Biomedical subjects
Publications and source records attributed to B Irthum.
Interleukin-6 (IL-6) is known to promote tumour growth and survival. We evaluated IL-6 gene amplification in tumours from 53 glioma patients using fluorescence in situ hybridisation. Amplification events were detected only in glioblastomas (15 out of 36 cases), the most malignant tumours, and were significantly associated with decreased patient survival.
OBJECTIVE: The purpose of the study was to report two cases of cervical and para-pharyngeal bone tumors. MATERIAL AND METHODS: Patients were two 29 and 67-year-old men. Presentation of the lesions included respectively a right cervical mass and a left para-pharyngeal mass. Clinical features and radiological, anatomopathological and therapeutic characteristics of the tumors were retrospectively studied. RESULTS: A cervical approach was made in both cases. Tumor biopsies revealed a vertebral aneurismal cyst and a corporeo-pedicular chordoma respectively. CONCLUSION: Vertebral bone tumors with cervical expression are very uncommon entities. Diagnosis could be systematically evoked in patients with a cervical or para-pharyngeal tumor presenting vertebral lysis.
BACKGROUND: The training of local clinicians is the best way to raise the standard of medical knowledge in developing countries. This requires transferring skills, techniques and resources. OBJECTIVES: Grid technology opens new perspectives for preparation and follow-up of medical missions in developing countries as well as support to local medical centers in terms of teleconsulting, telediagnosis and patient follow-up. Indeed, grids allow to hide the complexity of handling distributed data in such a way that physicians will be able to access patient data while ignoring where these data are stored. METHODS: To meet requirements of a development project of the French NPO Chain of Hope in China, we propose to deploy a grid-based federation of databases. FIRST RESULTS AND CONCLUSIONS: A first protocol was established for describing the patients' pathologies and their pre- and post-surgery states through a web interface in a language-independent way. This protocol was evaluated by French and Chinese clinicians during medical missions in the fall of 2003. The first sets of medical patients recorded in the databases will be used to evaluate grid implementation of services.
Cerebral cavernous malformations (CCMs) are hamartomatous vascular malformations characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. They cause seizures and cerebral hemorrhages, which can result in focal neurological deficits. Three CCM loci have been mapped, and loss-of-function mutations were identified in the KRIT1 (CCM1) and MGC4607 (CCM2) genes. We report herein the identification of PDCD10 (programmed cell death 10) as the CCM3 gene. The CCM3 locus has been previously mapped to 3q26-27 within a 22-cM interval that is bracketed by D3S1763 and D3S1262. We hypothesized that genomic deletions might occur at the CCM3 locus, as reported previously to occur at the CCM2 locus. Through high-density microsatellite genotyping of 20 families, we identified, in one family, null alleles that resulted from a deletion within a 4-Mb interval flanked by markers D3S3668 and D3S1614. This de novo deletion encompassed D3S1763, which strongly suggests that the CCM3 gene lies within a 970-kb region bracketed by D3S1763 and D3S1614. Six additional distinct deleterious mutations within PDCD10, one of the five known genes mapped within this interval, were identified in seven families. Three of these mutations were nonsense mutations, and two led to an aberrant splicing of exon 9, with a frameshift and a longer open reading frame within exon 10. The last of the six mutations led to an aberrant splicing of exon 5, without frameshift. Three of these mutations occurred de novo. All of them cosegregated with the disease in the families and were not observed in 200 control chromosomes. PDCD10, also called "TFAR15," had been initially identified through a screening for genes differentially expressed during the induction of apoptosis in the TF-1 premyeloid cell line. It is highly conserved in both vertebrates and invertebrates. Its implication in cerebral cavernous malformations strongly suggests that it is a new player in vascular morphogenesis and/or remodeling.
The presence of telomerase activity in a glioma may be a predictor of its malignant potential. Activation of telomerase is regulated at the transcriptional level of the human telomerase reverse transcriptase (hTERT). Here, we evaluated whether the amount of hTERT mRNA provides a molecular marker of glioma malignancy that would have clinical utility. We used a real-time RT-PCR to assess the number of hTERT transcripts in primary tumour samples derived from 70 glioma patients. Results were standardised by quantifying the number of ABL transcripts as internal control and expressed as hTERT/ABL ratio. The percentage of patients with detectable hTERT mRNA markedly increased with enhanced malignancy: low-grade gliomas expressed hTERT in one out of 14 cases (7.1%), anaplastic gliomas in four out of 13 cases (30.8%) and glioblastoma multiforme (GBM) tumours in 30 out of 43 cases (69.8%). The mean hTERT/ABL ratio was significantly higher in GBMs than in non-GBMs. Subdividing hTERT/ABL ratios as low (< pr = 25%) and high (>25%), we found that the overall survival among hTERT-positive GBMs was significantly worse in high hTERT expressors than in low hTERT expressors (P=0.0082). We conclude that the amount of hTERT mRNA may represent a diagnostic and prognostic indicator for GBM patients.
Conventional treatment of high-grade glioma includes maximal surgical resection followed by external radiation therapy. Despite this treatment, the prognosis for patients is poor. High doses of chemotherapy might be another way to increase the response rate and median survival. Increasing doses of BCNU might be more effective, but also provokes unacceptable myelotoxicity. This dose-limiting toxicity can be circumvented by using autologous blood stem cell rescue. We report our experience of high-dose BCNU followed by transplantation of autologous hematopoietic stem cells in 114 patients with high-grade gliomas. Of the 114 gliomas, 78 were glioblastoma multiforme (GM) (68%), 24 anaplastic astrocytomas (AA) (21%), and 12 anaplastic oligodendrogliomas (OD) (11%). Complete surgical resection was performed for 22 patients (18 GM and 4 AA). The median age was 44 years (range 17-65). A total of 84 patients received autologous hematopoietic stem cells from bone marrow harvest, while 30 patients received granulocyte colony-stimulating factor followed by apheresis and received peripheral blood progenitor cells (PBPC). High dose of BCNU (800 mg/m(2)) was given at least 1 month after neurosurgery. Bone marrow or PBPC was transplanted 48-72 h after chemotherapy. Radiotherapy was started approximately 40 days after transplantation to a total of 60 Gy. Median follow-up was 89 months (19-163). The overall survival (OS) was, respectively, 12 months for GM, 37 months for OD and 81 months for AA. Histological type appeared to be the main discriminating factor, with a worse prognosis for GM. Within the GM population, age, completeness of surgery, and response appeared to be one important prognostic factors. The AA and OD populations were small to reliably assess prognostic factors. On multivariate analysis, the main prognostic factors were histologic type, quality of surgery, and age (P<0.005). Five of 114 patients had lethal complications from the procedure. Four of these patients had a Karnovsky performance score (KPS) of 60%. The protocol thus appears to be feasible but patients should be selected for KPS more than 70%. We observed long-term survivors, although the OS and the time to treatment failure seem to be comparable to that described for other treatment. Additional pilot studies are unlikely to reveal more than a modest benefit from this procedure and therefore a randomized study should be performed.
We report two cases of spontaneous anteromedial temporo-sphenoidal meningo-encephalocele. The first patient was a 67-year-old man with a history of treated epiglotic cancer 6 years ago, was referred to us for CSF rhinorrhea. Lateral sphenoidal CSF fistula was confirmed by CT cisternography. Four lumbar taps were performed and rhinorrhea stopped for 2 years. At relapse of rhinorrhea, the patient was operated on via a subtemporal approach. The dural defect was filled by an intradural patch of pericranium. Four years later patient showed no evidence of cerebrospinal fluid leakage. The second patient was a 54-year-old woman referred to us after a one year history of CSF rhinorrhea. CT cisternography confirmed lateral sphenoidal CSF fistula. The patient was operated on via a subtemporal approach. The dural defect was filled by a patch of pericranium. Three years later the patient had no evidence of cerebrospinal fluid leakage. Twenty-one cases of anteromedial temporosphenoidal meningo-encephaloceles have been reported in the literature. They probably are of a developmental origin. Some cases have been successfully treated via a transsphenoidal route. However surgical failures related to this approach can be due to the lack of control of the lateral recess of the sphenoid sinus. Therefore, the frontotemporal approach seems more adequate but the transphenoidal endoscopic approach will probably be used in the future.
Slowly varying pressure oscillations in the cranial enclosure are well known, especially intracranial pressure waves as best described by the pioneering works of Janny and Lundberg. Nevertheless, in spite of over twenty five years research on intracranial pressure waves, their origin and regulation remain unclear but are often considered only as pathological. Our aim was to review data on these phenomena to clarify their biological status and the role that they could play in the management of patients suffering from such intracranial neurosurgical diseases as intracranial hypertension, severe head injury, and hydrocephalus. It appears that these pressure waves reveal important information on the function of the cerebral vasculature and as such have significance for influencing intracranial compliance. Pressure waves are also closely associated with autoregulation, in particular dynamic autoregulation. It seems evident that they are not only pathophysiological but also physiological, linked with other biological parameters such as the neurovegetative cardiovascular system, breathing, and sleeping. This study shows that it is not only important to continue to explore these slow waves, but also the methods of analysis in order to more fully clarify their clinical significance.
BACKGROUND: Intraoperative fluoroscopy imaging during coronaroplasty or transjugular intrahepatic portosystemic shunt may induce chronic radiodermatitis. Temporary hair loss is a peculiar form of radiodermatitis following endovascular surgery of the cerebral arteries. CASE REPORTS: Six patients (2 women, 4 men, age range: 27-47 years old) were seen for a solitary plaque of alopecia. In all of the cases, the plaque had appeared two weeks after a neuroradiologically guided embolization procedure. No other skin lesions were seen. Alopecia spontaneously and completely regressed within three to four months. However, it reappeared after a subsequent embolization (one case) but not after arteriographies (five cases). DISCUSSION: Five similar cases have been reported in the literature. Transient alopecia often occurs after neurologically guided endovascular surgery of the cerebral arteries. This side-effect is well known by neurosurgeons and thus, these patients are rarely referred to a dermatologist. Two differential diagnoses must be evoqued: alopecia aerata and anticonvulsant - induced alopecia. The role played as cofactor by carbamazepine which is a photosensitivant drug, is discussed.
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BACKGROUND AND PURPOSE: The aim of this study is to evaluate retrospectively the benefit of the surgical spinal decompression in 152 patients with spinal metastasis. METHODS: Based on clinical notes and GP inquiry, we determined the actuarial survival curve, and assessed the pain level one month post-operatively and the motor, the sensory and sphincter distrurbances three months after surgery. RESULTS: Thoraco-lumbar lesions were usually treated using a posterior approach, with a laminectomy and if necessary an osteosynthesis. Cervical lesions were treated with an anterior approach, i.e. a corporectomy and a methylmetacrylate stabilization. Sixty eight percent of patients (103/152) had pre or postoperative radiotherapy. After the surgical decompression for a spinal metastasis, our study demonstrated an improvement in sensory status (31 % of the patients), in motor ability (56 %), in sphincter function (51 %), and a decrease in the pain intensity in 47 % of the patients. Among 83 patients who could not walk on admission (grade A, B and C of Frankel), 52 % recovered a gait function 3 months post operatively. The best benefit after surgery concerned grade C patients, of which 71 % recovered the gait function. Two percent of the patients had postoperative worsening of their motor strength. No operative mortality was noted, and the postoperative mortality rate was 3 % at 7 days and 9 % at 30 days. The analysis of the actuarial survival curve demonstrated a mean follow up of 3.7 years. The mean survival time was 12 months with 25 % of patients surviving 2 years. CONCLUSION: Surgical decompression is effective in relieving neurological symptoms from spinal metastasis. In our experience a complete motor deficit does not seem to be a good surgical indication because of the lack of postoperative improvement.
We evaluated the direct location in the globus pallidus (GP) under stereotactic MRI (sMRI) guidance in five parkinsonians treated with chronic deep brain stimulation (four bilaterally). The sMRI consisted of three orthogonal (horizontal, frontal, sagittal) sets of images obtained with a stereotactic frame and its localiser. The sMRI was coupled with ventriculography to compare the location with the classic indirect method based on commissural landmarks. The target was defined on T2-weighted slices in the anterior part of the medial GP, at the vertex of the nucleus. It was reached via one track with a semi-micro-electrode and step by step high frequency stimulation, then replaced by a quadripolar electrode once we located the site enabling the optimal clinical improvement. Stereotactic x-rays localised the final position of the electrode. A company software matched sMRI, ventriculography, and peroperative (perop) x-rays, with reference to the stereotactic location boxes. We analysed the effects of acute (perop) and chronic (six-month follow-up) stimulation of active plots (acplots), i.e. leading to optimal clinical improvement. Three distances with reference to the acplots were measured both on sMRI and ventriculography: the laterality from the median sagittal plane of the third ventricle; the anterior position from the midpoint of the intercommissural line (Icl), and the vertical position with regard to the Icl. We then compared the differences in measurements (n = 64) with the Bland and Altman method. The mean difference was 0.09 mm with 95% of the values between +/- 1 mm, but only the laterality had a statistically significant agreement (all the differences included between +/- two times the standard deviation of the mean). The acplots distances from the dorsal, ventral, and medial boundaries of GP (defined by manual surrounding on frontal and horizontal planes) were measured on sMRI. With one exception, the acplots were all included in the nucleus. The six-month acplots were located dorsally with reference to the perop ones. Clinical benefit at six-months follow-up showed results comparable to the literature. Direct location of GP target based on sMRI seems a simple and reliable method.
The authors report the results of a retrospective study of 158 lumbar spinal stenosis (LSS), all operated (111 degenerative, 26 congenital, 21 mixed). Eighty seven percent of the patients had a low-back pain and 81.6% a radicular pain. Only 57.6% of them had a polyradicular claudication. A neurological deficit (motor, sensitive, or involving sphincters) was present in 36.6% of cases. A myelographic block was noted in 23.4% of cases, and in 20.3% a spondylolisthesis with an intact neural arch was found. Surgery consisted of a posterior lateral spinal canal calibration, sometimes associated with a ventral canal calibration (via the posterior route) (6.3%), and/or excision of a disc herniation at one (47.5%) or two levels (3.8%). Mean follow-up after surgery was 7.8 months. The global result was good or excellent in 75.2% of cases. Radicular pain was relieved in 89.1% of cases, and polyradicular claudication in 90.1% of cases. Neurological deficit improved in 50.6% of cases. In only 59.8% of cases relief of low-back pain was achieved. Statistically low-back pain (lasting for over 2 years) improved less, but a preoperative spondylolisthesis didn't influence the quality of the result regarding this symptom. Semiology, pathophysiology, and surgery particularly regarding spine stability are discussed.
Progressive cervical myelopathy due to an ossification of the posterior longitudinal ligament (OPLL) appeared in a sicilian women. Diagnosis was made by lateral conventional tomograms and CT Scan with mielography. Corporectomy of the 5th vertebra and ablation of the ligament dramatically improved the clinical symptoms. If OPLL seems a common cause of cervical myelopathy in Japan, it is very rate in Caucasian people. Instead of the origin of the patients, progressive tetraparesis of the 60 years old people is the usual presentation of this affection. The cause of OPLL remains unknown: genetic factors, vitamin A, association with diffuse idiopathic skeletal hyperostosis, calcium metabolism abnormalities are discussed. As far as surgical treatment is concerned, choice between anterior and posterior decompression is clarified on the basis of the type and the extension of OPLL.
A microcomputer program to analyse ICP signal in the frequential domain is described. It allows the detection and quantification of especially slow pressure waves (A, B, and C). 212 ICP data recordings were analysed with our software, and gave pertinent frequential informations. The status report and lessons learned are discussed.
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Single units were recorded using extracellular glass microelectrodes in all laminae of the superior colliculus of the rat under halothane nitrous oxide anaesthesia. Fifty-one units were encountered which responded to a low intensity mechanical stimulus applied to a contralateral or bilateral field located in the oral sphere (intraoral 11, perioral 16), on the face (29) or on the rest of the body (21). Sixteen units responded to a jaw movement. Sixty-one cells were recorded which were preferentially (10) or only (51) activated (30) or inhibited (21) by noxious stimuli. Contralateral or bilateral mechanoreceptive fields located in intraoral (34) and perioral (35) areas were frequent. There is therefore a high incidence of the nociceptive representation of the mouth in the superior colliculus. The other functional properties of the nociceptive units were similar to those reported in other studies. From the subsequent histological localization of the recorded units, it appeared that the nociceptive projections from the intraoral and perioral regions to the superior colliculus reach the lateral part of the intermediate and deep layers of the superior colliculus.