Multimodal mapping and monitoring is beneficial during awake craniotomy for intra-cranial tumours: results of a dual centre retrospective study.

BACKGROUND: The combination of awake craniotomy with multimodal neurophysiological mapping and monitoring in intra-axial tumour resection is not well described, but may have theoretical benefits which we sought to investigate. METHODS: All patients undergoing awake craniotomy for tumour resection with cortical and/or subcortical stimulation together with one or more of electrocorticography (ECoG/EEG), motor or somatosensory evoked potentials were identified from the operative records of two surgeons at two centres over a 5 year period. Patient, operative and outcome data were collated. Statistical analysis was performed to evaluate factors predictive of intra-operative seizures and surgical outcomes. RESULTS: 83 patients with a median age 50 years (18-80 years) were included. 80% had gliomas (37% low grade) and 13% metastases. Cortical mapping was negative in 35% (language areas) and 24% (motor areas). Complete or near total resection was achieved in 80% with 5% severe long-term neurological deficits. Negative cortical mapping was combined with positive subcortical mapping in 42% with no significant difference in extent of resection rates to patients undergoing positive cortical mapping (p = 0.95). Awake mapping could not be completed in 14%, but with no compromise to extent of resection (p = 0.55) or complication rates (p = 0.09). Intraoperative seizures occurred in 11% and were significantly associated with intra-operative EEG spikes (p = 0.003). CONCLUSIONS: Awake multi-modal monitoring is a safe and well tolerated technique. It provides preservation of extent of resection and clinical outcomes in cases of aborted awake craniotomy. Negative cortical mapping in combination with positive subcortical mapping was also shown to be safe, although not hitherto well described. Electrocorticography further enables the differentiation of seizure activity from true positive mapping, and the successful treatment of spikes prior to full clinical seizures occurring.

Awake brain surgery for autistic patients: Is it possible?

Background: Awake neurosurgery is currently the mainstay for eloquent brain lesions. Opting for an awake operation is affected by a number of patient-related factors. We present a case of a patient with autistic spectrum disorder (ASD) that was successfully operated for a brain tumor through awake craniotomy. To the best of our knowledge, this is the first reported case in the literature. Case Description: A 42-year-old patient, with known ASD since his childhood, underwent awake craniotomy for a left supplementary motor area tumor. Detailed preoperative preparation of the patient was done to identify special requirements and establish a good patient-team relationship. Intraoperatively, continuous language and motor testing were performed. Conversation and music were the main distractors used. Throughout the operation, the patient remained calm and cooperative, even during a focal seizure. Mapping allowed for >80% resection of the tumor. Postoperatively, the patient recovered without any deficits. Conclusion: This case shows that with growing experience and meticulous preparation, the limits of awake craniotomy can be expanded to include more patients that were previously considered unfit.

Convexity meningioma associated with noncontiguous dural arteriovenous fistula.

BACKGROUND: Concomitant dural arteriovenous fistulas (DAVFs) and meningiomas have been rarely described. DAVFs can be either continuous or at a distant location from the meningioma, with different pathophysiologic mechanisms involved in each situation. CASE DESCRIPTION: We report the case of a 74-year-old woman presenting with left-sided hemiparesis secondary to a large right convexity meningioma, associated with a noncontiguous Borden 3 DAVF. Both lesions were treated surgically in the same setting. The patient improved after surgery, and postoperative imaging showed complete resection of the meningioma and absence of recurrence of the fistula at 4 years. CONCLUSION: To the best of our knowledge, this is the first case of concomitant surgical treatment of a meningioma and noncontiguous DAVF.

Craniocervical junction arachnoid cyst.

We report the case of a 33-year-old patient who underwent fenestration of a large symptomatic cranio-cervical junction arachnoid cyst.

Onyx embolization of extradural spinal arteriovenous malformations with intradural venous drainage.

BACKGROUND: Extradural arteriovenous malformations (AVMs) are uncommon vascular malformations of the spine. They are characterized by an arteriovenous communication (localized in the paraspinal soft tissues and the epidural venous plexus) that may have retrograde intradural venous drainage. Surgical treatment of extradural AVMs can be challenging because of the common location ventral to the dural sac and the presence of arterialized venous lakes. OBJECTIVE: To assess the effectiveness of embolization with a liquid embolic material (Onyx; ev3 Inc, Irvine, California) in 7 consecutive patients with extradural spinal AVMs and intradural venous drainage. METHODS: We retrospectively collected information regarding patient characteristics, clinical presentation, symptom duration, diagnostic imaging, treatment, complications, and clinical and radiological follow-up. RESULTS: Patients were 5 men and 2 women (mean age, 66 years; range, 45-76 years). Symptom duration varied from 2 months to 6 years. Six patients underwent embolization of the AVM as the primary treatment; 1 patient was treated after attempted surgery. Complete obliteration was demonstrated in each patient on completion angiography and was confirmed during follow-up in 6. Three patients had resolution of their preembolization symptoms. Three patients had improvement but not complete resolution of their motor deficits. In 1 patient, the AVM was an incidental finding during an investigation for chronic low-back pain; symptoms were not thought to be related to the AVM, and symptoms were not ameliorated by successful embolization. CONCLUSION: Our early experience with these uncommon lesions suggests that Onyx embolization is a valuable and effective strategy for extradural spinal AVMs and intradural venous drainage.

Pontine extension of a tentorial schwannoma without cranial nerve involvement: a case report.

INTRODUCTION: Intracranial schwannomas unrelated to the cranial nerves are uncommon. We report a new case of tentorial schwannoma unrelated to the cranial nerves, with extension into the pons. A literature review with discussion of the most relevant pathogenetic aspects is also performed. CASE PRESENTATION: A 42-year-old Caucasian man was admitted with right-sided paresthesias and weakness of his upper and lower extremities. The neurological examination revealed right hemiparesis and hemi-hypoesthesia. A brain magnetic resonance imaging scan revealed a cerebellopontine lesion, arising from the left free edge of the tentorium, and extending into his pons. A piecemeal removal was performed through a retrosigmoid approach. The lesion was not found to be associated with any cranial nerves. The histological examination revealed a schwannoma Antoni type A. His postoperative course was uneventful. At one year follow-up, the patient was neurologically intact and the magnetic resonance imaging of his brain performed at that time showed complete removal without signs of recurrence. CONCLUSION: Tentorial schwannomas are rare clinical entities. Knowledge of their clinical, radiological and anatomical characteristics is very important for the correct diagnosis and management.

Seizures and anticonvulsants after aneurysmal subarachnoid hemorrhage.

Seizures and seizure-like activity may occur in patients experiencing aneurysmal subarachnoid hemorrhage. Treatment of these events with prophylactic antiepileptic drugs remains controversial. An electronic literature search was conducted for English language articles describing the incidence and treatment of seizures after aneurysmal subarachnoid hemorrhage from 1980 to October 2010. A total of 56 articles were included in this review. Seizures often occur at the time of initial presentation or aneurysmal rebleeding before aneurysm treatment. Seizures occur in about 2% of patients after invasive aneurysm treatment, with a higher incidence after surgical clipping compared with endovascular repair. Non-convulsive seizures should be considered in patients with poor neurological status or deterioration. Seizure prophylaxis with antiepileptic drugs is controversial, with limited data available for developing recommendations. While antiepileptic drug use has been linked to worse prognosis, studies have evaluated treatment with almost exclusively phenytoin. When prophylaxis is used, 3-day treatment seems to provide similar seizure prevention with better outcome compared with longer-term treatment.

Retrospective protein expression and epigenetic inactivation studies of CDH1 in patients affected by low-grade glioma.

Aberrant methylation of CpG islands in the promoter regions of tumour cells results in loss of gene function. In addition to genetic lesions, changes in the methylation profile of the promoters may be considered a factor for tumour-specific aberrant expression of the genes.We investigated the methylation status of E-cadherin gene (CDH1) promoter in low-grade glioma and correlated it with clinical outcome. Eighty-four cases of low-grade glioma (43 diffuse astrocytomas, 27 oligodendrogliomas and 14 oligoastrocytomas) with assessable paraffin-embedded tumour blocks and normal brain tissue, derived from non-cancerous tissue adjacent to tumour and commercially normal brain tissue, were collected, from which we determined CDH1 promoter methylation status and E-cadherin protein expression by methylation-specific polymerase chain reaction (MSP) and immunohistochemistry, respectively. CDH1 promoter was found hypermethylated in 54 out of 84 low grade gliomas (64%) compared with 84 normal brain tissue. CDH1 hypermethylation was found in 65% astrocytomas, 66% oligodendrogliomas and 57% oligoastrocytomas. A significant correlation between hypermethylation status, patient survival and progression-free survival was found (P = 0.04). Survival and progression-free survival were lower in patients with hypermethylated CDH1 promoter. We found that 15 astrocytomas, 9 oligodendrogliomas and 6 oligoastrocytomas were immunoreactive for E-cadherin. The incidence of loss of immunoreactivity for E-cadherin decreased significantly with age, overall survival and progression-free survival (P = 0.001, Kaplan-Meier test). We have demonstrated that CDH1 promoter hypermethylation significantly associated with down-regulated E-cadherin expression and overall survival of patients. This may have a bearing on the prognosis of low-grade glioma.

Giant cell angiitis of the central nervous system with atypical presentation.

Giant cell angiitis of the CNS is an uncommon form of vasculitis. Neurological manifestations, both of the peripheral and CNS, are common. The most frequent manifestations are visual loss and stroke. Hemorrhagic onset is uncommon. Most cases have a fatal outcome and a tissue diagnosis is rarely established in life. We describe an unusual case of giant cell angiitis beginning as a hemorrhagic tumoral-like lesion. The results of the histological and ultrastructural analysis have also been reported. Our case illustrates that giant cell angiitis should be considered as a cause of intracerebral hemorrhage, particularly when associated with a relapsing and remitting disease of the CNS.

Intramedullary solitary fibrous tumor of dorsal spinal cord.

Solitary fibrous tumors (SFT) are rare neoplasms of mesenchymal origin involving soft tissues, mainly serosal sites; the spinal cord location is uncommon. We report a case of SFT occurring in the thoracic spinal cord, discussing histological, ultrastructural and molecular aspects. A 75-year-old woman with an MRI suggesting a dorsal intracanalar lesion was admitted to our institution. T5-T7 laminectomies were performed and an intramedullary tumor was discovered. The tumor arose within the spinal cord and was completely removed. Tumor samples were processed for histological, ultrastructural and molecular analysis (comparative genomic hybridization [CGH], methylation status of O6-methylguanine-DNA methyltransferase [MGMT], p16, deleted in colorectal cancer [DCC] and death-associated protein kinase 1 [DAPK1]). The histological examination demonstrated a proliferation of spindle-shaped cells with a collagen-matrix background. Immunohistochemical staining was positive for vimentin and CD34 and negative for S-100 and epithelial membrane antigen. A histological diagnosis of SFT was made. The ultrastructural examination showed undifferentiated cells within a collagenous matrix and sparse extravascular basement membrane. CGH analysis revealed deletion of 9p21 and losses on 2q, 3p, 16q and 19q and gains on 7q; furthermore, no aberrant methylation pattern was found in the promoter region of MGMT, p16, DCC and DAPK1 genes. On the second-year follow-up, the patient was neurologically intact. The occurrence of SFT within the spinal cord parenchyma and its histological characteristics demonstrate that SFTs are not restricted to serosal surfaces. The course of spinal cord SFT is unknown and long-term follow-up is necessary. The histological, ultrastructural and molecular findings are important for the diagnosis and the authors provide a literature review of these aspects.

Gliomatosis cerebri type II: two case reports.

INTRODUCTION: TWO TYPES OF GLIOMATOSIS CEREBRI EXIST: Type I and Type II. We report the results of a histological and genetic study of two cases of gliomatosis cerebri Type II, correlating these results with therapy and prognosis. CASE PRESENTATION: Two patients, a 52-year-old man (Patient 1) and a 76-year-old man (Patient 2) with gliomatosis cerebri II were admitted to our institution; they underwent surgical treatment and received radiotherapy and chemotherapy. At the 24-month follow-up, Patient 1 was still alive, while Patient 2 had died. The poor prognosis of Patient 2 was underlined by molecular analysis which showed that the angiogenesis related genes VCAM1 and VEGF were overexpressed, reflecting the high degree of neovascularization. CONCLUSION: Genes involved in drug resistance and metallothioneins were highly expressed in Patient 2 and this, associated with unmethylated O6-methylguanine methyltransferase, can explain the lack of response to chemotherapy.

Correlative analysis of gene expression profile and prognosis in patients with gliomatosis cerebri.

BACKGROUND: In modern clinical neuro-oncology, the pathologic diagnoses are very challenging, creating significant clinical confusion and affecting therapeutic decisions and prognosis. METHODS: TP53 and PTEN gene sequences were analyzed, and microarray expression profiling was also performed. The authors investigated whether gene expression profiling, coupled with class prediction methodology, could be used to determine the prognosis of gliomatosis cerebri in a more consistent manner than standard pathology. RESULTS: The authors reported the results of a molecular study in 59 cases of gliomatosis cerebri, correlating these results with prognosis. The well-known prognostic factors of gliomas (ie, age, Karnofsky performance status, histology [grade 2 vs 3], and contrast enhancement) were found to be predictive of response or outcome in only a percentage of patients but not in all patients. The authors identified a 23-gene signature that was able to predict patient prognosis with microarray gene expression profiling. With the aim of producing a prognosis tool that is useful in clinical investigation, the authors studied the expression of this 23-gene signature by real-time quantitative polymerase chain reaction. Real-time expression values relative to these 23 gene features were used to build a prediction method able to distinguish patients with a good prognosis (those more likely to be responsive to therapy) from patients with a poor prognosis (those less likely to be responsive to therapy). CONCLUSIONS: The results of the current study demonstrated not only a strong association between gene expression patterns and patient survival, but also a robust replicability of these gene expression-based predictors.

Cervicothoracic postarachnoiditic hydrosyringomyelia secondary to pedicular hook dislocation: case report.

BACKGROUND: Pedicular hook dislocation is a rare complication of spinal instrumentation. We report on the first case of hydrosyringomyelia secondary to intracanalar hook displacement after scoliosis surgery. CASE DESCRIPTION: A 15-year-old girl presented to our institution with a 7-month history of persistent neck and occipital pain as well as numbness of the lower extremities and previous dorsolumbar instrumentation with dorsal pedicular hooks and lumbar screws. Magnetic resonance imaging showed intramedullary cystic cavity from C5 to T7, isointense to cerebrospinal fluid (CSF) on T1- and T2-weighted images. Computed tomographic scan showed intracanalar displacement of the left hook. On admission, the patient presented with mild weakness of the lower extremities, hypalgesia below the level of T4, and urinary disturbance. The patient underwent surgical hook removal, T4 laminectomy, and midline dural opening: the arachnoid membrane was found to be thick and adhered to the dura and dorsolateral spinal cord. The arachnoid scarring was dissected, and the cord was untethered. A small posterior-median myelotomy was performed, and a syringosubarachnoid catheter was placed into the subarachnoid space to restore CSF flow. CONCLUSIONS: Late intracanalar displacement of spinal devices is an event that may complicate spinal instrumentation for scoliosis. This case highlights the importance of correct spinal device positioning and that of careful follow-up after instrumentation to detect complications early. We also discuss the pathogenetic pathway of the postarachnoiditic syringomyelia in this case.