Les kystes dermoïdes du système nerveux central sont très rares. La symptomatologie clinique habituelle est dominée par un tableau d’hypertension intracrânienne, épilepsie et atteintes des nerfs crâniens. Le mode de révélation peut être des méningites aseptiques récidivantes.Le but de ce rapport est de considérer le kyste dermoïde dans le diagnostic différentiel des enfants traités pour des méningites aseptiques récidivantes afin d’éviter les erreurs diagnostiques et le traitement inadéquat.Il s’agit de deux enfants hospitalisés au niveau du service de pédiatrie pour des méningites aseptiques récidivantes, dont l’imagerie a été évocatrice de kyste dermoïde.Disparition des méningites après exérèse microchirurgicale.Le diagnostic de kyste dermoïde est à reconsidérer précocement dans les méningites aseptiques afin d’instaurer une thérapeutique adéquate qui est la chirurgie.Dermoid cysts of central nervous system are very rare. The usual clinical presentation is dominated by intracranial hypertension, epilepsy and cranial palsy. The revelation mode could be recurrent aseptic meningitis.The aim of this case report is to consider the dermoid cyst as regards the differential diagnosis in children treated for recurrent aseptic meningitis to avoid misdiagnosis and ice qui a orienté le diagnostic à une méningitnadequate treatment.Two children were admitted in the pediatric department for recurrent aseptic meningitis. The MRI confirmed the presence of a posterior fossa dermoid cyst.Loss of meningitis after microsurgical resection.The diagnosis of dermoid cyst is performed and reconsidered at an early stage in aseptic meningitis in order to establish an adequate therapy, which is surgery.

For suprasellar meningioma, the fronto-basal exposure is considered the standard approach. The superior interhemispheric (IH) approach is less described in the literature.To assess the surgical complications, functional outcome (visual, olfaction), morbidity and mortality rates and late recurrence, after resection by superior IH approach of midline skull base meningioma.Between 1998 and 2008, 52 consecutive patients with midline meningioma on the anterior portion of the skull base (mean age: 63.8 ± 13.1; sex ratio F/M: 3.7) were operated on via the superior IH approach. After a mean follow-up of 56.9 ± 32.9 months, an independent neurosurgeon proposed a prospective examination of functional outcome to each patient, as well as a visual and olfactory function assessment.Fifty-two patients were divided into a group with olfactory groove meningioma (n = 34) and another with tuberculum sellae meningioma (n = 18). The outcome was characterized by postoperative complications in 13 patients (25%), mortality rate in two (3.8%) and long-term morbidity at in 17 (37%) of 50 surviving patients. Based on multivariate analysis, no prognosis factor was significant as regards the favorable outcome. The mean postoperative KPS score (86.6 ± 9.4) was significantly improved. However, dysexecutive syndrome was observed in four patients (8%), hyposmia-anosmia in 34 (68%) and visual acuity deteriorated in one (2%).The superior IH approach could be considered a safe anteriorly orientated midline approach for removal OGM and TSM meningioma.L’approche inter-hémisphérique (IH) supérieure des méningiomes supra-sellaires est peu décrite par comparaison avec la conventionnelle exposition sous-frontale. L’objectif de notre travail était d’analyser les complications chirurgicales, les taux de morbimortalité et l’évolution fonctionnelle des patients traités de méningiome de la portion antérieure de la base du crâne par une approche IH supérieure.Entre 1998 et 2008, 52 patients consécutifs (âge moyen : 63,8 ± 13,1 ; sex ratio F/M : 3,7) ont été traités par voie IH supérieure d’un méningiome olfactif (n = 34) ou du tubercule sellaire (n = 18). Après un suivi moyen de 56,9 ± 32,9 mois, un neurochirurgien indépendant évaluait chez chaque patient un score global fonctionnel en utilisant l’échelle de Karnofski (KPS), leur évolution cognitive par usage de l’index de syndrome dysexécutif (ICDS) et leur qualité de vie (QOL) en appliquant l’échelle de réintégration à une vie normale (RNLI, score maximal de 100). En outre, l’évolution de la fonction visuelle (acuité et champ visuel) après décompression chirurgicale était classée en détériorée, stable ou améliorée. L’olfaction postopératoire évaluée qualitativement était classée en conservée et détériorée. Pour l’analyse uni- et multi-variée de facteurs prédictifs du pronostic, l’évolution était considérée comme favorable lorsque le RNLI était supérieur à 90.Dans cette série, une complication chirurgicale survenait chez 13 patients (25 %), un décès chez deux (3,8 %) et une morbidité à long terme persistait chez 17 (37 %) des 50 survivants. À distance, le score moyen du KPS était significativement augmenté (86,6 ± 9,4) par comparaison avec le score préopératoire de chaque patient. La QOL était inchangé chez huit patients (16 %) et modérément affecté chez 32 (64 %). Par comparaison avec l’état préopératoire, la fonction visuelle était améliorée chez 18 patients (36 %), stable chez 31 (62 %) et détériorée chez un (2 %). La fonction olfactive était altérée ou perdue chez 22 patients (44 %) mais normale chez 28 (56 %). Aucun facteur pronostic d’une évolution favorable n’était déterminé. Une récidive survenait chez quatre patients (4 %).L’approche supérieure IH peut être considérée comme une voie d’exposition sans morbidité surajoutée permettant une résection de qualité des méningiomes de la ligne médiane insérés sur l’éthmoïde et le tubercule sellaire.

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis. It is an inflammatory disorder associated with BRAF V600E mutation in 50% of cases. This multisystem disease is rarely associated with spinal involvement. Neurological involvement is an independent predictive factor of poor prognosis. The diagnosis is histopathological based on CD68-positive and CD1A-negative histiocytes. Treatment with interferon-alpha is an independent predictor of survival in Erdheim-Chester disease and vemurafenib has also been shown to be effective for BRAF V600E mutation. We report a clinical case of a 51-year-old patient with multiple and rare locations of Erdheim-Chester disease, particularly at the sphenoid sinus.

Endoscopy of pineal region tumours has been developed since the year 2000 either via a transventricular or extracerebral approach. The initial purpose of applying neuroendoscopy in the management of pineal region tumours was to resolve the obstructive hydrocephalus, and identify the pathological characteristics of the tumour. Based on this approach, a piecemeal resection of the tumour can be performed. The approaches, derived from the microsurgical pathway using an endoscope to expose the operative field, have been proposed either via an infratentorial supracerebellar approach or posterior transtentorial interhemispheric approach. Neuroendoscopic procedures can be considered as a therapeutic alternative to the microsurgical approach when CSF markers are negative. This procedure is considered mini-invasive for the approach along the surgical corridor access but extensive and in depth at the interface between the tumour and the surrounding neurological parenchyma. The limitations and complications are related to the type of procedure (mono- or bimanual) as well as the tumoral characteristics. Different approaches are presented in detail in order to avoid the occurrence of any surgical complications.L’endoscopie des tumeurs de la région pinéale est proposée depuis les années 2000, que ce soit par voie transventriculaire ou extracérébrale. Au départ, l’objectif était de résoudre l’hydrocéphalie obstructive par perforation du plancher du 3e ventricule. Utilisant cet accès pour réaliser des biopsies, la possibilité de résection tumorale a été rapidement proposée par cette voie transventriculaire. Les approches extracérébrales, dérivées des voies microchirurgicales supracérébelleuses ou transtentorielles permettent de travailler selon une dissection bimanuelle comparable à celle réalisée sous microscope opératoire. Ces procédures considérées comme mini-invasives le sont uniquement pour l’approche réduisant à son strict minimum les dissections tissulaires mais en profondeur, la largeur du champ opératoire en fait une procédure extensive à haut risque fonctionnel pour les structures environnantes. La difficulté opératoire est liée à la procédure selon l’usage de procédure mono- ou bimanuelle mais aussi aux caractéristiques de la tumeur en termes de vascularisation, de consistance, de taille et de risque de dissémination liquidienne de cellules tumorales. Le futur serait un système hybride entre le microscope opératoire et l’endoscopie.

Results of a close prospective follow-up of patients treated for a high grade glioma with or without biodegradable Carmustine wafers but receiving treatment according to Stupp schedule. From May 2007 to June 2008, 65 patients were operated on for a high grade glioma with (28 patients) or without implantation of Gliadel (37 patients). Overall patients received radiotherapy with concomitant temozolomide following or not by 5-day monthly schedule of adjuvant témozolomide. Median of follow-up was 11 months. The two groups were comparable. For overall patients, the median of relapse-free survival was 11 months. Six- and 12-month relapse free survival rates were respectively 80% and 49%. In monovariate analysis, there was no difference of relapse-free survival between patients who received Gliadel and those did not, median relapse free survival and 1-year relapse free survival rates were respectively, 12.9 and 11.7 months and 50.2% and 44% (p = 0.98). However, significant difference of relapse-free survival were shown for pathologic grade (p = 0.0025), age (p = 0.0066) and MGMT methylation status (p = 0.02). In multivariate analysis, only methylated MGMT was a favorable relapse free-survival (p = 0.04, RR: 2.7 CI [1-6.7]). Median of overall survival was not reached. For overall patients, 6- and 12-months overall survival rates were respectively, 92% and 76% (p = 0.12). In monovariate analysis, there was no difference of overall survival between patients who received Gliadel® and those did not, 1-year overall survival rates were respectively, 91.6% and 76.2%, medians of overall survival were not reached for both groups. However, difference of overall survival were shown for irradiation dose (p = 0.014), MGMT methylation status (p = 0.02) and age (p = 0.048). In multivariate analysis, unmethylated MGMT and irradiation dose lower than 60 Gy were unfavorable prognostic factor of overall survival, respectively p = 0.006, RR: 9.8 CI [1.8-51] and p = 0.001, RR: 8,4 CI [2.3-33]. We did not observe difference of peritumor edema volume between the both groups. With Carmustine median GTV plus edema was 84 [27-229] and without Carmustine, it was 68 [10-362], (p = non significant). We observed four cases of thrombopenia, all in the Carmustine Wafers group. Adding Gliadel® before a Stupp schedule for patient with high grade glioma did not decrease survivals.

Stenosis and thrombosis of the cavernous ICA is well known in cavernous sinus meningiomas. Ischemic events following ICA thrombosis are rare because of the slow process of ICA stenosis and the progressive vascular supply provided by the circle of Willis. We report a case of a patient who developed repeated episodes of cerebral infarction due to left cavernous sinus meningiomas with ICA thrombosis and radiation-induced stenotic change of the left A1 segment. A 56-year-old woman with left intracavernous sinus meningiomas presented with a sudden onset of upper right arm paresis that recovered in 24 hours. She was treated 34 months prior to admission with fractionated stereotactic radiotherapy (50.4 Gy). The MRI size of the left cavernous sinus meningiomas was stable compared with the MRI performed before radiotherapy. However, a complete ICA thrombosis was detected. Several lacunar infarcts were identified in the left hemispheric white matter. Angiogram confirmed intracavernous ICA thrombosis and revealed isolated stenosis and irregularities of the left A1 segment immediately above the tumor, suggesting a radiation-induced vasculopathy. There was no filling of the posterior communicating artery. SPECT and measure of the vasoreactivity using perfusion of acetazolamide revealed a reduced perfusion capacity in the left hemisphere. The patient had no history or clinical or radiological signs of heart and vascular disease that could predispose to cerebrovascular disease. An EC-IC bypass between the left common carotid artery and the M2 segment of the left MCA using the radial artery was performed with good patient recovery.

The association of spontaneous intracranial hypotension (SIH) with cerebral venous thrombosis (CVT) is rare. We are reporting two new cases. In one case, CVT is extensive concerning three sinuses and bilateral parietal cortical veins and, in the other case, only a cortical vein is involved. Both the patients presented had no thrombosis risk factors. There is certainly a link between these two pathologies. SIH should be considered as a risk factor of CVT. Modification of symptoms of SIH leading to CVT must be known to start early treatment.