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Biomedical subjects

J C Marchal

Publications and source records attributed to J C Marchal.

At least 19 recordsLinked to original sources

Germinoma located in the basal ganglia in an 8-year-old girl.

CASE REPORT: The authors describe a unique case of an 8-year-old girl with a germinoma located in the left basal ganglia. Medical history begins 5 months before with a central diabetes insipidus, loss of weight (5 kg at admission), vomiting and asthenia. Computed tomography (CT) and cranial magnetic resonance imaging (MRI) are performed and demonstrate a left basal ganglia tumour (nucleus lentiformis). Diabetes insipidus is considered as non-visible germinoma localization on the pituitary stalk rather than as a possible consequence of peri-tumoural oedema surrounding the hypothalamus. Spinal MRI is normal. Neurological as well as general examination is normal. DISCUSSION: The first hypothesis is low-grade glioma, but pathological examination following a stereotactic biopsy of the lesion reveals a cerebral germinoma. A few days before the biopsy, the girl experienced a mild left facial palsy, and CT scans at the time of biopsy reveals an intra-tumoural haemorrhage. Alpha fetoprotein and human chorionic gonadotrophin were negative as blood and cerebrospinal fluid markers, whereas placental alkaline phosphatase was positive on immunohistochemical profile of the tumour samples. Dedicated chemotherapy, followed by focal irradiation (40 Gy, 30 sessions, 45 days; SIOP CNS GCT 93 protocol), is performed with a complete response. The outcome is good (Glasgow Outcome Scale=I), without any cognitive impairment and the persistence of a mild facial palsy and a slight right arm dystonia on last neurological examination. There is still no evidence of tumour recurrence.

Antineoplastic Agents↗

Nano-alpha-Al2O3 by liquid-feed flame spray pyrolysis.

Nanometre-sized particles of transition (t)-aluminas are important for the fabrication of high-quality alumina ceramics. Multiple tons are produced each year using a variety of gas-phase processes. The nanoparticles produced by these methods consist mainly of the undesired delta phase with some gamma- and theta-Al(2)O(3). Nano-t-aluminas should provide access to dense nano/submicrometre-grained alpha-Al(2)O(3) shapes offering significant advantages over micrometre-grained shapes. Unfortunately, polymorphism coupled with the high activation energy for nucleating alpha-Al(2)O(3) greatly impedes efforts to process dense alpha-Al(2)O(3) with controlled grain sizes, especially for submicrometre materials. Typically alpha-Al(2)O(3) nucleation within t-aluminas is sporadic rather than uniform, leading to exaggerated grain growth and vermicular microstructures without full densification (5). Thus, production of quantities of nano-alpha-Al(2)O(3) from multiple nano-t-aluminas for seeding or direct processing of alpha-Al(2)O(3) monoliths could greatly change how alpha-Al(2)O(3) components are processed. We report here that liquid-feed flame spray pyrolysis of nano-t-aluminas converts them to dispersible 30-80 nm alpha-Al(2)O(3) powders (50-85% phase transformed). Surprisingly, the powder surfaces are fully dehydrated. These powders pressureless sinter to more than 99.5% dense alpha-Al(2)O(3) with final grain sizes < or =500 nm without sintering aids.

Journal Article↗

Neurosurgical concepts and approaches for orbital tumours.

Orbital tumours are lesions that appear within the orbital craniofacial borders. To this end, treatment of these tumours is assured by teams of different specialists. Furthermore, these pathologies are different in adults and in children. We have endeavoured, in this chapter, to highlight the specifically neurosurgical features of orbital tumours or, to be more precise, tumours affecting the posterior two thirds of the orbit and tumours originating in or intruding into the optic canal. The list of aetiologies is long. After recapitulating the main types of tumour (as well as those of most concern), we have also studied the different stages of surgery, namely approaches and reconstructions which we have illustrated at each stage by a tumour that, in our view, seemed emblematic of the problem in question: the lateral eyebrow approach for schwannoma and cavernous angioma, the transorbital subfrontal approach for optic nerve glioma, the pterional and orbital approaches for spheno-orbital meningioma, problems with reconstruction and with plexiform neurofibroma affecting the orbit and fibrous dysplasia of bone.

Adult↗

Individualized treatment of craniopharyngioma in children: ways and means.

BACKGROUND: Medium- and long-term prognosis of craniopharyngioma is overwhelmed by the risks of hypothalamic and visual impairment. This problem has been underestimated for a long time because the major concern for the neurosurgeon was the risk of recurrences, their best prevention being thought to be complete tumour resection. Today, we know that radical surgery not only is not an absolute guarantee against recurrences but also can cause hypothalamic and visual complications. METHODS: The authors suggest that instead of complete removal, the first choice treatment should be, when possible, a less aggressive, multistaged and personalized treatment. In this perspective they focus on other therapeutic methods: endocavity treatment of cysts with rhenium-186, triconformational radiotherapy, radiosurgery, and widespread use of the trans-sphenoidal approach. CONCLUSIONS: These simple methods should reduce the risks of visual aggravation and metabolic syndrome.

Child↗

Thalamic ependymal cyst presenting with tremor.

CASE REPORT: We report the case of a 6-year-old boy presenting with intention tremor. CT and MR scanning showed a large thalamic cyst. TREATMENT: Stereotactic tapping of this cyst was carried out as the first step in surgical management. Following drainage of the cyst the tremor disappeared, but recurred 18 months later. Repeat MRI showed that the thalamic cyst had refilled. As the initial tapping of the cyst had resulted in effective but only transient clinical improvement a cysto-peritoneal shunting procedure was performed. OUTCOME: The clinical symptoms again disappeared and the child has now remained tremor free for 3 years.

Central Nervous System Cysts↗

[Methylation status of RARbeta gene promoter in low and high grade cerebral glioma. Comparison with normal tissue. Immuno-histochemical study of nuclear RARbeta expression in low and high grade cerebral glioma cells. Comparison with normal cells. 48 tumors].

Retinoic acid receptor beta (RARbeta) is a nuclear receptor often deregulated in tumors. An immunohistochemical study was conducted to examine the level of expression of this receptor in the nucleus of glial cell tumors (low and high grade glioma) as well as a study of the methylation status of the gene promoter coding this receptor on the same tumor samples. A comparison with normal tissue was done each time. 48 tumors were eligible for the study (15 glioblastomas, 20 grade III oligodendrogliomas and 13 grade II oligodendrogliomas). A constant decrease of RARbeta expression was found by comparison with normal tissue whatever the histological grade of the tumor, suggesting a deregulation of RARbeta gene expression. Methylation of RARbeta promoter gene was a rare event (12.5% of all cases), except for grade III oligodendrogliomas (20%), and is thus not a major event of this gene deregulation. Other reasons of this deregulation of RARbeta should be studied, such as loss of 3p24 heterozygoty, mRNA studies and RARbeta interactions with other retinoid receptors.

Brain↗

Acute spontaneous hemorrhage after embolization of brain arteriovenous malformation with N-butyl cyanoacrylate.

PURPOSE: To investigate factors predisposing to spontaneous hemorrhage in the early period after embolization of brain arteriovenous malformations (AVMs). METHODS: The authors report 15 cases of spontaneous hemorrhage in a consecutive series of 492 brain AVMs totally or partially treated with intranidal injections of a polymerizing mixture, from 1984 to June 1998. Retrospective analysis of the records was performed with special attention to the angio-architectural features before embolization, details of embolization procedures and induced angiographic modifications. RESULTS: Pre-embolization features of greater incidence were the presence of steal phenomena (87%), multiple feeding arteries (100%), a compact aspect of the nidus (93%), and lobar topography (87%). In most cases the volume of injected glue exceeded 1 ml (80%), venous embolization was significant (67%) and immediate angiographic control showed venous stagnation in and/or around the nidus (80%). Four patients were asymptomatic (incidental discovery on systematic CT at day 3). Among the remaining 11 symptomatic patients, 6 were operated to evacuate an intracerebral hematoma, outcome was good for 4, 5 were left with sequelae and 2 died. CONCLUSION: Spontaneous hemorrhagic complications affected 3.04% of embolized patients. The combination of certain angio-architectural features, significant venous embolization and persistent venous stagnation within the nidus seem to have some predictive value of high risk hemorrhage. In light of this, additional preventive measures must be taken and a neurosurgical team systematically kept on standby.

Acute Disease↗

Chemotherapy for unresectable and recurrent intramedullary glial tumours in children. Brain Tumours Subcommittee of the French Society of Paediatric Oncology (SFOP).

Adjuvant treatment for intramedullary tumours is based on radiotherapy. The place of chemotherapy in this setting has yet to be determined. Between May 1992 and January 1998, eight children with unresectable or recurrent intramedullary glioma were treated with the BB SFOP protocol (a 16-month chemotherapy regimen with carboplatin, procarbazine, vincristine, cyclophosphamide, etoposide and cisplatin). Six children had progressive disease following incomplete surgery and two had a post-operative relapse. Three patients had leptomeningeal dissemination at the outset of chemotherapy. Seven of the eight children responded clinically and radiologically, while one remained stable. At the end of the BB SFOP protocol four children were in radiological complete remission. After a median follow-up of 3 years from the beginning of chemotherapy, all the children but one (who died from another cause) are alive. Five patients remain progression-free, without radiotherapy, 59, 55, 40, 35 and 16 months after the beginning of chemotherapy. The efficacy of this chemotherapy in patients with intramedullary glial tumours calls for further trials in this setting, especially in young children and patients with metastases.

Antineoplastic Combined Chemotherapy Protocols↗

[Endovascular treatment of vasospasm].

Cerebral vasospasm remains a leading cause of heightened morbidity and mortality rates following aneurysmal subarachnoid hemorrhage despite the apparent benefit of recent medical therapeutics. Successful resolution of medically refractory angiographically demonstrated vasospasm with concomitant reversal of delayed neurological deficit has been observed after balloon angioplasty. Subsequent reports confirmed these encouraging results but also emphasized the limitations of the technique and the risks of complications. Intraarterial papaverine infusion has been performed for the treatment of diffuse cerebral vasospasm with controversial results and has also been combined with angioplasty either to facilitate balloon navigation or to treat arteries inaccessible to balloon catheterization. All these different endovascular approaches explain the confusion existing about the indications, timing and efficacy of the endovascular treatments. This article reviews several clinical and experimental studies dealing with these questions.

Angioplasty, Balloon↗

[Intrasellar epidermoid cysts].

Two patients presenting with an intrasellar epidermoid cyst and operated on through a transsphenoidal approach, are reported. Problems regarding pathological controversies about the differential diagnosis (particularly craniopharyngiomas) are addressed. In order to establish the adequate preoperative diagnosis, the interest of MRI is discussed.

Adult↗

[Dysembryoplastic neuroepithelial tumors. Analysis of 16 cases].

OBJECTIVES: We report on 16 cases of dysembryoplastic Neuroepithelial tumor (DNT) treated in the Nancy University Hospital from 1987 to 1997. PATIENTS AND METHODS: There were 9 males and 7 females. Mean age at onset of symptoms was 9.5 years (range: 3 months to 29 years) and the mean age at surgery was 16 years. Nine patients experienced partial complex seizures, 5 patients generalized seizures, 1 patient partial seizures with secondary generalization and one patient ataxia. The diagnosis of DNT was made under consideration of clinical, radiological and neuropathological features. All patients underwent surgery. RESULTS: Removal of the tumor was complete for 10 patients, subtotal or partial for 6 patients. Histological examination revealed that 7 cases were specific forms of DNT due to the presence of the specific glioneuronal element. For the 9 remained cases, the diagnosis of DNT could only be made with the consideration of clinical and radiological features. Mean post-surgical follow-up was 3.5 years (range: 1-8 years). Eight patients were seizures-free, 7 had a significative reduction in seizures frequency with minimal anti-convulsivant treatment. The patient with ataxia remained unchanged. For the patients with partial resection of the tumor, follow-up MRI and CT scan showed no significant growth of the remnant and the remaining patients have had no recurrence to date. CONCLUSION: The recognition of this surgically curable entity is mandatory. Knowledge of the good prognosis associated with the DNT is essential to avoid deleterious side effects of overtreatment by radiotherapy and/or chemotherapy.

Adolescent↗

Prognostic factors in pediatric spinal cord astrocytoma.

BACKGROUND: Factors that could optimize the management of pediatric spinal cord astrocytoma remain unclear and controversial. METHODS: To determine the factors that influence the prognosis of pediatric patients with spinal cord astrocytomas, a series of 73 consecutive patients at 13 French treatment centers with histologically proven spinal cord astrocytomas was retrospectively reviewed. Hospital records, operative records, and results of radiologic investigations were available in all cases. Follow-up was achieved in 94% of cases. RESULTS: Seventy percent of the patients had low grade (1 or 2) tumors. Total or subtotal surgical resection was achieved in 43%. Thirty-six patients were irradiated following surgery. Fifty-one patients were alive at a median follow-up of 54 months. Twenty-three patients relapsed. Univariate analysis showed that good outcome was correlated with male gender, age younger than 7 years, duration of presenting symptoms longer than 2 months, the presence of spinal deformities, and low grade histology, whereas sensory loss was associated with decreased survival. Multivariate analysis using the Cox proportional hazards model confirmed that histology (relative risk [RR] = 7.69) and the interval between first symptoms and diagnosis (RR = 4.93) were significant independent prognostic factors. The extent of surgery or radiotherapy had no clear influence on survival. CONCLUSIONS: This review sheds light on the prognoses of pediatric patients with spinal cord astrocytomas and may help to determine therapeutic strategies based on patients' clinical, radiologic, and pathologic features.

Adolescent↗

[Meningiomas of the sellar diaphragm. Apropos of 4 cases].

Since 1987, we have treated four patients with diaphragma sellae meningioma. Tuberculum sellae meningiomas with intrasellar extension were strictly excluded from this retrospective study. A complete tumor removal was performed in two patients. The two other patients underwent post-operative radiotherapy. According to the Kinjo's classification, the four meningiomas were classified as follows: one type A (supradiaphragmatic-prepituitary), one type B (supradiaphragmatic-retropituitary), one type C (subdiaphragmatic), one type not described in this classification characterized by sub and supradiaphragmatic extension. Based on our experience and data in the literature, the clinical and neuroradiological features of the diaphragma sellae meningiomas are reviewed. Diaphragma sellae visualization at MRI, which is not always possible, allows to localize the tumor on a supra- or infra- diaphragmatic position and to decide the optimal surgical approach. The diaphragma sellae is more visible on protonic density, or T2-weighted sequences, but can be located on T1-weighted images. Appropriate surgical approaches are the sub-fronto-pterional route for supradiaphragmatic meningiomas and the transsphenoidal approach for subdiaphragmatic meningiomas.

Adult↗

[An exceptional variety of medulloblastoma: melanotic medulloblastoma].

We report a new case of melanotic medulloblastoma of the vermis in a 3 1/2 year old boy. This tumor showed a typical histological appearance with pseudoepithelial pigmented structures immunoreactive for S100 protein and vimentin. The tumor did not recur after total surgical removal and post operative radiation. However, after a 10 year follow-up, imaging demonstrated that a second tumor occurred in the left cerebellar hemisphere, which, on histological examination, was a typical glioblastoma. Hypothesis concerning the histogenesis of the second tumor, as well as a causal association with radiation therapy and possible contribution of growth hormone therapy are discussed.

Cerebellar Neoplasms↗

Endovascular occlusion of intracranial aneurysms of the posterior circulation: comparison of balloons, free coils and detachable coils in 38 patients.

We treated 38 patients with 39 aneurysms of the posterior circulation by an endovascular technique using balloons, free coils or Guglielmi detachable coils (GDC) from 1986 to May 1993. The patients ranged in age from 10 to 71 years. Subarachnoid haemorrhage was the most frequent presentation (29 patients), followed by mass effect (5 patients) and epilepsy (2 patients). Treatment consisted of embolisation of the aneurysm with preservation of the parent vessel (in 29) or occlusion of the parent vessel (in 5). Multiple procedures were performed in 12 patients (32%, maximum 3 embolisations, total 17 treatments), because of incomplete initial aneurysm occlusion (in 8 cases) or re-opening of the aneurysm (in 9). Treatment could not be achieved in 5 patients. Good to excellent aneurysm occlusion was obtained in 28 patients (72%). Little or no neurological impairment occurred in 31 patients (82%). Complications related to treatment (11 patients, 29%) included 4 cases of transient cerebral ischaemia, 7 of stroke resulting in minimal neurological impairment (in 5), severe impairment (in 1) and one death. There were 6 patients who died, of rebleeding from the same aneurysm (2), basilar stroke (1) and unrelated causes (3). Comparison of the different occlusion techniques showed that the best angiographic results were obtained with balloons (good to excellent aneurysm occlusion was obtained in 17 of 22 patients treated) and the GDC (12 of 13 patients), and that less good results were given by free coils (4 of 9 patients). Complications related to treatment were fewest in patients treated with GDC (1 of 16 treatments, including multiple procedures) or free coils (2 of 12 treatments) and were more frequent with balloons (6 of 27 treatments). All five treatment failures occurred with balloon embolisation, whereas treatment was possible in all cases treated with free coils or GDC.

Adolescent↗

[Subarachnoid hemorrhage caused by aneurysm rupture. Surgery or embolization?].

Traditionally the aneurysms of the circle of Willis have been an indication for neurosurgery. New technologies of endovascular treatment with electrically detachable coils resulted in a different therapeutical concept since four years. A series including 140 patients has been treated in our institution from 1 January 1992 to 31 December 1994, 94 of them presenting with a subarachnoid haemorrhage. Out of these 140 patients, 84 were treated with surgery, 51 with the endovascular technique, five with surgery after incomplete or unsuccessful endovascular treatment. Surgery was indicated in patients presenting early after bleeding, devoid of vasospasm, with a favourable Hunt and Hess grading and in aneurysms located in the anterior part of the circle of Willis. Endovascular treatment was indicated in patients admitted with delay, with severe vasospasm, a poor Hunt and Hess grading and in all aneurysms of the vertebrobasilar arterial network. Age was of less importance in comparison to the status of the vessels for selection of the method of treatment. Giant aneurysms are difficult to treat as surgery is faced with the size of the aneurysmal itself and endovascular technique with the width of the aneurysmal neck.

Aneurysm, Ruptured↗

[Endovascular treatment of intracranial aneurysms].

Major improvements have been achieved in endovascular treatment of intracranial aneurysms during the last twenty years. Initial techniques of selective balloon occlusion were progressively replaced by the packing of the aneurysmal pouch with very small and soft metallic devices, named coils, with controlled detachment. In some rare cases, balloon occlusion is still used in order to occlude the parent vessel or to reverse the flow, especially in the vertebro-basilar system. Essential aims for the anaesthesiologist include: complete immobility of the patient throughout the endovascular procedure, systemic anticoagulation in order to avoid any thromboembolic complication during and after treatment, prevention and/or treatment of vasospasm, precise evaluation of fluid balance during the procedure. Our experience is based on 145 aneurysms in 135 patients, treated by endovascular route; 91 (63%) of them were revealed by a subarachnoid haemorrhage (SAH). Their main locations were: the carotid siphon (33.1%), the anterior communicating artery (19.3%), the basilar artery (17.2%) and the middle cerebral artery (17.2%). Thirty-seven of these aneurysms (25.5%) were very small in size with a diameter less than 4 mm. Total or sub-total (more than 90%) occlusion was achieved in 141 aneurysms (90.3%). Thromboembolic complications occurred in eight patients (5.9%), neurological sequelae in one. Other treatment-related complications included the migration of a coil outside the aneurysm in six cases (4.4%) without neurological deficit in one and five ruptures of the aneurysm (3.7%) during the procedure with one lethal issue due to the spontaneous bleeding of a controlateral arterio-venous malformation. Thirteen patients died (9.6%). The cause of the death was related on the initial SAH in 11 of them. The two others were both due to rupture: of a second untreated aneurysm in one case and of an associated arteriovenous malformation in the second one. At present the main indications of endovascular treatment are poor or non indications for neurosurgery. Due to the quality of results, endovascular therapy will be more and more used in case of ruptured or non ruptured aneurysms.

Cerebral Angiography↗

[Endovascular treatment of vasospasm].

Various therapeutic strategies have been recently proposed to prevent the vasospasm after subarachnoid haemorrhage, and to avoid its clinical consequences. Despite these progresses, mortality and morbidity of delayed ischaemic consequences of vasospasm remain important. Two endovascular treatments have been proposed. The successful use of transluminal angioplasty for vasospasm was first reported by Zubkov in 1984. Transluminal angioplasty is very effective with clinical improvement when the treatment is undertaken without delay after the onset of symptoms. Limitations of this technique are the inaccessibility of distal arteries and the risks (vascular rupture or occlusion). More recently, to overcome these limitations, a selective intraarterial infusion of papaverine has been proposed. These infusions are less risky and can be employed in distal vasospasm. However, clinical results seem to be less favourable and often transient. These two techniques are still used with slightly different indications. According to our experience, it seems reasonable to reserve transluminal angioplasty for symptomatic vasospasm, associated with papaverine or not, and to use papaverine alone in all other cases.

Angioplasty, Balloon↗