Targeted sequencing of cancer-related genes reveals a recurrent TOP2A variant which affects DNA binding and coincides with global transcriptional changes in glioblastoma.

High-grade gliomas are aggressive, deadly primary brain tumors. Median survival of patients with glioblastoma (GBM, WHO grade 4) is 14 months and <10% of patients survive 2 years. Despite improved surgical strategies and forceful radiotherapy and chemotherapy, the prognosis of GBM patients is poor and did not improve over decades. We performed targeted next-generation sequencing with a custom panel of 664 cancer- and epigenetics-related genes, and searched for somatic and germline variants in 180 gliomas of different WHO grades. Herein, we focus on 135 GBM IDH-wild type samples. In parallel, mRNA sequencing was accomplished to detect transcriptomic abnormalities. We present the genomic alterations in high-grade gliomas and the associated transcriptomic patterns. Computational analyses and biochemical assays showed the influence of TOP2A variants on enzyme activities. In 4/135 IDH-wild type GBMs we found a novel, recurrent mutation in the TOP2A gene encoding topoisomerase 2A (allele frequency [AF] = 0.03, 4/135 samples). Biochemical assays with recombinant, wild type (WT) and variant proteins demonstrated stronger DNA binding and relaxation activity of the variant protein. GBM patients carrying the altered TOP2A had shorter overall survival (median OS 150 vs 500 days, P = .0018). In the GBMs with the TOP2A variant we found transcriptomic alterations consistent with splicing dysregulation. luA novel, recurrent TOP2A mutation, which was found exclusively in four GBMs, results in the TOP2A E948Q variant with altered DNA binding and relaxation activities. The deleterious TOP2A mutation resulting in transcription deregulation in GBMs may contribute to disease pathology.

Morphological alterations of the neuronal Golgi apparatus upon seizures.

AIMS: Epilepsy is one of the most common chronic neurological disorders, affecting around 50 million people worldwide, but its underlying cellular and molecular events are not fully understood. The Golgi is a highly dynamic cellular organelle and can be fragmented into ministacks under both physiological and pathological conditions. This phenomenon has also been observed in several neurodegenerative disorders; however, the structure of the Golgi apparatus (GA) in human patients suffering from epilepsy has not been described so far. The aim of this study was to assess the changes in GA architecture in epilepsy. METHODS: Golgi visualisation with immunohistochemical staining in the neocortex of adult patients who underwent epilepsy surgery; 3D reconstruction and quantitative morphometric analysis of GA structure in the rat hippocampi upon kainic acid (KA) induced seizures, as well as in vitro studies with the use of Ca2+ chelator BAPTA-AM in primary hippocampal neurons upon activation were performed. RESULTS: We observed GA dispersion in neurons of the human neocortex of patients with epilepsy and hippocampal neurons in rats upon KA-induced seizures. The structural changes of GA were reversible, as GA morphology returned to normal within 24 h of KA treatment. KA-induced Golgi fragmentation observed in primary hippocampal neurons cultured in vitro was largely abolished by the addition of BAPTA-AM. CONCLUSIONS: In our study, we have shown for the first time that the neuronal GA is fragmented in the human brain of patients with epilepsy and rat brain upon seizures. We have shown that seizure-induced GA dispersion can be reversible, suggesting that enhanced neuronal activity induces Golgi reorganisation that is involved in aberrant neuronal plasticity processes that underlie epilepsy. Moreover, our results revealed that elevated cytosolic Ca2+ is indispensable for these KA-induced morphological alterations of GA in vitro.

Usefulness of time-resolved MR angiography in spinal dural arteriovenous fistula (SDAVF)-a systematic review and meta-analysis.

Spinal dural arteriovenous fistulas (SDAVFs) constitute the most common type of spinal vascular malformations. Their diagnosis requires spinal digital subtraction angiography (DSA), which is time-consuming, requires catheterizing many vessels, and exposes patient to a high radiation and contrast doses. This study aims to evaluate the usefulness of time-resolved MR angiography (TR-MRA) in SDAVF diagnosis. We performed a systematic review of the PubMed and EMBASE databases followed by a meta-analysis. TR-MRA was an index test, and spinal DSA was a reference. Of the initial 324 records, we included 4 studies describing 71 patients with SDAVFs. In 42 cases, TR-MRA was true positive, and in 21 cases, it was true negative. We found 7 false-positive cases and 1 false negative. TR-MRA allowed for shunt level identification in 39 cases. Of these, the predicted level was correct in 23 cases (59%), to within 1 level in 38 cases (97.4%) and to within 2 levels in 39 cases (100%). The diagnostic odds ratio was 72.73 (95% CI [10.30; 513.35]), z = 4.30, p value < 0.0001. The pooled sensitivity was 0.98 (95% CI [0.64; 1.00]), and the pooled specificity was 0.79 (95% CI [0.10; 0.99]). The AUC of the SROC curve was 0.9. TR-MRA may serve as a preliminary study to detect SDAVFs and localize the shunt level with sensitivity and specificity as high as 98% and 79%, respectively. Unless the TR-MRA result is unequivocal, it should be followed by a limited spinal DSA.

Anatomical aspects of the insula, opercula and peri-insular white matter for a transcortical approach to insular glioma resection.

The insula is a lobe located deep in each hemisphere of the brain and is surrounded by eloquent cortical, white matter, and basal ganglia structures. The aim of this study was to provide an anatomical description of the insula and white matter tracts related to surgical treatment of gliomas through a transcortical approach. The study also discusses surgical implications in terms of intraoperative brain mapping. Five adult brains were prepared according to the Klingler technique. Cortical anatomy was evaluated with the naked eye, whereas white matter dissection was performed with the use of a microscope. The widest exposure of the insular surface was noted through the temporal operculum, mainly in zones III and IV according to the Berger-Sanai classification. By going through the pars triangularis in all cases, the anterior insular point and most of zone I were exposed. The narrowest and deepest operating field was observed by going through the parietal operculum. This method provided a suitable approach to zone II, where the corticospinal tract is not covered by the basal ganglia and is exposed just under the superior limiting sulcus. At the subcortical level, the identification of the inferior frontoocipital fasciculus at the level of the limen insulae is critical in terms of preserving the lenticulostriate arteries. Detailed knowledge of the anatomy of the insula and subcortical white matter that is exposed through each operculum is essential in preoperative planning as well as in the intraoperative decision-making process in terms of intraoperative brain mapping.

Unruptured intracranial aneurysms: relation between morphology and wall strength.

AIM OF THE STUDY: To determine the morphological features distinguishing small unruptured saccular intracranial aneurysms (sIAs) with high and low wall strength (WS) in post mortem subjects. CLINICAL RATIONALE FOR THE STUDY: Subarachnoid haemorrhage caused by sIA rupture is associated with increased mortality and morbidity. Analysis of the morphology and biomechanical properties of sIAs might facilitate the identification of clinically relevant risk factors for sIA rupture. MATERIAL AND METHODS: Eight single unruptured sIAs were found among eight subjects during 184 post mortem examinations. After assessment of the dimensions, aspect ratio (AR), size ratio (SR), height/width ratio (HW), bottleneck factor (BNF), and shape, sIAs with adjacent cerebral arteries were subjected to quasi-static increasing pressure until the wall of the cerebral artery or sIA ruptured. RESULTS: In three specimens, the sIA ruptured at a significantly lower average pressure than the other cases, in which the rupture occurred within the wall of the adjacent cerebral artery (769 vs. 1,259 mmHg; p = 0.035). The sIAs with low WS, i.e. sIAs that ruptured during experiments, were characterised by significantly increased dome dimensions compared to sIAs with high WS (p < 0.05). At the same time, no significant differences were observed between high and low WS categories regarding AR, SR, HW, and BNF, or the presence of an irregular dome shape. CONCLUSIONS AND CLINICAL IMPLICATIONS: Dome dimension was the only feature that distinguished unruptured sIAs as having low or high WS, and this supports observations that sIAs with increased dome dimensions are characterised by an increased risk of rupture. Thus, dome dimension may be more useful than other morphometric parameters, such as AR, SR, HW and BNF, in assessing the rupture risk assessment of small unruptured sIAs.

Chordoma and meningioma arising as a collision tumor in the petroclival region: Case report and literature review.

INTRODUCTION: The co-existence of two independent brain tumors at the same anatomical site is rare and occurs as a "collision tumor" or "tumor-to-tumor metastasis." In intracranial location, meningioma is the most common neoplasm in such coincidences. CASE DESCRIPTION: We present a case involving a 31-year-old woman with a complex tumor consisting of chordoma and meningioma in the petroclival region. The patient presented with left facial numbness, ataxia, and left-sided hemiparesis. Computed tomography and magnetic resonance imaging showed a well-demarcated, intradural, extra-axial tumor mass in the petroclival region. After complete total resection, histopathological examination revealed two different parts of the tumor, consisting of chordoma and meningioma. Therefore, additional radiation therapy and adjuvant chemotherapy were given. DISCUSSION: To the best of our knowledge, this is the first description of the simultaneous occurrence of chordoma and meningioma in the same anatomical location. In such a scenario, differential diagnosis of choroid meningioma and chordoma is required. The correct recognition of both components is important for treating this complex tumor, and its separate elements may require independent approaches.

Predictors of Class I epilepsy surgery outcome in tumour-related chronic temporal lobe epilepsy in adults.

OBJECTIVE: Temporal lobe tumours, especially low-grade gliomas and glioneuronal tumours, are common causes of seizures in patients referred for epilepsy surgery. We here present our experience of surgical treatment of patients with intractable chronic epilepsy associated with temporal lobe tumours, focusing on the long-term surgical outcomes and the features associated with better seizure control. METHODS: In this study, we retrospectively analysed 44 consecutive patients from a total of 182 with refractory temporal lobe epilepsy presenting with long-term intractable epilepsy due to a temporal lobe tumour who were surgically treated at our institution between 2005 and 2015 with post-surgical follow-up of at least two years. All patients underwent a standard pre-surgical evaluation that included: history and physical examination with a description of the seizure semiology, serial scalp EEG recording, brain MR imaging, and a detailed neuropsychological evaluation. Our surgical strategy comprised tumour resection, and combined mesial temporal and neocortical resection in most cases. RESULTS: No patient died during surgery or the postoperative course. Seven patients had postoperative complications, of whom two had permanent hemiparesis due to ischaemic stroke. At the final follow-up, a favourable seizure outcome (Engel Class I) was found in 37 patients (84%), including 31 (70.5%) in Engel Class IA (excellent result). Two (4.5%) patients presented with an Engel Class II outcome (unfavourable outcome). Five patients (11.5%) were in Engel Classes III or IV (surgical failure). We found that complete resection of the hippocampus along with tumour and temporal pole removal was strongly associated with seizure freedom (p = 0.015). Pathological diagnosis was also a significant prognostic indicator of tumour-related seizure freedom. Patients with a diagnosis of a glioneuronal tumour benefited from more seizure freedom after resection compared to those who had a low-grade glioma (p = 0.024). CONCLUSION: The most appropriate management of tumour-related chronic temporal lobe epilepsy in adults appears to be tai-lored temporal lobe resection including tumour and hippocampal complex removal. Surgical treatment of tumoural temporal lobe epilepsy demonstrates excellent results in terms of seizure improvement, especially in patients with glioneuronal tumours.

Trigone ventricular meningiomas - clinical characteristics, histopathology and results of surgical treatment.

AIM OF THE STUDY: Intraventricular meningiomas (IVMs) are rare tumours accounting for 0.5-3.0% of all meningiomas. IVMs require different surgical approaches and preparation in deep brain areas. The aim of our study was to present the clinico- -histopathological characteristics and treatment outcomes of trigone IVMs in a series of 15 patients. MATERIALS AND METHODS: Eight women and seven men (mean age 52) with 15 trigone IVMs were retrospectively analysed. Patients presented with headache (47%), psychoorganic syndrome (40%), hemianopsia (33%) or paresis (20%), including three (20%) patients with Karnofsky Performance Scale (KPS) < 80. Mean tumour size was 55.2 mm (range: 30-100 mm). RESULTS: Gross total tumour resection was performed in 14 (93%) cases, and subtotal in one (7%). A new deficit appeared in 83% (5/6) following a transparietal approach, in 14% (1/7) following a transtemporal approach, and in none of two patients following a transoccipital approach. Postoperative complications occurred in six (40%) patients; no patient died, but in two (13%) the new deficit was permanent. Tumour re-growth was found in two (13%) patients after 14 and 31 months. Meningiomas of WHO grade I occurred in 12, grade II in three, and grade III in one tumour recurrence. In long-term follow-up (mean: 60.8 months), including the results of revision operations, KPS: 80-100 was in 13 (87%) patients, KPS: 50 in one (severe hemiparesis after revision) and one patient was lost to follow-up (KPS: 100 on discharge). CONCLUSIONS: 20% of IVMs in our series were atypical. The results of surgery for IVMs, although satisfactory in general, require further improvement by reducing the rate of focal deficits resulting from a surgical approach.

Social Perception in Mesial Temporal Lobe Epilepsy: Interpreting Social Information From Moving Shapes and Biological Motion.

Dysfunction in the understanding of social signals has been reported in persons with epilepsy, which may partially explain lower levels of life satisfaction in this patient population. Extensive assessment is necessary, particularly when the mesial temporal lobe, responsible for emotion processing, is affected. The authors examined multiple levels of social perception in patients with mesial temporal lobe epilepsy (MTLE), including judgments of point-light motion displays of human communicative interactions (Communicative Interactions Database-5 Alternative Forced Choice format) and theory-of-mind processes evaluated using geometric shapes (Frith-Happé animations [FHA]). This case-control study included MTLE patients with anterior temporal lobectomies (ATL+) (N=19), MTLE patients without lobectomies (ATL-) (N=21), and healthy controls (HCs) (N=20). Both groups of MTLE patients were less efficient in recognizing goal-directed and mentalizing interactions of FHA compared with HC subjects. The ATL+ group attributed emotions to FHA less accurately than HC subjects. Both the ATL- and ATL+ groups classified individual point-light animations more often as communicative than the HC group. ATL+ patients were also less efficient in interpreting point-light animations in terms of individual actions than the HC group. The number of years of epilepsy duration was inversely correlated with recognition of FHA interactions. The mean number of seizures was inversely correlated with the interaction identification in point-light stimuli. Patients with MTLE, irrespective of surgical treatment, present impaired social perception in domains assessed with abstract moving shapes or nonabstract biological motion. This impairment may be the basis of problems faced by patients reporting difficulties in understanding the intentions and feelings of other individuals.

Symptomatic medulla compression by vertebral artery.

OBJECTIVE: Vertebral artery medulla compression syndrome (VAMCS) is a very rare condition manifesting as different neurological focal deficits. The case of a 36-year-old male with symptomatic brainstem compression by vertebral artery (VA) treated by means of microvascular decompression (MVD) and a review of the literature is presented. CASE REPORT: On admission, a 36-year-old patient presented with hypoalgesia, hypothermesthesia and hemiparesis on the left side. Magnetic resonance imaging (MRI) of the head disclosed the right VA loop compressing the ventrolateral medulla and excluded other entities such as brain tumor, stroke and multiple sclerosis. Since displacement and significant compression of the right pyramidal tract was confirmed by diffusion tensor imaging (DTI), neurovascular compression syndrome was diagnosed. The patient underwent MVD of the medulla using a Gore-Tex implant as a separating material via the right far-lateral approach. The left hemiparesis and hemisensory loss remitted rapidly after the procedure. The post-procedural neurological improvement was maintained at one year follow-up. Based on a review of the literature, a total of 33 cases of surgically treated VAMCS has been reported so far. CONCLUSION: VAMCS should be considered as the cause of neurological deficits when other pathological entities are ruled out. In symptomatic conflict of the VA with the medulla, microvascular decompression using a Gore-Tex implant can be an effective method of treatment. Nevertheless, a statistical analysis on all reported cases showed favorable results using the VA repositioning technique when compared with MVD (success rate 91% vs. 58%, p<0.05).

Diffusion tensor tractography of pyramidal tracts in patients with brainstem and intramedullary spinal cord tumors: Relationship with motor deficits and intraoperative MEP changes.

PURPOSE: To evaluate whether pyramidal tracts course alterations observed in diffusion tensor tractography (DTT) in cases of brainstem and intramedullary spinal cord tumors reflect patient clinical status and prognosis. MATERIALS AND METHODS: For this purpose, we assessed in 17 patients relationships between pyramidal tracts course alterations observed in DTT (classified into four categories: unaffected; displaced or interspaced; partially disintegrated and completely disintegrated) performed on a 1.5 Tesla scanner and the presence of preoperative motor deficits, changes observed in motor evoked potentials (MEPs) records at the beginning of the operation, deterioration of the MEPs records during the operation, and perioperative deterioration of muscle strength. RESULTS: We found that, if the picture of pyramidal tracts in DTT was worse, motor deficit was more common (P = 0.062). This observation was even more evident (P = 0.027), when cases with at least partially destroyed pyramidal tracts were compared with cases with normal or at most displaced or interspaced by tumor but still preserved pyramidal tracts. Significant relationships were also found between changes in DTT and abnormal MEP records at the beginning of the operation (P = 0.032) and perioperative deterioration of muscle strength (P = 0.0058). CONCLUSION: A close relationship was found between pyramidal tracts course alterations in DTT imaging and preoperative motor status and especially with changes in the MEP records at the beginning of the operation. DTT may be a method that allows the better planning of brainstem and intramedullary spinal cord tumors operations and may help in the risk assessment of postoperative motor deficits. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 4 J. MAGN. RESON. IMAGING 2017;46:715-723.

Gaze matters! The effect of gaze direction on emotional enhancement of memory for faces in patients with mesial temporal lobe epilepsy.

PURPOSE: The aim of the study was to examine if gaze and emotional expression, both highly self-relevant social signals, affect the recollection accuracy of perceived faces in patients with mesial temporal lobe epilepsy (MTLE). METHODS: Forty patients with MTLE (twenty-one without surgery and nineteen after anterior temporal lobectomy) as well as twenty healthy controls (HC) took part in the study. We used a set of 64 facial stimuli: 32 neutral and 32 emotional displays (16 fearful; 16 angry) from well-established affective stimuli databases. Half of the faces in each condition had eyes directed straight and half - away from the observer. Participants performed a gender identification task, and then, after a 45-minute delay were asked to identify the previously seen stimuli, presented among a new set of photos. RESULTS: Increased automatic learning of angry and fearful compared to neutral expressions was found in HC. There was no emotional enhancement of memory in MTLE but an increased learning for faces with averted than direct gaze. CONCLUSION: Our results expand on previous research by demonstrating that emotion expression and gaze direction can affect memory of faces. The study supports the hypothesis that healthy individuals and patients with temporal lobe abnormalities present different patterns of emotional gazes processing. The potential consequences of altered emotional gaze processing and social cognition impairments need to be further investigated to improve the quality of life of patients with MTLE.

Surgical treatment of intramedullary ependymomas.

OBJECT: The aims of this study were to present the results of surgery for intramedullary ependymomas (IEs), analyze complication and recurrence rates, and analyze factors that might influence outcome. METHODS: The charts of 29 consecutive patients (women - 8, men - 21; mean age - 38 years; range: 18-72) operated for IE were retrospectively analyzed. Mean follow-up was 9 years. Eighteen tumors (62%) were located in the cervical or cervicothoracic spine, and average tumor length was four spinal levels. Twenty patients (69%) presented with neurological deficit. RESULTS: Gross total resections (GTRs) comprised 87% of cases, subtotal resections (STRs) 10%, and partial resections 3%. The neurological outcome on postoperative day 1 was as follows: modified McCormick scale (mMS) grade I - 6%, grade II - 21%, grade III - 21%, grade IV - 31%, and grade V - 21%; at follow-up, outcomes were mMS grade I - 42%, grade II - 34%, grade III - 10%, and grade V - 14% of patients. Compared to the preoperative period, 69% of patients deteriorated postoperatively; however, 62% improved or remained without deficit in follow-up, and deterioration persisted in 24%. The functional results were significantly worse when the intraoperative monitoring potentials dropped below 50% (p=0.005) and if the tumor involved >3 spinal levels (p=0.039). Fourteen postoperative complications in 10 patients (34%) included respiratory failure (14%), pneumonia (7%), urinary infection (10%), bed sores (10%), and CSF leak (7%). Two tumors progressed after STR, with progression-free survival times of 5 and 14 years. No recurrence was observed after GTR. CONCLUSIONS: Total tumor resection is the treatment of choice in cases of IEs: no tumor re-growth occurred after total resection, 86% of patients were independent at follow-up, and the 10-year survival rate was 79%.

Surgical treatment for spinal dural arteriovenous fistulas: Outcome, complications and prognostic factors.

BACKGROUND AND PURPOSE: Spinal dural arteriovenous fistulas (SDAVFs) are rare, acquired pathology and they inevitably lead to severe disability if untreated. The aim of this study is to present the outcome and complications, and to find factors that may affect the outcome after surgical treatment. METHODS: Seventeen consecutive patients (men - 14, women - 3, age: 41-79) were retrospectively analyzed. The patients presented with paraparesis (88%), bladder symptoms (71%) and/or sensory disturbances (65%). The fistula was found in the upper thoracic spine in 2 cases, in the lower thoracic (T7-Th12) in 11 cases, and in the lumbar spine in 4 cases. Microsurgical shunt interruption was performed in all, followed by epidural arteries coagulation in 12 cases. RESULTS: In the long term, improvement or achievement of a good stable condition was observed in 13 patients (76%), and no patient deteriorated. All 5 paraplegic patients improved by at least 1 grade in MCS. Satisfactory results (modified McCormick Scale grades I-II) were found in 10 patients (59%), and 15(88%) were independent. Postoperative complications occurred in 4 patients (24%), two of them (12%) required revision surgery for epidural hematoma. The success rate was 94%; one patient required revision surgery for recurrent SDAVF. Better neurological condition on admission (p=0.0098) and age >60 years (p=0.0498) were the factors associated with satisfactory outcome. CONCLUSIONS: Microsurgical closing of a SDAVF brings good and stable results over time. Aggressive treatment should be attempted even in cases of total loss of spinal cord function. Neurological condition before surgery and age may influence the outcome.

Differences in quality of life of women and men with drug-resistant epilepsy in Poland.

PURPOSE: The aim of the study was to assess the differences in health-related quality of life in groups of men and women suffering with drug-resistant epilepsy and to determine which factors influence quality of life. METHODS: The examined group consisted of 64 subjects with drug-resistant epilepsy - 31 men and 33 women. The mean duration of epilepsy was 17.56±8.92 and 19±9.56years, respectively. The following diagnostic tools were used: QOLIE-31-P, Wechsler Adult Intelligence Scale - Revised (WAIS-R (PL)), and Hamilton Rating Scale for Depression (HRSD). RESULTS: Scores in QOLIE-31-P did not differ significantly between groups of men and women with drug-resistant epilepsy; however, a more detailed analysis revealed certain disparities. Multiple regression analyses indicated that some distinct factors were associated with quality of life in each sex. In the group of women, there were no significant predictors of their quality of life. Among the group of men, depression intensity was the only statistically significant QoL predictor, explaining 16% of the variance (adjusted R(2)=0.16, F(6, 24)=19.7, p<0.01). Moreover, patients with depression had lowered scores in the Emotional Well-Being and Energy/Fatigue subscales, regardless of the sex. CONCLUSION: The study revealed that, despite similar scores in QOLIE-31-P, specific factors may differentially affect the quality of life of men and women with drug-resistant epilepsy in Poland. Nevertheless, replication of these results with a larger number of participants is needed for a more definitive conclusion.

Epidermoid cysts of the cerebellopontine angle: Clinical features and treatment outcomes.

OBJECTIVE: To report clinical characteristics, treatment outcomes and risk of recurrence in patients with surgically treated cerebellopontine angle epidermoids. METHODS: In 1994-2013, we operated 17 patients, including 7 with tumor limited to the cerebellopontine angle, 7 with cerebellopontine angle tumor penetrating supratentorially, and 3 with cerebellopontine angle tumor extending along skull base to contralateral cerebellopontine angle. All patients were followed-up for the mean duration of 126 months. RESULTS: On admission cranial nerve symptoms predominated. Total tumor removal was achieved in 5 patients, and incomplete removal (with small tumor remnants left on vessels, nerves, or brainstem) in 12 patients. Postoperatively, preoperative deficits worsened in 2 and new postoperative deficits occurred in 10 patients. The extent of tumor expansion had no effect on postoperative morbidity and risk of recurrence. During long-term follow-up, improvement or resolution of preoperative deficits was seen in 11 of 17 patients, and new postoperative deficits in 8 of 10 patients. Symptomatic recurrences after an average of more than 9 years were noted in 5 patients, 3 of whom were reoperated. Recurrences occurred in some younger patients and always in area of primary tumor. No effect of extent of tumor removal on risk of recurrence was found. CONCLUSIONS: The extent of tumor removal had no effect on the risk of recurrence, and thus it may be acceptable to leave tumor capsule fragments adhering closely to nerves, vessels, or brainstem. During long-term follow-up, resolution or improvement of present preoperatively and new postoperative neurological deficits may be expected in most patients.

Surgery for sporadic vestibular schwannoma. Part II. Complications (not related to facial and auditory nerves).

INTRODUCTION: The aim of this study was to analyze the frequency and consequences of postoperative complications (PC) after surgery for sporadic vestibular schwannoma and to find factors that increase the risk of PC occurrence. MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; age ranged from 18 to 74) operated on with the retrosigmoid (217) or translabyrinthine (3) approach. Complicated postoperative period was defined as an occurrence of at least one of: cerebrospinal fluid (CSF) leakage, hematoma in the tumor bed, intracerebellar hematoma, cerebellar swelling, brainstem stroke, hydrocephalus (HCP), healing problems, meningitis and cranial nerves (excluding VII-VIII) palsies or cerebellar symptoms. Correlation studies and multivariate regression analysis were performed. RESULTS: PC occurred in 55 patients (25%). PC included lower cranial nerve (LCN) palsy (8.2%), cerebellar symptoms (7.3%), CSF leakage (5.9%), HCP (5%), CNVI palsy (3.1%), meningitis (1.8%), cerebellar swelling (1.4%), CNV dysfunction (0.9%), intracerebellar hematoma (0.5%) and lethal brainstem stroke (0.5%). In long term follow-up, LCN deficit was present in 2 patients (0.9%), cerebellar syndrome in 4(1.8%) and facial hypoesthesia in 2(0.9%). One patient (0.5%) developed bilateral blindness, secondary to preoperative optic nerve atrophy. As a result of PC, 10 patients (4.5%) required 11 additional surgical procedures. In statistical analysis, PC were independently related to preoperative cerebellar syndrome (p=0.002) and tumor size (>30 mm vs.<30 mm, p<0.05). The risk of PC diminished significantly with the increased number of performed procedures from 40% at the beginning to 16.4% in the last 55 cases. CONCLUSIONS: Tumor size, cerebellar syndrome at presentation and experience of the team were the three most important risk factors for PC occurrence. Permanent deficit secondary to PC remained in only 4% of the patients.

Surgery for sporadic vestibular schwannoma. Part I: General outcome and risk of tumor recurrence.

BACKGROUND: Vestibular schwannomas are slow growing, benign tumors. There are three possible management options: surgery, radiation treatment or active surveillance. The aim of this study was to assess the general outcome and risk of tumor recurrence. MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; the age ranged from 18 to 74) operated with the retrosigmoid transmeatal approach. The largest extrameatal diameter of the tumor ranged from 8 to 72mm (mean 30mm). According to the Samii grading scale, the tumors were classified as follows: T2-12 (6%), T3-51 (23%) and T4-157 (71%). Gross total resection was performed in 217 patients and neartotal in 3. RESULTS: Two hundred and eighteen (99.1%) patients were discharged home in a satisfactory neurological condition (GR or MD in GOS). One (0.5%) patient died due to brainstem infarction. One (0.5%) patient had unchanged severe cerebellar syndrome in comparison to the preoperative period (SD in GOS). In long-term follow-up, one patient went blind within a few months after surgery. Including the results of further neurosurgical procedures for CSF leak, shunt implantation, tumor regrowth and facial nerve reanimation, 98.6% of the patients were fully independent but with different neurological deficits. Tumor recurrence was observed in 5 (2.3%) patients during the follow-up period (mean term: 6.4 years). The average time to recurrence diagnosis was 8.8 years. All those patients were operated on again without any adjuvant therapy and there was no further re-growth at mean follow-up of 5.2 years. CONCLUSIONS: Complete removal of VS is usually curative and poses very low risks of severe disability (if audio-facial sequels are not included), mortality and long-term recurrence. For recurrent tumors, carefully tailored revision surgery without irradiation offers a high efficacy with low risk of complications.

Management of spinal tumors in neurofibromatosis type 2 patients.

OBJECTIVE: We sought to determine clinical characteristics of NF2 patients with spinal lesions and to define when and like the spinal tumors are a major problem in the treatment of patients with NF2. METHODS: The authors retrospectively reviewed the clinical records, neuroimaging studies, and follow-up data of the 34 patients with neurofibromatosis type 2, who were treated at our institution between 1998 and 2014. 23 patients harbored one or multiple spinal tumors. RESULTS: Patients with spinal tumors had a lower age at first symptoms of the disease, a higher number of intracranial meningiomas and non-vestibular schwannomas. 11 patients had one or more intramedullary tumors with MRI characteristics of spinal ependymomas. 22 patients had intradural extramedullary tumors. 7 patients presented with symptomatic spinal tumors on admission or developed symptoms during the follow-up. Only two intramedullary and four extramedullary tumors demonstrated growth in the mean radiological follow-up period of over 6 years. It was found that symptomatic both intra- and extramedullary tumors were associated with younger age at the onset of NF2-related symptoms. 2 patients with intramedullary tumors and 12 patients with extramedullary tumors underwent their tumors resection. In case of symptomatic tumors partial recovery was observed in two patients. CONCLUSION: It seems that close surveillance with MR imaging is a reasonable option for asymptomatic spinal tumors. Nevertheless, intramedullary tumor removal in non-growing and asymptomatic cases may be an option when ABI implantation is considered. Symptomatic tumors and those of documented growth should be eligible for surgical intervention.

Sensorimotor C5 and C6 radiculopathy caused by thrombosed vertebral artery dissection and successfully treated with limited oblique corpectomy - Case report.

The authors report the case of an exceptional presentation of vertebral artery dissection. A 44-year-old man who presented with left shoulder weakness, radicular pain and numbness of the left forearm and thumb was admitted to our hospital with an initial diagnosis of cervical disc herniation. Due to the inconsistency between the levels of radiculopathy (C5 and C6) and discopathy (C6-C7), neuroimaging examinations were extended. Based on MRI, MRA, CTA and DSA, left vertebral artery dissection with intramural hematoma was diagnosed. The patient underwent surgical decompression of the affected nerve roots using the anterolateral approach described by Bernard George. The radicular pain resolved immediately and sensorimotor deficit completely disappeared within 4 months. MRI/MRA performed 6 months after surgery showed the normal image of the vertebral artery. There were no ischemic events within 2.5 years of follow-up.