The relevance of the artery of Adamkiewicz for microsurgical resection of spinal tumors- short overview and case series: Technical note.

INTRODUCTION: There is of major interest to know the exact anatomical location of artery of Adamkiewicz (AKA) for many spinal microsurgical procedures, so as to avoid postoperative ischemia of the spinal cord, with further devastating clinical impact. METHODS AND RESULTS: We detail the interest of preoperative angiography for medullary lesions in our experience. We further report two cases where the AKA was located at the same level and side with the treated lesion. The first case underwent an intracapsular decompression. The second patient undertook radiosurgery by Cyberknife. CONCLUSION: For spinal tumor where the AKA is exactly on the same level and side, an intracapsular decompression can be safely performed, with an immediate decrease of the symptomatic mass effect, while decreasing the risk of neurological injury. A second valuable alternative in such situations can also be radiosurgery, for small to medium size tumors. In our experience, this proved safe and effective both for tumor and eventual pain control.

[Cervical spinal sarcoidosis mimicking compressive cervical myelopathy: Poor prognosis after surgery]. / Sarcoïdose médullaire mimant une myélopathie cervico-arthrosique : mauvais pronostic après chirurgie.

INTRODUCTION: Cervical spinal sarcoidosis can mimic compressive cervical myelopathy leading to potentially harmful surgical procedures before the diagnosis can be made. METHODS: Retrospective description of 3 patients and review of the literature. RESULTS: Twenty-seven patients (16 men/11 women), median age 58 years [range 29-74] were described. Neurosurgical procedures consisted of laminectomy (n=10), laminoplasty (n=15) and anterior discectomy (n=2). Immediately after surgery, 17 patients (63%) worsened or remained disabled. Among the 10 patients who improved, 9 worsened secondarily. The analysis of preoperative MRI showed T2 hypersignal lesions and contrast enhancement in all patients. Neurological symptoms were inaugural in 25/27 patients, and systemic involvement of the sarcoidosis was found after surgery in 15/27 patients. After surgery, all patients received corticosteroids, along with immunosuppressive therapy in 8 cases/27. After a follow-up of 24 [16-72] months; 13 patients were stabilized or worsened, 7 were partially improved. Three died of other cause. Only 5 recovered without sequelae. CONCLUSION: In patients with compressive cervical myelopathy, leptomeningeal contrast enhancement, a T2-weighted hypersignal exceeding the compression level on MRI, and the presence of extraneurological symptoms should point to inflammatory disease. These rare manifestations may be the first symptoms of sarcoidosis and should be recognized to avoid harmful surgical procedures and to provide appropriate medical treatment.

[Adult intramedullary gliomas]. / Tumeurs gliales intramédullaires de l'adulte : la série du rapport.

OBJECTIVES: Intramedullary gliomas are rare tumors accounting for less than 4% of all primary central nervous system tumors. The aims of this retrospective multicenter study were to assess their natural outcome as well as management. METHODS AND MATERIALS: We studied 332 patients from 1984 to 2011. Histopathological examination revealed 72% ependymomas (94% were low grade tumors), 24% astrocytomas (29% were high grade tumors), 2.4% mixed gliomas and 1.7% oligodendrogliomas. RESULTS: The mean age at diagnosis was 42.4 years for ependymomas, with male predominance, versus 39.6 years for astrocytomas. Pain was the most common initial presentation. In 20% of cases, astrocytomas were biopsied alone, but more than 80% of ependymomas had surgical resection. Radiotherapy and chemotherapy were reserved for malignant tumors, especially if they were ependymomas. The 5-year survival rate was 76.8% for astrocytomas and 94.5% for ependymomas. Histology, functional status prior to surgery, and tumor grade are among the prognostic factors. CONCLUSION: Our study showed that surgical treatment of gliomas is well codified, at least for ependymomas, but adjuvant treatment continues to play a marginal role in the management even in astrocytomas, which are infiltrative tumors.

[Intramedullary ependymomas: A French retrospective multicenter study of 221 cases]. / Épendymomes intramédullaires : étude rétrospective multicentrique française sur 221 cas.

AIM: Intramedullary ependymomas (IE) are the most frequent intramedullary tumors in the adult population. The gold standard treatment is to obtain gross total removal without any new postoperative neurological deficit. The authors report the results of a multicenter series with a long-term follow-up and the analysis of clinical, radiological, surgical data as well as the complementary treatments. MATERIALS AND METHODS: A retrospective analysis of adult patients with IE operated on between January 1984 and December 2011 at 7 French centers (Bordeaux, Kremlin-Bicêtre, Lille, Lyon, Marseille, Montpellier, Nice, and Nîmes) was performed. The minimal follow-up was 12 months. The clinical evaluation was based on the McCormick classification in the pre and postoperative period at 3 months, 1 and 5 years. RESULTS: Data of 221 adult patients with a pathologically confirmed diagnosis of IE were considered: 134 patients were treated at the Neurosurgical Department of Kremlin-Bicêtre Hospital, 26 were treated at Lille and 61 were treated in the southern region of France (Marseille, Bordeaux, Montpellier, and Lyon). The epidemiological analysis was performed on the entire cohort of patients, while follow-up considerations were made solely on the 134 patients managed at Kremlin-Bicêtre Hospital to obtain homogeneous data. A slight male prevalence was observed (59 % of cases), with an average age of 41.8 years at diagnosis. The mean age at first clinical manifestations was 39.6 years, thus the average duration of symptoms before the diagnosis was 29 months. Neuropathic pain and neurological deficit were the most revealing symptoms in 64 % and 32 % of cases respectively. The localization of the IE was basically cervical in 35.7 %, primarily thoracic in 25.8 % and cervico-thoracic in 22.2 %. The mean tumor length in the sagittal plan was 20.4mm (range 1 to 99mm). A cystic cavity was present in 76.5 % of cases while an intratumoral hemorrhage was detected in 30.3 % of cases. The rate of complete removal was performed in 79.2 % of cases when considering the whole cohort and in 91 % of cases treated at Bicêtre Hospital. In 95 % of cases a WHO grade I or II ependymoma was isolated and in 5 % of cases a WHO grade III. The McCormick scale (MCs) (Lou et al., 2012) [1] was used to rate the degree of preoperative functional impairment in 4 grades. In the immediate postoperative period a worsening of functional capacity was observed. Only 28.8 % of patients had MCs 1 in the immediate postoperative period; 34.8 % had MCs 2; 20.4 % had MCs 3 and 16 % had MCs 4. At 5 years of follow-up (101 patients) the frequencies of grades 1 and 2 were increased: 59 % of patients had MCs 1, 20.8 % had MCs 2, 10,9 % had MCs 3 and 9.3 % had MCs 4. The extension of the lesion on the sagittal plan calculated on the preoperative MRI, was the only predictive factor associated with the immediate postoperative outcome and the short-term follow-up (P=0.04), whereas the preoperative neurological status is the only predictive factor for long-term follow-up (P=0.005). CONCLUSION: Gross total removal remains the mainstay treatment for IE. Early surgery is indicated if the patient is symptomatic or the tumor increases in size. A postoperative regular follow-up is mandatory for at least 10 years due to the risk of recurrence. If a growing residue is detected, a second intervention is recommended without any adjuvant treatment if a WHO grade I lesion is confirmed by the pathological analysis. Complementary treatment should be reserved for high-grade ependymomas or in case of unresectable and progressive residue.

[Intramedullary astrocytomas: A French retrospective multicenter study]. / Astrocytomes intramédullaires : analyse rétrospective française multicentrique.

AIM: The authors report the results of a multicenter retrospective series with a long-term follow-up and the analysis of clinical, radiological, surgical data as well as the complementary treatments in patients with intramedullary astrocytomas (IA). MATERIALS AND METHODS: We performed a retrospective analysis of all the patients with IA operated on between 1984 and 2011 at 7 French centers (Kremlin-Bicêtre, Lille, Lyon, Marseille, Montpellier, Nice, and Nîmes). The minimum follow-up was 12 months. The clinical evaluation was based on the McCormick scale (MCS) results from the pre- and postoperative period. RESULTS: Data from 95 patients with a pathologically confirmed diagnosis of IA were considered: 54 patients were treated at the Neurosurgical Department of Kremlin-Bicêtre Hospital, 8 were treated at Lille and 33 were treated in the south region of France. The epidemiological analysis was performed on the whole cohort of patients while follow-up considerations were made solely on the 54 patients managed at Kremlin-Bicêtre Hospital to obtain homogeneous data. The average age at diagnosis was 35.6 years without significant gender difference (47 % men for 53 % women). The age at first clinical manifestation was 33.7 years. The average duration of the symptoms before the diagnosis was 22.9 months. Neuropathic pain was the principal revealing symptom (76 % of cases). The localization of IA was thoracic in 40 %, purely cervical in 28.4 %. Complete removal was achieved in 29.5 % of cases when considering the whole cohort and in 38 % of cases treated at Bicêtre Hospital. The histological distribution recorded was: grade 1 in 35 %; grade 2 in 35 %; grade 3 in 22 % and grade 4 in 8 %. During the early postoperative period (3 months) a worsening of functional capacity was observed with an increase in the frequencies of ranks 3 and 4 of MCS in 18.4 %. At 5 years follow-up, the frequencies of ranks 1 and 2 were increased. The application of a Cox model for the determination of the relative risk of death for IA grade 1 and 2 (66 patients) showed a probability of survival at 5 years of 78.6 % (CI 95 %: 68.6 %-87.6 %). Survival at 10 years is to 76.8 % (CI 95 %: 62.3 %-84.2 %). CONCLUSION: Surgery is indicated if the patient is symptomatic or the tumor increases in size. A radical excision remains the mainstay of treatment, while searching to preserve the motor function. A total resection was however only possible in 38 % of cases. A regular postoperative follow-up is compulsory and the adjuvant treatment is based on chemotherapy and radiotherapy according to the histological type.

Development of a lateralization index for intramedullary astrocytomas and ependymomas.

INTRODUCTION: Intramedullary ependymomas are classically described as tumors developing in the center of the spinal cord, whereas intramedullary astrocytomas are more dispersed. This description only remains approximate. The aim of this study was to establish a lateralization index (LI), which is based on radiological measurements and may help in differentiating the two tumors. MATERIAL AND METHODS: Retrospective analysis based on the analysis of MRI of patients operated on for intramedullary astrocytoma or ependymoma in the Neurosurgical Department of Bicêtre Hospital. We consider the distance between the center of the spinal cord and the lateral edge of the tumor, this measurement is called a. Measurement b is the maximal axial diameter of the tumor, measured on the same axis of a. Measurements are made on T1-weighted axial MR images after gadolinium administration. The LI is calculated by the ratio a/b. RESULTS: Twenty-one patients were included in the study, 11 astrocytomas and 10 ependymomas. The average LI was 0.7425 (0.5611 to 1.1673) for astrocytomas and 0.5462 (0.5276 to 0.5995) for ependymomas. The difference of LI between the two types of tumors was statistically significant (P<0.05). CONCLUSION: This study confirms that for ependymomas the LI is close to 0.5, which is the value of a completely centered tumor, unlike astrocytomas. The LI can be an additional tool in helping to differentiate the two types of tumors on the preoperative imagery.

Subarachnoid and intra-cerebral hemorrhage in young adults: rare and underdiagnosed.

OBJECTIVES: Convexity subarachnoid and intra-cerebral hemorrhages, in patients aged<50 years, are always a diagnostic challenge. This condition is characterized by acute headaches with or without neurological symptoms and/or seizures, and by the radiological demonstration of subarachnoid and/or intra-cerebral hemorrhages and, more rarely, by the association of ischemic events. PATIENTS AND METHODS: In a prospective series of 30 consecutive patients (median age 31 years; 22 women) with a subarachnoid and intra-cerebral hemorrhages, 19 were diagnosed with reversible cerebral vasoconstriction syndrome (RCVS), 7 with cerebral venous sinus thrombosis (CVST), and 4 with a bleeding mycotic aneurysm (MA). RESULTS: RCVS appeared spontaneously in 16 patients and was related to the postpartum period in three cases. Subarachnoid hemorrhage (SAH) was demonstrated in 24 patients as follows: 18 cases were in cortical areas, 4 were in the polygon of Willis, one was inter-hemispheric, and one was inter-hemispheric/intra-cerebral. A convexity pure intra-cerebral hemorrhage (ICH) was recorded in 6 cases. Among the 7 patients suffering from CVST, the superior sagittal sinus was involved in 4 cases, the transverse sinuses (TS) in 2, and the TS plus sigmoid sinus (SS) in one. CONCLUSION: The three most common causes in this series were RCVS, followed by CVST and bleeding from MA. Because of atypical clinical or radiological presentations, this large spectrum of etiologies can cause diagnostic difficulties. Therefore, careful analysis is needed to ensure correct and prompt diagnosis and to avoid any dangerous delays in management.

MALIGNANT TRANSFORMATION OF CRANIAL NERVE SCHWANNOMA AFTER RADIOSURGERY - CASE REPORT.

We describe an unusual case of malignant transformation of benign cranial nerves schwannoma eleven months after surgery and five months post radiotherapy. There has been no evidence of recurrence after 5 years follow-up. This early malignant transformation of a schwannoma involved cranial nerves IX, X, XI. Due to the uncommon presentation and the uncertainty of the actual role of the radiation on the tumor behavior, the report of this case was considered clinically important.