previous stroke was higher in patients with multiple ICHs. Other associated risk factors were similar in both groups except for higher incidence of hypercholesterolemia in multiple ICHs group. Patients with simultaneous multiple ICHs had a much worse outcome compared to those with solitary ICH. CONCLUSIONS: As with solitary ICH, hypertension is still the most important etiological factor for simultaneous multiple ICHs. The widespread and prolonged degeneration of intracerebral arterioles predispose patients to the development of multiple ICHs, which could be justified by the longer history of hypertension and higher incidence of former strokes. Only hypercholesterolemia was identified to be significantly associated with this unusual brain event in our study. The mechanism underlying the development of simultaneous multiple ICHs is not clear although structural and haemodynamic changes of first haemorrhage may be responsible for the second one. Poorer outcome in patients with multiple ICHs can be explained by the concomitant destruction of crossing and non-crossing fiber tracts and bilateral diaschisis phenomenon.

Factors influencing seizures in adult patients with supratentorial astrocytic tumors.

Seizures and epilepsy in adults are important and increasingly common clinical problems. Despite this, the investigation of seizures in adults with astrocytic tumors remains a grey area. The incidence and influencing factors of preoperative and postoperative seizures were evaluated in 101 patients of 45 years or older with supratentorial astrocytic tumors. Preoperative seizures occurred in 14 (14%) patients. Seizures at presentation were significantly correlated with pathological grades of astrocytic tumors (p = 0.0318). The risk of seizures at presentation was greatest in patients with well-differentiated astrocytomas as compared with anaplastic astrocytomas (Odds ratio = 4.364, p = 0.056) or glioblastomas multiforme (Odds ratio = 5.673, p = 0.007). There was no association of preoperative seizures with age, sex, location or site of the tumors. Postoperative seizures occurred in 18 (18%) patients, including 8 (8/14, 57%) recurrent seizures and 10 (10/87, 12%) late-onset seizures. Postoperative seizures were significantly correlated with the presence of preoperative seizures (p = 0.0003). The presence of preoperative seizures was potentially predictive of postoperative seizures when evaluated by logistic regression model (Odds ratio = 6.650). Thirteen (72%) of 18 patients with postoperative seizures were associated with tumor recurrence in 7 cases, hemorrhage in 3 cases and malignant progression in 3 cases. There was no association of postoperative seizures with age, sex, location or site of the tumors, grades of tumors, type of preoperative seizures, duration of preoperative seizures, serum level of anticonvulsant drug, extent of surgery, postoperative radiation or chemotherapy. The patients with preoperative seizures had a higher risk of postoperative seizures and should be carefully monitored. Imaging examination of brain to exclude the possibilities of tumor recurrence or hemorrhage is warrantable in supratentorial astrocytoma patients with postoperative seizures.

The conditional probabilities of survival in patients with anaplastic astrocytoma or glioblastoma multiforme.

BACKGROUND: By the use of conditional probabilities of survival, we studied the yearly survival rates for individual tumor survivors. METHODS: Conditional survival rate was estimated in 114 consecutive patients with anaplastic astrocytoma or glioblastoma multiforme. Conditional probabilities of surviving some years given survival to a specific period of time after craniotomy and 95% confidence intervals were calculated in the individual tumor survivors. RESULTS: The estimated median survival was 30 months for 45 patients with anaplastic astrocytoma and 12 months for 69 patients with glioblastoma multiforme. The conditional probabilities of surviving next one year given survival to 1 year, 2 years, 3 years, 4 years, or 5 years after craniotomy for anaplastic astrocytoma were 86.2%, 75.0%, 85.9%, 77.8%, or 85.7%, respectively; for glioblastoma multiforme 64.8%, 58.7%, 85.7%, 80.0%, or 75.0%, respectively. The conditional probability of surviving to 5 years given survival to 2 years after craniotomy for anaplastic astrocytoma, i.e., surviving an additional 3 years, was 50.1%, which was better than observed 5-year survival rate (28.6%); for glioblastoma multiforme it was 40.2%, which also was better than observed 5-year survival rate (12.4%). CONCLUSIONS: The conditional probability of survival was a good method to clinically predict yearly survival rate for individual tumor survivors. In addition, the method can estimate the probabilities of surviving next some years given survival to a specific period of time after craniotomy. It also showed a more encouraging result than observed survival rate in patients with supratentorial malignant astrocytomas.

Effects of endothelin-converting enzyme inhibitors on hemolysate-induced morphological changes and production of endothelin-1 in bovine cerebrovascular endothelial cells.

The effects of CGS 26303, a dual inhibitor of endothelin-converting enzyme (ECE) and neutral endopeptidase 24.11, and its prodrug, CGS 26393, on bovine cerebrovascular endothelial cells stimulated with hemolysate were investigated. Upon incubation with hemolysate for 48 h, cell density was significantly decreased, with concomitant increases in endothelin-1 (ET-1) (42 vs 11 pg/ml) and big ET-1 (79 vs 27 pg/ml) levels in culture medium when compared with controls. Simultaneous addition of CGS 26303 (10 and 100 microM) and hemolysate protected against cell loss and decreased cellular vacuolization caused by hemolysate. The levels of ET-1 and big ET-1 in the culture medium were decreased dose-dependently. More drastically, pretreatment with 100 microM CGS 26303 for 30 min decreased the production of ET-1 and big ET-1 by 94% and 87%, respectively, when compared with the untreated control. However, treatment with CGS 26393 was much less effective. These results suggest that suppression of ET-1 production by ECE inhibitors may prove to be efficacious for the treatment of hemolysate-induced cytotoxicity on cerebral endothelial cells.

Growing skull fractures.

Growing skull fractures are rare complications of severe closed head injury occurring almost exclusively in infants and children under the age of three. The reported incidence is between 0.05% and 0.6%. Two such cases were treated at our institution. Both cases presented with bulging mass and underlying skull defect two months after head injury. They received surgical treatment and achieved excellent results. Early diagnosis and surgical intervention are essential for these patients to prevent progressive brain damage. Therefore, close follow-up for patients at risk of developing growing skull fracture is necessary.

The long-term results of upper dorsal sympathetic ganglionectomy and endoscopic thoracic sympathectomy for palmar hyperhidrosis.

To assess and compare the long-term results of upper dorsal sympathetic ganglionectomy (UDS) and endoscopic thoracic sympathectomy (ETS), we examined 84 patients who underwent UDS and 71 patients who underwent ETS for the treatment of palmar hyperhidrosis. The period of follow-up ranged from 37 to 228 months. The immediate success rate was 100% in the UDS group and 98.6% in the ETS group. Troublesome compensatory hyperhidrosis occurred in 67.8% of the UDS patients and 84.8% of the ETS patients; however, 55% of the UDS patients and 63% of the ETS patients felt satisfied with their operation. The main reasons for dissatisfaction were recurrence and compensatory hyperhidrosis. Interestingly, simultaneous cure of plantar hyperhidrosis occurred in 28 (40%) of the UDS patients and 28 (44%) of the ETS patients with concomitant plantar hyperhidrosis. ETS required both a shorter operation time and hospital stay than UDS. Thus, we now perform ETS as the treatment of choice because of its excellent illumination and adequate magnification via a minimally invasive approach. The use of ETS as the first choice of treatment for palmar hyperhidrosis is supported not only by the immediate results, complications, and cure of plantar hyperhidrosis, but also by the long-term results. Nevertheless, compensatory hyperhidrosis was also a major complication after ETS.

Identification and characterization of a functional human Ig V lambda VI germline gene.

We have isolated from a human genomic library a potentially functional and distinctive germline gene, designated IGLV6S1, that encodes for light chains of the V lambda VI subgroup. An identical germline gene was cloned from fibroblasts obtained from a patient with light-chain-associated amyloidosis (AL amyloidosis) whose serum and urine contained, respectively, a monoclonal IgG lambda VI protein and a lambda VI Bence Jones protein. Isolation and characterization of cDNA cloned from the patient's bone marrow-derived monoclonal plasma cells revealed that the nucleotide and predicted protein sequences of the rearranged gene were approximately 95% and approximately 90% homologous to those of the germline gene, respectively. The finding that the transcriptional start site for lambda VI RNA synthesis was located upstream of the putative TATA-box promoter, rather than downstream as found for the V lambda II subgroup, implies that a different transcriptional machinery controls the expression of the human V lambda VI-gene family.