Long Time to Diagnosis of Medulloblastoma in Children Is Not Associated with Decreased Survival or with Worse Neurological Outcome

International audienceBACKGROUND: The long time to diagnosis of medulloblastoma, one of the most frequent brain tumors in children, is the source of painful remorse and sometimes lawsuits. We analyzed its consequences for tumor stage, survival, and sequelae. PATIENTS AND METHODS: This retrospective population-based cohort study included all cases of pediatric medulloblastoma from a region of France between 1990 and 2005. We collected the demographic, clinical, and tumor data and analyzed the relations between the interval from symptom onset until diagnosis, initial disease stage, survival, and neuropsychological and neurological outcome. RESULTS: The median interval from symptom onset until diagnosis for the 166 cases was 65 days (interquartile range 31-121, range 3-457). A long interval (defined as longer than the median) was associated with a lower frequency of metastasis in the univariate and multivariate analyses and with a larger tumor volume, desmoplastic histology, and longer survival in the univariate analysis, but not after adjustment for confounding factors. The time to diagnosis was significantly associated with IQ score among survivors. No significant relation was found between the time to diagnosis and neurological disability. In the 62 patients with metastases, a long prediagnosis interval was associated with a higher T stage, infiltration of the fourth ventricle floor, and incomplete surgical resection; it nonetheless did not influence survival significantly in this subgroup. CONCLUSIONS: We found complex and often inverse relations between time to diagnosis of medulloblastoma in children and initial severity factors, survival, and neuropsychological and neurological outcome. This interval appears due more to the nature of the tumor and its progression than to parental or medical factors. These conclusions should be taken into account in the information provided to parents and in expert assessments produced for malpractice claims

Des résolutions spontanées de syringomyelie (l' expérience de l' hôpital de Bicêtre et revue de la littérature)

Les syringomyélies évoluent le plus souvent vers une aggravation progressive. Cependant, une stabilisation est possible, voire même dans de très rares cas une régression spontanée. Une analyse de 5 cas de résolution spontanée sur une cohorte de 450 patients suivis pour une syringomyélie à l Hôpital de Bicêtre et une revue de la littérature ont été effectuées pour comprendre et caractériser des facteurs de résolution spontanée de syringomyélie. Différents mécanismes peuvent expliquer ce phénomène. Une syringomyélie peut régresser lors de la disparition de l obstacle à la circulation du LCS, la plus fréquente étant une anomalie de la charnière crânio-rachidienne. Des modifications de morphologie de la fosse cérébrale postérieure, lors de la croissance de l enfant notamment ou sous l influence de variations du taux d hormone de croissance, permettent une amélioration de la circulation du LCS au foramen magnum. Un retour à la normale de la pression intracrânienne, diminution d une hypertension intracrânienne ou disparition d une hypotension de LCS, autorise la réintégration des amygdales cérébelleuses dans la boite crânienne et ainsi favorise la régression d une syringomyélie associée. Par ailleurs, d autres hypothèses de résolution spontanée de syringomyélie sont des modifications des pressions intra-kystique ou péri-médullaire et la rupture du syrinx dans les espaces sous-arachnoïdiens.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

Physiopathologie et prise en charge des lésions modullaires rares

LE KREMLIN-B.- PARIS 11-BU Méd (940432101) / SudocSudocFranceF

Clinical Image of a Spinal Ependymoma Discovered 8 Years after Initial Misdiagnosis as an Idiopathic Syringomyelia

International audienceWith the increasing frequency of neuroimaging, incidental intramedullary cavities are diagnosed more frequently. We present a case of asymptomatic incidental intramedullary cervical cavity diagnosed as an idiopathic syringomyelia as initial magnetic resonance imaging (MRI) showed an isolated cystic image without contrasted component. The patient had no subsequent MRI follow-up, but eventually showed symptoms 8 years later. By this stage of the disease, the MRI appearance had changed, showing a solid and enhanced component. The patient underwent surgical resection and histopathology concluded a papillary ependymoma (grade 2). This case illustrates how asymptomatic intramedullary cavities may hide an underlying tumoral process and why these cavities should not be considered as idiopathic syringomyelia by default, except after prolonged MRI follow-up

Microsurgical resection of a radicular hemangioblastoma with subarachnoid hemorrhage: how I do it

International audienceBACKGROUND: Hemangioblastomas (HBL) are benign tumors occurring sporadically or associated with Von Hippel-Lindau syndrome (VHL).METHOD: We present the pre-, per-, and postoperative course of a rare case with radicular HBL presenting with subarachnoid hemorrhage (SAH) in the frame of VHL. We describe the microsurgical approach.CONCLUSION: Complete microsurgical in bloc resection has been performed. Postoperative course was uneventful

Early Detection of Poor Outcome after Mild Traumatic Brain Injury: Predictive Factors Using a Multidimensional Approach a Pilot Study

Mild traumatic brain injury (MTBI) is a common condition within the general population, usually with good clinical outcome. However, in 10–25% of cases, a post-concussive syndrome (PCS) occurs. Identifying early prognostic factors for the development of PCS can ensure widespread clinical and economic benefits. The aim of this study was to demonstrate the potential value of a comprehensive neuropsychological evaluation to identify early prognostic factors following MTBI. We performed a multi-center open, prospective, longitudinal study that included 72 MTBI patients and 42 healthy volunteers matched for age, gender, and socioeconomic status. MTBI patients were evaluated 8–21 days after injury, and 6 months thereafter, with a full neurological and psychological examination and brain MRI. At 6 months follow-up, MTBI patients were categorized into two subgroups according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) as having either favorable or unfavorable evolution (UE), corresponding to the presence of major or mild neurocognitive disorder due to traumatic brain injury. Univariate and multivariate logistical regression analysis demonstrated the importance of patient complaints, quality of life, and cognition in the outcome of MTBI patients, but only 6/23 UE patients were detected early via the multivariate logistic regression model. Using several variables from each of these three categories of variables, we built a model that assigns a score to each patient presuming the possibility of UE. Statistical analyses showed this last model to be reliable and sensitive, allowing early identification of patients at risk of developing PCS with 95.7% sensitivity and 77.6% specificity

Paragangliomas of the spine: a retrospective case series in a national reference French center.

International audienceINTRODUCTION: Primary paragangliomas (PG) of the spine are extremely rare entities. The present study reviews our experience over a period of 30 years.METHODS: This is a retrospective, single center, observational study. Patients surgically treated for a spinal PG with confirmed anatomopathological diagnosis were included. The McCormick classification was used as a reference for clinical evaluation. Follow-up MRI and clinical assessment took place at 6 weeks, 3 months, 6 months, and 1 year after surgery and on yearly basis after.RESULTS: Six cases have been operated in our institution. The mean age was 37.8 (median 35.5, 30-53). The mean follow-up period was 9.6 years (median 9.5, 1-23). Preoperative duration of symptoms varied between a few hours to 4 years. Low back pain was most common sign. One presented with hemorrhage and acute onset of paraplegia. All patients underwent single surgery, with the exception of one case, which had two surgeries on the same anatomical site and a third surgery on another location of the same tumor type. Preoperatively, McCormick scale was I in four cases, and II and IV in one case, respectively. Postoperatively, all patients in McCormick I retained the same class; one patient in McCormick II passed to McCormick III; the case in McCormick IV recovered to McCormick II. Five of eight surgeries achieved total resection, while two surgeries accomplished a partial microsurgical excision and one a gross total resection. Three patients had spinal leptomeningeal dissemination. Two of them benefited from extended spine radiotherapy, while the other of a "wait-and-scan" policy. Spinal leptomeningeal dissemination was stable in all patients at last follow-up.CONCLUSION: We consider surgery as primary treatment in all PG. In our experience, preoperative diagnosis is difficult and caution must be taken to perioperative course in these cases. We do not routinely perform postoperative radiation if there is a residual tumor. We regularly perform clinical and radiological follow-up, so as to be able to document recurrent cases, which have been reported even up to 30 years after primary surgical excision

Prospective Follow-up of Intramedullary Slitlike Cavities: A Consecutive Series of 48 Patients

International audienceObject: Predicting whether intramedullary slitlike cavity (SC) will worsen over time or remain stable is an outstanding clinical challenge. The aim of this study was to identify early features of SC (clinical and magnetic resonance imaging [MRI] findings).Methods: We prospectively included all patients referred to our institution following the discovery of a SC and divided them in two groups: typical SC (defined as a cavity spanning fewer than three vertebrae, not enlarging the spinal cord, and located at the midline between the anterior third and posterior two-thirds of the spinal cord) or atypical SC (all others). Clinical evolution and changes in MRI features were evaluated during follow-up. In some patients, diffusion tensor imaging was performed and cervical cord cross-sectional area was analyzed.Results: A total of 48 consecutive patients were included in the study. The mean follow-up was 58 months. Of the seven patients presenting with deficits at first consultation, two worsened and five remained stable. Of the 41 patients without deficits, seven worsened and 34 remained stable. None of the patients developed severe motor deficits or experienced enlargement of the cavity; 7% of patients who presented with typical SC worsened compared with 35% with atypical SC. The negative predictive value was 0.93 (P = 0.02).Conclusion: Most patients remained stable and a subset of patients developed minor motor deficits. For clinical management, we propose surveillance of patients with a typical SC and close follow-up of those with an atypical SC and/or presenting with deficits

Surgical management of unruptured intracranial aneurysms that are inappropriate for endovascular treatment: experience based on two academic centers.

OBJECTIVE: To analyze the results of the surgical management of unruptured intracranial aneurysms (UIA) when coil embolization (CE) was considered first but deemed inappropriate by our multidisciplinary groups. METHOD: In two institutions, all UIAs recommended for treatment were considered first for a CE procedure if accessibility, neck width, and fundus-to-neck ratio were appropriate. Patients with UIAs considered inappropriate for CE were to undergo a surgical clipping procedure. We reviewed the medical records of all patients who underwent surgical clipping between February 1996 and February 2006. RESULTS: A total of 325 patients with 440 UIAs were treated. Of them, 149 patients were selected by our multidisciplinary staff for treatment by CE, and 176 patients with 238 UIAs were treated by 207 surgical procedures. Angiographic studies revealed complete occlusion in 95% and near total occlusion in 2.5% of surgically treated UIAs. No deaths related to surgery occurred. Sixteen patients (9.1%) experienced postoperative complications, four of which persisted 1 year after surgery (two cases of diplopia and two aphasic disorders). The 1-year morbidity rate was 2.2% (four of 176) by patient and 1.7% (four of 238) by aneurysm. For UIAs smaller than 10 mm in patients younger than 65 years old, the morbidity rate was 0.56%. CONCLUSION: Our results gathered from two centers with the same management of UIAs show that SC remains a safe and effective treatment for UIAs even when CE is considered first