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Li-Fraumeni syndrome is a rare autosomal dominant cancer-prone condition characterized by the occurrence of a large set of different types of cancer in a patient and their family. A germline disease-causing mutation of the gene encoding the p53 protein is associated with the syndrome. We report on a family in which segregation of a TP53 mutation in two generations was associated with two brain tumours, a leiomyosarcoma and a thyroid carcinoma in four male patients. The main patient presented with seizures revealing several primary brain tumours. We review recent views on its molecular basis and discuss management of the condition as well as a review of the literature., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Intraoperative imaging, in particular intraoperative MRI, is a developing area in neurosurgery and its role is currently being evaluated. Its role in epilepsy surgery has not been defined yet and its use has been limited. In our experience with a compact and mobile low-field intraoperative MRI system, a few epilepsy surgeries have been performed using this technique. As the integration of imaging and functional data plays an important role in the planning of epilepsy surgery, intraoperative verification of the surgical result may be highly valuable. Therefore, teams that have access to intraoperative MRI should be encouraged to use this technique prospectively to evaluate its current relevance in epilepsy surgery.

Background and Purpose: Optimal treatment for low-grade glioma remains controversial. Moreover, though surgery is recommended for disease management, evidence is lacking concerning the appropriate extent of surgery and the use of adjunctive therapy. Available data is basically retrospective, coming from series with substantial limitations. We reviewed our institution's series in order to evaluate the efficiency of surgical management and the influence of the extent of surgical removal for patients with low-grade glioma., Methods: Data were collected from a series of 201 patients who underwent first-intention therapy for low-grade glioma, the standard practice in our institution between 1994 and 2005. After applying certain exclusion criteria, we retained for analysis 123 patients with grade II glioma (WHO classification). We compared progression-free survival curves for the three surgical treatment groups defined as biopsy, partial removal, or total removal., Results: Statistical evaluation of the progression free survival shows a benefit in total surgery as a first intention treatment. No statistical significance was demonstrated between partial surgery and stereotactic biopsy., Conclusion: For patients with low-grade glioma we recommend total surgical removal as first intention management.

Intramedullary cavernomas are rare, but with routinely use of MRI detection has improved, raising the problem of choosing the adequate management approach: conservative or surgical. Cavernomas are vascular malformations, but, as hemangioblastomas they appear as vascular tumors of the spinal cord. They can be durably asymptomatic. The symptoms are a progressive clinical deterioration or acute spinal dysfunction (tetra or paraplegia) in case of hemorrhage. Cavernomas have a typical aspect with MRI in contrast with intramedullary gliomas. The lesion is often superficial, covered by the pia-mater, visible immediately after opening the dura, the approach is direct; but in few cases the cavernoma is deep seated in the spinal cord and not visible, the approach is through the midline. It is recommended to perform a complete "en bloc" resection. A yearly MRI control is necessary to search possible "de novo" cases.

A brief history of the most current scales of aneurysmal subarachnoid hemorrhage and follow-up is presented. Advantages and inaccuracies of these scales are discussed. The World Federation of Neurological Surgeons (WFNS) classification is recommended as the most objective and reliable although some critics exist about its use in particular conditions. The grading of the follow-up is also analyzed. Here, the Glasgow Outcome Scale (GOS) is the most common employed and promoted in a first approach in spite of its briefness. Secondary functional and neuropsychological examination at 6 or 12 months is to be recommended to enable a more accurate evaluation. In conclusion, the WFNS scales for subarachnoid hemorrhage and follow-up is proposed as the best way to allow comparison between work of different centers.

Introduction: Appropriate evaluation of resection remains one of the major difficulties of surgical treatment of pituitary adenoma. The transsphenoidal approach does not allow direct visual control. Endoscopy provides useful information but may no distinguish well residual adenoma from the pituitary gland. Intraoperative MRI offers new perspectives for assessing the quality of resection. We report our experience with low field intraoperative MRI in surgical treatment of pituitary adenoma., Population: Intraoperative MRI (Polestar N10, 30 patients and Polestar N20, 17 patients) was performed in 45 consecutive patients undergoing surgery for pituitary adenoma. Thirty-seven patients had a macroadenoma. Patients were in the prone position with the head fixed with a three-pin MRI-compatible headholder., Method: Coronal T1 MRI scans with enhancement were acquired pre and per operatively. We compared scans and surgical filling (complete removal). If there was a difference, a surgical control was undertaken., Results: Intraoperative images were unavailable for two patients due to small size of the neck and the pituitary glands which were not in the middle in the field of view. For the others, the pituitary glands were in the field of view and the intraoperative scans could be used for comparison. For four patients, there was a discrepancy between surgeon filling and the intraoperative MRI. A control showed no residual adenoma but hemostatic tissue., Conclusion: Low field intraoperative MRI is an excellent technique for controlling the size of pituitary adenoma resection.

Background and Purpose: Management of patients with low-grade glioma is a major challenge for the neurosurgeon. When is neurosurgery indicated? Should chemotherapy or radiotherapy be used? Many questions without an answer. We reviewed our experience with 65 patients treated for low-grade glioma who had preoperative PET images (FDG or/and MET). We examined the prognostic value of PET and also determined the sensitivity and the specificity of PET images to predict outcome., Methods: Sixty-five patients with a FDG or MET PET images were analyzed. We used two visual scales and had complete follow-up data for 63 patients. The free interval was the principal criterion for statistical analysis. The sensitivity and the specificity of PET images was determined., Results: Strong FDG uptake was correlated with a short free interval (p=0.001). Similar results were found with the MET analysis (p=0.0076). We had a PET with MET and FDG for 36 patients. The sensitivity was 66% and the specificity 94% for FDG PET. Sensitivity was 100% and specificity 53% for MET PET., Conclusions: PET imaging provides a prognostic factor independent from histology. MET PET is the best exam for the follow-up of patients with low-grade glioma and is helpful for separating aggressive from low-grade glioma.

Background and Purpose: Different approaches to the pineal region are described in the literature with high mortality and morbidity. We report our experience with two different approaches (occipitoparietal or infratentorial supracerebellar approaches) for pineal region., Methods: Between 1986 and 1997, 25 operations were performed in 22 patients. Preoperative work-up included a CT-scan with conventional angiography (for older patients), magnetic resonance imaging with angiographic study (angio-MRI) for the others. Two different approaches, the occipitoparietal (n=14) or the infratentorial supracerebellar (n=8) ones, were used. Three patients had a stereotactic biopsy, followed by a total resection for one of them., Results: The mortality and morbidity induced by the approaches were low compared to the series of the literature. Only 2 patients were worsened by the surgery: Patient 4 developed hemianopsia after an occipitoparietal approach and patient 21 temporary Parinaud syndrome after resection of a teratoma by supracerebellar infratentorial approach. One patient improved his neurological status but died on the 10th postoperative day from pulmonary embolism., Conclusions: Total or partial resection of well-defined pineal region lesions by direct neurosurgical approaches can be achieved in most cases with low morbidity. The choice among the two surgical occipitoparietal or infratentorial supracerebellar approaches depends on the size and the location of the lesion in the pineal area, its relation to the ventricular system, the median line and the splenium. Angiographic sequences in magnetic resonance with study of the deep veins are helpful in this respect.

Postoperative cerebellar hemorrhage after a supratentorial craniotomy represents a rare event. We report a case of a patient with a meningioma of the jugum who developed suddenly after surgery a neurological deterioration due to a cerebellar hemorrhage detected on the CT scan. An occipital craniectomy and an external ventricular drainage were performed in emergency. A complete neurological recovery was observed after surgery. Nineteen similar other cases are found in the literature. Pre- and postoperative high blood pressure, lowered intracranial pressure and mispositioning of the head during surgery could be at the origin of the hemorrhage. Size of the hemorrhage, time between diagnosis and treatment represent two prognostic factors. All patients, who present a neurological deterioration in postoperative course, must have CT scan including posterior fossa.

Primitive malignant rhabdoid tumor of the central nervous system are extremely rare. Less than 70 cases are reported in the literature. This tumor grows during the first months of life and prognosis is very poor. We report a case of primitive malignant rhabdoid tumor in a 17-year-old young adult. To our knowledge it is the first such case reported in the literature. We review the clinical feature, the neuradiological and therapeutical aspects.

Background and Purpose: In 1964, the first case of "de novo" aneurysm has been reported by Graf and Hamby. The risk of late formation of aneurysm is unknown. Some factors could influence risk: history of hypertension, oral contraceptives, cigarette smoking. We report a rare case of "de novo" aneurysm associated with Ehlers-Danlos syndrome., Results: A 35-year-old female developed a subarachnoid hemorrhage (SAH). Angiography demonstrated 4 aneurysms. Five years before, she presented a SAH. At that time, four vessels angiography demonstrated only one PICA aneurysm. A Ehlers-Danlos syndrome was suspected, which was demonstrated on skin biopsies., Conclusion: At our knowledge, this is the first case of "de novo" aneurysm associated with a Ehlers-Danlos syndrome.

Background: A few cases of brain metastasis from gynecological cancer have been reported. This tumor usually dissaminates via the blood stream, normally through surface implantation or lymphatic spread., Purpose: We report four cases of brain metastases from gynecological cancer (2 from ovarian cancer and 2 from endometrial cancer)., Results: Several months later, three patients developed brain metastases without any other location elsewhere. For the other patient, brain metastasis was the first sign of her malignant disease., Conclusions: In brain metastases from gynecological cancer, treatment should consist in surgical resection, radiation therapy and chemotherapy.

A rare case of sphenoid sinus osteoma associated with a mucocele is presented. Progressive unilateral amaurosis is the only clinical sign. Subtotal resection of the tumor had been realised through a frontal extra-dural approach. The operative indications are discussed.

For this study, we reviewed a series of 564 consecutive SAH among which 72 cases (12.7%) were older than 65 years. Etiology of SAH was: arterial aneurysms (65), arteriovenous malformations (1), unknown origin (6). Among the 65 aneurysms, 28 operated cases were between 65-69 years old. One year after surgery, the results are: GOS 1 and 2: 22 cases (57%); GOS 3: 4 cases; GOS 5: 13 cases. In the non operated group, 19 cases died (73%) and among the alive patients, one was GOS 2 (4%), 5 GOS 3 and 1 GOS 4. In the operated group, mortality is similar to other published series. In the non operated group, mortality is unsurprisingly higher (73%) than in the operated group, but also than in the literature. An explanation could be found with analyses of the income of our patient whose many are not referred cases from other hospital. Our analysis confirmed that the severity of SAH after 65 years is increasing. The raise of anterior communicating aneurysms location is demonstrated. Operative indication and timing are dependent of neurological grading, general condition and secondarily of the location and anatomy of the aneurysm.

Traumatic aneurysms constitute a very small percentage of all intracranial aneurysms. A high mortality results of a catastrophic rupture and they run an unpredictable course. Traumatic aneurysms usually involve the large basal arteries or the middle meningeal arteries. We report a case of an association of a traumatic aneurysm with a congenital aneurysm located on the same middle cerebral artery. At our knowledge, it is the first described case of the literature.

Surgical treatment of pineal-tentorial region lesions remains a challenge. The difficulty in approaching the pineal region can be verified with the number of operative plans that have been proposed to reach this area: transcallosal, occipital transtentorial, infratentorial supracerebellar approaches and sitting, prone or Concorde positions. This emphasizes the surgeon's dissatisfaction with the surgical techniques described. Recently, a three-quarter prone position with the bone flap placed under the midline has been described (1, 3, 8). We have decided to test this approach that we have slightly modified and we report our results on 13 cases: 2 arachnoid cysts, 3 vascular malformations and 8 tumors (3 brainstem gliomas, 2 dysgerminomas, 1 quadrigeminal plate metastasis and 1 meningioma plus 1 metastasis of the falx). Keeping the table in a horizontal plane, risks of air embolus are eliminated. Using the natural effect of gravity, traction on the occipital lobe is no more necessary and hemianopsia no more occurs. We recommand the parieto-occipital route which is the shortest way to reach epiphysis and falco-tentorial notch. We confirm the results of american colleagues (1, 3, 8, 15) and we advise to use this approach which seems to us the best way to treat pineal-tentorial lesions.

Fifty-nine subarachnoid hemorrhage with normal panangiography were reviewed (1982-1989). The mean age is 50 + 10. The grades at admission are compared following the Hunt and Hess and the World Federation Classification. Fifty cases (Hunt and Hess) or 45 (W.F.N.S.) are grade I or II. Compared with 278 cases of subarachnoid hemorrhage of aneurysmal etiology, 76% were in good grades versus 55% only for the aneurysms. Hypertension was present in 21 cases (35%) and represented a factor of gravity. Hydrocephaly is a rare complication in these cases and only 2 cases were shunted. The repetition of angiographic exploration seems to be unnecessary: all the examens remained negatives. The aspect on C.T. Scan was the same that the subarachnoid hemorrhage of aneurysmal origin. Angiography of 4 pedicles were performed in all cases and in the late 15 cases, external carotid explorations were added for exclusion of dural malformations. Seven suspected cases had had a secondary exploration between 12 days. The other cases were reexamined at 3 months. Three cases deceased from a second hemorrhage without aneurysm at autopsy. These cases confirm that subarachnoid hemorrhage of unknown etiology is of good prognosis. Low grades, rare complications, few rebleeding and good outcome scale are common. Etiology remains hypothetic.

The intrastriatal injection of the excitotoxin quinolinic acid (Q.A.) in rats produces neuroanatomical and neurochemical changes mimicking those appearing in the striatum in Huntington's disease (H.D.). Although its cause is unknown, it has been hypothesized that the neurodegenerative changes seen in H.D. may result from the action of an endogenous toxin. Therefore, the development of new strategies for limiting or preventing Q.A.-or other neurotoxic-induced degeneration may be of therapeutic interest for neurodegenerative disorders. Accordingly, we tested the ability of various tissue transplants to protect the rat striatum against a subsequent Q.A. insult. Using a "unilateral model", i.e. unilateral intrastriatal grafts followed by an ipsilateral intrastriatal injection of Q.A., we were able to quantify a behavioral protective effect of the grafts in recording the apomorphine-induced rotational behavior that normally appears after the striatal Q.A.-induced lesion. Our results show that one of the tested tissue, fetal striatum, protects the recipients against the lesioned-induced rotational behavior that appeared in non-grafted lesioned animals. The other grafted tissues (adrenal medulla, peripheral nerve, adipose tissue) seemed to provide a less dramatic protection than fetal striatum; however, this difference did not reach significance. Quantification of the striatal neuronal loss showed that the behavioral protection is significantly correlated with a better neuronal survival in the grafted animals. These results suggest that intracerebral grafts can protect the host brain against a toxin-induced damage, like the one resulting from Q.A. intrastriatal injection. Though fetal striatal grafts seem to exert an optimal protection, this protective effect may at least partially result from a host-mediated response to the transplantation procedure. The mechanism underlying this protective effect is unclear, but the present data suggest that it might be related to a transplantation-induced astroglial reaction resulting in an increased neuronotrophic activity that could protect against the toxic effect of Q.A. The results of this study also support the concept that the effect of transplantations could occur through processes other than a direct restoration of deficient transmitters or a reconstruction of damaged pathways. Further characterization of the factors implicated in the present paradigm might conceivably open avenues for possible therapeutic preventive interventions in neurodegenerative disorders.

In cases of solitary brain metastasis of lung cancers other than small cell lung carcinoma, which has an even poorer prognosis, long-term patient survival over 1.2 years is rare. One case with a 12 year survival is presented. The authors review the other 18 cases with a minimum 5 year survival described in the literature. It is concluded that surgical aggressivity is justified in these cases.

The authors present the results of the postoperative I.C.P. records in 35 patients. The results emphasize the relationship between the clinical grading and the degree of the intracranial pressure: 4/7 grades III (Hunt and Hess grading) and all the grades IV (4/4) had an abnormal intracranial pressure. The location of the aneurysm does not clearly appear as a significant factor of increased intracranial pressure in the postoperative period.

Two cases of meningiomas with metastasis are presented. The first case is typically a fibroblastic meningioma. After a local recurrence, dissemination occurs in the CSF with multiples localizations. The second case is a parasagittal meningioma of meningotheliomatous type. After two recurrences, a lymph node metastasis is discovered. Three modes of dissemination are known: through the blood vessels, the lymphatics or the CSF. The different spreads and mechanisms are discussed.

Operative findings of degenerative changes in pituitary adenomas are not uncommon; however, clinical apoplexy is rare. We report the case of a 43-year-old man who presents a sudden right hemiplegia with aphasia and right ophthalmoplegia, in relation with an ischemic pituitary apoplexy and cerebral vasospasm. A few cases of arterial obstruction or vasospasm associated with pituitary apoplexy have been reported in the literature. Cerebral arterial spasm has also been observed after pituitary surgery. Inclusion of blood or necrotic material in the subarachnoid space seems not to be the only mechanism of vasospasm. The role of hypothalamic dysfunction is considered.

The authors present, among 200 intracranial arterial aneurysms, 13 cases where the angiographic diagnosis of the aneurysm had been difficult. Among them, there were ten mistakes through default, and four mistakes through excess. Angiographic studies were done early, about the third day following subarachnoidal hemorrhage, so that conscious patients may have rapid surgery. The main diagnosis defaults were observed in aneurysms of the anterior communicating artery (cases 1, 2, 3, 4, 6), where the flexurae and arterial anomalies explain the diagnostic mistakes; double anterior cerebral artery, median anterior cerebral artery of Lazorthes, early origin of the frontopolar artery or of the callosomarginal artery. The aneurysms of the posterior wall of the carotid bifurcation, were hidden by this artery in the A.P. view and by the middle cerebral artery in the profile view (cases 5 and 7). Oblique views are necessary for the two localizations of these aneurysms. The middle cerebral aneurysms (case 8) may be hidden by a bony superposition. The Ziedses des Plantes subtraction method obviates this diagnostic default. Aneurysms of the peripheral branches of cerebral arteries (case 9) are filled later and slightly. Aneurysms of an artery less than 3 mm wide (posterior communicating artery, lenticular artery) are difficult to discriminate from their enlarged infundibulum (case 10). The small size of the aneurysm is not the only explanation of the difficult diagnosis. Indeed, few aneurysms are not evidenced by the first angiography, done at an early stage, even if any spasms exist. Only repeated angiographies could show the aneurysm particularly in young people. We repeat the angiography 15 to 20 days after the first one, and even some months later. In two young patients, angiographic studies were repeated a few times in three years and were normal; but after four years in one case and five years in the other, a new subarachnoidal hemorrhage occurred, and only then was the aneurysm shown by a new angiography (cases 4 and 5). In our series, the angiographic spasm could not afford an explanation for the diagnostic default. This, soon after the subarachnoidal hemorrhage, might be explained by the hemostatic clot which compresses or plugs the aneurysm. Further, the clot's lysis occurs, and the aneurysm can be evident. A double conlcusion must be drawn: --first, the angiography must be repeated if the image is dubious; the angiographic technique must be perfect and varied (oblique view, subtraction method, enlargement technique). Mistakes through excess, leading to useless operations, are as dangerous as mistakes through default; --secondly, great care should be exerted when reading and interpreting the X-ray films in the post-operative period in order to evaluate the surgical results, as well as in the preoperative period.