External validation of the Ruptured Arteriovenous Malformation Grading Scale (RAGS) in a multicenter adult cohort.

PURPOSE: While Ruptured Arteriovenous Malformation Grading Scale (RAGS) has recently been validated in children, the literature lacks validation on adults exclusively. Therefore, we aimed to determine the validity of RAGS on the external multicenter adult cohort and compare its accuracy with other scales. METHODS: A retrospective analysis was performed in five neurosurgical departments to extract patients who presented with the first episode of acute brain arteriovenous malformation (bAVM) rupture between 2012 and 2019. Standard logistic regression and area under the receiver operating curve (AUROC) calculations were performed to determine the value of the following scales: intracerebral hemorrhage (ICH), AVM-associated ICH (AVICH), Spetzler-Martin (SM), Supplemented SM (Supp-SM), Hunt and Hess (HH), Glasgow Coma Scale (GCS), World Federation of Neurological Surgeons (WFNS), and RAGS to predict change in categorical and dichotomized modified Rankin Scale (mRS) across three follow-up periods: within the 6 months, 6 months to 1 year, and above 1 year. RESULTS: Sixty-one individuals with a mean age of 43.6 years were included. The RAGS outperformed other grading scales during all follow-up time frames. It showed AUROC of 0.78, 0.74, and 0.71 at the first 6 months, between 6 and 12 months, and after 12 months of follow-up, respectively, when categorized mRS was applied, while corresponding values were 0.79, 0.76, and 0.73 for dichotomized mRS, respectively. CONCLUSION: The RAGS constitutes a reliable scale predicting clinical outcomes following bAVM rupture among adults. Furthermore, the RAGS proved its generalizability across medical centers with varying treatment preferences.

The prognostic value of emergency microsurgical clipping of ruptured anterior circulation aneurysms.

BACKGROUND: The treatment of aneurysmal subarachnoid hemorrhage poses a formidable challenge, given the high mortality rate and associated mortality. Current recommendations are for treatment to be initiated within 24 hours of diagnosis. METHODS: In our study, we compared a cohort of 66 patients who received prompt microsurgical treatment within 6 hours of diagnosis with a cohort of 51 patients who received prompt microsurgical treatment within 12 hours of diagnosis. RESULTS: The modified Rankin Scale was utilized to evaluate the follow-up of patients at 30 days, 12 months, and 18 months following surgery. We performed a parametric comparison of the distributions of the means of groups, and our results indicate that treatment within 6 hours of diagnosis results in a lower incidence of obstructive hydrocephalus and a more favorable outcome. CONCLUSIONS: A favorable outcome was observed in patients who were treated within 6 hours. The availability of a specialized vascular team ensures the highest levels of care.

Adult IDH Wild-Type Glioblastoma Ultrastructural Investigation Suggests a Possible Correlation between Morphological Biomarkers and Ki-67 Index.

Glioblastoma is an aggressive brain tumor with an average life expectancy between 14 and 16 months after diagnosis. The Ki-67 labeling index (LI), a measure of cellular proliferation, is emerging as a prognostic marker in GBM. In this study, we investigated the ultrastructure of glioblastoma tissue from 9 patients with the same molecular profile (adult IDH wild-type glioblastoma, wild-type ATRX, and positive for TP53 expression, GFAP expression, and EGFR overexpression) to find possible ultrastructural features to be used as biomarkers and correlated with the only parameter that differs among our samples, the Ki-67 LI. Our main results were the visualization of the anatomical basis of astrocyte-endothelial cells crosstalk; the ultrastructural in situ imaging of clusters of hyperactivated microglia cells (MsEVs); the ultrastructural in situ imaging of microglia cells storing lipid vesicles (MsLVs); the ultrastructural in situ imaging of neoplastic cells mitophagy (NCsM). The statistical analysis of our data indicated that MsEVs and MsLVs correlate with the Ki-67 LI value. We can thus assume they are good candidates to be considered morphological biomarkers correlating to Ki-67 LI. The role of NCsM instead must be further evaluated. Our study findings demonstrate that by combining ultrastructural characteristics with molecular information, we can discover biomarkers that have the potential to enhance diagnostic precision, aid in treatment decision-making, identify targets for therapy, and enable personalized treatment plans tailored to each patient. However, further research with larger sample sizes is needed to validate these findings and fully utilize the potential of ultrastructural analysis in managing glioblastoma.

Role of 1p/19q Codeletion in Diffuse Low-grade Glioma Tumour Prognosis.

BACKGROUND/AIM: In the latest 2021 WHO classification of central nervous system tumours (CNS), gliomas that present isocitrate dehydrogenase (IDH) mutations are defined as diffuse low-grade gliomas (DLGGs). IDH mutations are commonly observed in this tumour type. The Extent of Resection (EOR) positively influence survival; however, it is still debated whether the predictive value of EOR is independent of the 1p/19q co-deletion. We carried out a retrospective analysis on patients operated on for DLGG at the Sant'Andrea University Hospital Sapienza University of Rome, correlating the outcome with the presence of 1p/19q co-deletion and EOR. PATIENTS AND METHODS: The study examined 66 patients with DLGG who had undergone surgery for tumour resection between 2008 and 2018. Patients with DLGG were divided into two groups; diffuse astrocytoma (DA) in which 1p/19q codeletion is absent and oligodendroglioma (OG) in which 1p/19q codeletion is present. According to EOR, both groups were divided into two subgroups: subtotal resection (STR) and gross total resection (GTR). Three end-point variables were considered: overall survival (OS), progression-free survival (PFS) and time to malignant transformation (TMT). RESULTS: In the DA group, the GTR subgroup had an average OS of 81.6 months, an average PFS of 45.9 months and an average TMT of 63.6 months. After surgery, these patients had an average Karnofsky Performance Score (KPS) of 83.4. The STR subgroup had an average OS of 60.4 months, PFS was 38.7 months, and TMT was 46.4 months, post-operative KPS was 83.4. In contrast, in the OG group, the GTR averagely had 101.7 months of OS, 64.9 months of PFS, 80.3 months of TMT and an average post-operative KPS of 84.2, and the STR subgroup had an average of OS of 73.3 months, PFS of 48.2 months, TMT of 57.3 and an average postoperative KPS of 96.2. CONCLUSION: In patients affected by DLGGs, 1p/19q codeletion is significantly associated with prolonged survival and longer time-to-malignant transformation (TMT) compared to the absence of 1p/19q codeletion. Also, the extent of surgical resection (EOR) in DLGG patients has been confirmed as one of the main prognostic factors. However, its predictive value is substantially influenced by the presence of the 1p/19q codeletion.

Cortical atrophy in chronic subdural hematoma from ultra-structures to physical properties.

Several theories have tried to elucidate the mechanisms behind the pathophysiology of chronic subdural hematoma (CSDH). However, this process is complex and remains mostly unknown. In this study we performed a retrospective randomised analysis comparing the cortical atrophy of 190 patients with unilateral CSDH, with 190 healthy controls. To evaluate the extent of cortical atrophy, CT scan images were utilised to develop an index that is the ratio of the maximum diameter sum of 3 cisterns divided by the maximum diameter of the skull at the temporal lobe level. Also, we reported, for the first time, the ultrastructural analyses of the CSDH using a combination of immunohistochemistry methods and transmission electron microscopy techniques. Internal validation was performed to confirm the assessment of the different degrees of cortical atrophy. Relative Cortical Atrophy Index (RCA index) refers to the sum of the maximum diameter of three cisterns (insular cistern, longitudinal cerebral fissure and cerebral sulci greatest) with the temporal bones' greatest internal distance. This index, strongly related to age in healthy controls, is positively correlated to the preoperative and post-operative maximum diameter of hematoma and the midline shift in CSDH patients. On the contrary, it negatively correlates to the Karnofsky Performance Status (KPS). The Area Under the Receiver Operating Characteristics (AUROC) showed that RCA index effectively differentiated cases from controls. Immunohistochemistry analysis showed that the newly formed CD-31 positive microvessels are higher in number than the CD34-positive microvessels in the CSDH inner membrane than in the outer membrane. Ultrastructural observations highlight the presence of a chronic inflammatory state mainly in the CSDH inner membrane. Integrating these results, we have obtained an etiopathogenetic model of CSDH. Cortical atrophy appears to be the triggering factor activating the cascade of transendothelial cellular filtration, inflammation, membrane formation and neovascularisation leading to the CSDH formation.

Visual Aura Secondary to Supratentorial Lipomatous Meningioma: A Rare Case Report.

BACKGROUND/AIM: Lipomatous meningioma is a rare type of meningioma that is formed as the result of an accumulation of lipids inside the cell due to metabolic activity dysregulation. It differs from other types of meningiomas in its radiological and immunohistochemical characteristics. We report a rare case of a patient treated in our department for this particular type of meningioma who developed a type of migraine with the aura component as the first clinical symptom. CASE REPORT: A 55-year-old woman presented with a migraine and reported having phosphenes in recent years. Head Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) scans were performed; these showed an extensive hypodense and hypointense formation located in the left parieto-occipital region. This formation was implanted in the tentorium region, with a prevailingly adipose-type signal intensity. The patient underwent an occipital craniotomy with the total removal of the lesion. The histological examination indicated a lipomatous metaplastic meningioma. CONCLUSION: We reported the first case of a lipomatous meningioma presenting with a migraine with a visual aura. Seizures and headaches can be included as possible symptoms. According to the current literature, lipomatous meningiomas affect women more commonly than men. The patient of our reported case presented visual disturbances in the form of a visual aura, which occurred 10 years before finding the meningioma, and surgery dramatically improved the symptoms and quality of life.

En Bloc Resection of Solitary Brain Metastasis: The Role of Perilesional Edema.

BACKGROUND/AIM: The treatment of solitary brain metastasis is a challenging intervention since the incidence increases and prognosis is poor. This study investigated the role of perilesional edema in the overall mass effect of solitary brain metastasis. PATIENTS AND METHODS: We conducted a retrospective analysis on 88 patients with single supratentorial brain metastasis and concomitant perilesional edema undergoing en bloc resection. Each patient was evaluated for perilesional brain edema grading. We stratified patients into three groups based on the size of the metastatic lesion and the extent of perilesional edema. RESULTS: The grade of perilesional edema at 30 days after surgical removal did not correlate with the maximum diameter of the metastasis (Pearson's correlation 0.098, p=0.494). In patients with a maximal metastatic diameter ≤2 cm, the grade of perilesional edema before surgical treatment was 1.63 (STD 0.43), while 30 days after removal it was significantly reduced; 0.47 (STD 0.26), p<0.001. CONCLUSION: The overall mass effect of solitary supratentorial brain metastases is not correlated to the size of the lesion and the grade of the associated perilesional edema should be considered. Surgical en bloc resection can be considered the first choice of treatment in the presence of solitary metastasis ≤2 cm in maximal diameter but with high-grade edema, since this treatment reduces the overall mass effect.

Lipomatous Meningioma: Clinical-Pathological Findings, Imaging Characterisation and Correlations of a Rare Type of Meningioma.

Lipomatous meningioma (LM) is a form of metaplasia, originating from intracellular lipid bodies accumulation due to metabolic alterations. A comprehensive literature review was performed introducing further elements of evaluation. The parameters utilized were age, sex, location, clinical presentation, imaging features, treatment, and recurrences. Seizure and headache are the primary onsets of symptoms. Further LM clinical features, such as visual disturbances and visual epileptic seizures were examined. Symptoms may occur ten years prior to LM finding and it can resolve completely with the indicated surgery. LM computed tomography imaging analysis reveals hypodense regions due to the presence of fat content. On magnetic resonance imaging, the lesion displays hyperintense signal in T1-T2 with signal loss in the fat-suppression sequences. Immunohistochemically, lipidized meningioma cells are positive for Epithelial-Membrane Antigen, Vimentin, CD99, S-100 protein, and progesterone receptor. The recurrence risk rate of LM is estimated to be around 17%. Precise immune-histological findings have been correlated with imaging features to help with early diagnosis. A defined diagnosis of LM is a crucial factor in the choice of treatment.

Intramedullary Spinal Cord Metastasis Mimicking Astrocytoma: A Rare Case Report.

Intramedullary spinal cord metastases (ISCMs) are infrequent lesions. Their incidence is estimated to range from 0.9 to 2.1%, found in autopsies of cancer patients. However, as the life expectancy of malignant tumour patients constantly increases, the reported incidences of ISCMs are consequently rising. This report presents a case of the misdiagnosis of an anaplastic astrocytoma type of tumour due to its similarities to small-cell neuroendocrine carcinoma. Therefore, we would like to underline the importance of further investigation that could assist and support the surgeon in the making of the differential diagnosis. We present the clinical case of a 73-year-old woman with a solitary intramedullary spinal cord metastasis as the initial manifestation of a carcinoid type of tumour. The patient was admitted to our department while presenting a rapid onset of paraparesis. Magnetic resonance imaging was performed, which showed an intramedullary mass at the C2-C6 vertebral level with a heterogeneous contrast enhancement. In light of these findings, the patient underwent surgery for a partial tumour resection. The lesion resulted in being a small-cell neuroendocrine type of carcinoma. This peculiar type of tumour presents similar radiological characteristics to the anaplastic astrocytoma type, which is why our diagnostical mismatch occurred. This is the report of a rare case of solitary intramedullary spinal cord metastasis, which is the result of an initial presentation of a lung small-cell neuroendocrine type of carcinoma. We conclude that ISCMs should be regularly considered as a part of the differential diagnosis of intramedullary lesions, especially in the case of a rapid onset and deterioration of neurological symptoms.

Entrapment of the temporal horn: case series and systematic review of literature.

INTRODUCTION: Entrapment of the temporal horn (ETH) is a form of focal, obstructive hydrocephalus. Etiology and clinical presentation are diversified. Though known since 1947, standard treatment has not yet been defined. The objective of our study was to perform a systematic review on ETH. EVIDENCE ACQUISITION: Data from patients treated at our Institution from 2008 to 2019 were retrospectively collected and analyzed. A systematic PRISMA review of literature was also performed using PubMed and Google Scholar. The following search terms were used: "temporal horn entrapment" [All Fields] OR "trapped temporal horn" [All Fields]. EVIDENCE SYNTHESIS: One hundred and twenty-one cases (mean age 41 years; M/F ratio: 1/1) were analyzed. In 65 (vs. 56) cases (53.7% vs. 46.3%), ETH was not surgery related. Headache was the most common symptom (42%). "Major" treatments were ventriculoperitoneal/ventriculoatrial shunt (42 cases, 34.7%), and endoscopic ventriculocisternostomy (12 cases, 9.9%). In the first group, no perioperative complications were found, 39 patients (92.9%) had a favorable outcome, three patients (7.1%) died for the underlying disease, four cases (9.5%) went through revision; also considering the cases in which another procedure was performed as definitive treatment, there were six shunt failures (13.6%). In the second group, one case (8.3%) developed a deep intracerebral hemorrhage, 11 cases (91.6%) had a favorable long-term outcome, one case (8.3%) had a favorable short-term outcome; also considering the cases in which another procedure was performed as definitive treatment, there were six endoscopic ventriculocisternostomy failures (37.5%). CONCLUSIONS: Described as uncommon, ETH is probably underestimated. Early diagnosis and appropriate treatment are critical. VP shunt is still the most commonly performed treatment. Further randomized clinical trials are, however, needed to establish the gold standard.

First-in-man craniectomy and asportation of solitary cerebellar metastasis in COVID-19 patient: A case report.

INTRODUCTION: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak has an impact on the delivery of neurosurgical care, and it is changing the perioperative practice worldwide. We present the first case in the literature of craniectomy procedure and asportation of a solitary cerebellar metastasis of the oesophagus squamous carcinoma in a 77 years old woman COVID-19 positive. In these particular circumstances, we show that adequate healthcare resources and risk assessments are essential in the management of COVID-19 patients referred to emergency surgery. PRESENTATION OF CASE: The case here presented was treated in 2019 for squamous carcinoma of the oesophagus. In April 2020, she presented a deterioration of her clinical picture consisting of dysphagia, abdominal pain, hyposthenia and ataxia. A Head CT scan was performed, which showed the presence of a solitary cerebellar metastasis. Her associated SARS-CoV-2 positivity status represented the principal clinical concern throughout her hospitalisation. DISCUSSION: The patient underwent a suboccipital craniectomy procedure with metastasis asportation. She tested positive for SARS-CoV-2 in the pre- and post-operative phases, but she was not admitted to the intensive care unit because she did not present any respiratory complications. Her vital parameters and inflammation indexes fell within the reference ranges, and she was kept in isolation for 16 days in our neurosurgical unit following strict COVID-19 measures. She was asymptomatic and not treated for any of the specific and non-specific symptoms of COVID-19. CONCLUSION: This is the first case reported of solitary cerebellar metastasis of oesophagus carcinoma operated on a COVID-19 positive patient. It shows that asymptomatic COVID-19 positive patients can undergo major emergency surgeries without the risk of infecting the operating team if adequate Personal Protection Equipment (PPE) is used. The patient remained asymptomatic and did not develop the disease's active phase despite undergoing a stressful event such as a major emergency neurosurgical procedure. In the current crisis, a prophylactic COVID-19 screening test can identify asymptomatic patients undergoing major emergency surgery and adequate resource planning and Personal Protective Equipment (PPE) for healthcare workers can minimise the effect of the COVID-19 pandemic.

Retrospective and Randomized Analysis of Influence and Correlation of Clinical and Molecular Prognostic Factors in a Mono-Operative Series of 122 Patients with Glioblastoma Treated with STR or GTR.

Glioblastoma is a solid, infiltrating, and the most frequent highly malignant primary brain tumor. Our aim was to find the correlation between sex, age, preoperative Karnofsky performance status (KPS), presenting with seizures, and extent of resection (EOR) with overall survival (OS), progression-free survival (PFS), and postoperative KPS, along with the prognostic value of IDH1, MGMT, ATRX, EGFR, and TP53 genes mutations and of Ki67 through the analysis of a single-operator series in order to avoid the biases of a multi-operator series, such as the lack of homogeneity in surgical and adjuvant nonsurgical treatments. A randomized retrospective analysis of 122 patients treated by a single first operator at Sapienza University of Rome was carried out. After surgery, patients followed standard Stupp protocol treatment. Exclusion criteria were: (1) patients with primary brainstem and spinal cord gliomas and (2) patients who underwent partial resections (resection < 90%) or a biopsy exclusively for diagnostic purposes. Statistical analysis with a simultaneous regression model was carried out through the use of SPSS 25® (IBM). Results showed statistically significant survival increase in four groups: (1) patients treated with gross total resection (GTR) (p < 0.030); (2) patients with mutation of IDH1 (p < 0.0161); (3) patients with methylated MGMT promoter (p < 0.005); (4) patients without EGFR amplification or EGFRvIII mutation (p < 0.035). Higher but not statistically significant survival rates were also observed in: patients <75 years, patients presenting with seizures at diagnosis, patients affected by lesions in noneloquent areas, as well as in patients with ATRX gene mutation and Ki-67 < 10%.

Minimally Invasive Surgery for the Treatment of Traumatic Monosegmental Thoracolumbar Burst Fractures: Clinical and Radiologic Outcomes of 144 Patients With a 6-year Follow-Up Comparing Two Groups With or Without Intermediate Screw.

STUDY DESIGN: This was a retrospective study of the clinical and radiologic outcomes of traumatic thoracolumbar (TL) burst fractures. OBJECTIVES: We aimed to evaluate the clinical and radiologic outcomes after 6 years of follow-up of 144 patients with monosegmental TL burst fractures treated with percutaneous short-segment pedicle screw fixation, comparing two groups with versus without placement of an intermediate screw at the fractured vertebra. SUMMARY OF BACKGROUND DATA: Traumatic TL fractures are the most common vertebral fractures, especially at the TL junction (T10-L2). Minimally invasive surgery (MIS) is a valuable treatment option for traumatic TL burst fractures. MATERIALS AND METHODS: The clinical outcomes and radiologic parameters (Cobb angle, midsagittal index, and sagittal index) of 144 patients with traumatic monosegmental TL fractures treated with MIS were evaluated preoperatively, postoperatively, and after 3 and 6 years of follow-up. Patients were categorized into a nonintermediate screw group (nISG) and an intermediate screw group (ISG), and the groups were compared. RESULTS: There were 71 patients (49.3%) in the nISG and 73 patients (50.7%) in the ISG. The radiologic parameters improved significantly more from the preoperative evaluation to the 6-year follow-up in the ISG than in the nISG (P<0.025). There were no significant differences in the mean Oswestry Disability Index (ODI) and Visual Analog Scale scores at the 6-year follow-up between the ISG and the nISG: 15.6% (ISG) versus 16.8% (nISG) for ODI (P<0.1) and 2.2 (ISG) versus 2.4 (nISG) for Visual Analog Scale score (P<0.85) (P<0.73). CONCLUSIONS: MIS showed good clinical outcomes 6 years after surgery in both the ISG and the nISG. The additional intermediate screw significantly improved radiologic parameters but not clinical outcomes.

Completely Thrombosed Distal Middle Cerebral Artery Aneurysm Mimicking a Cavernous Angioma: Case Report and Review of the Literature.

BACKGROUND: Distal middle cerebral artery (MCA) aneurysms originate from branches of MCA distal to its main bifurcation or the peripheral branches. Distal MCA aneurysms are uncommon compared with saccular aneurysms, which develop along the proximal trunks of MCA. However, thrombotic aneurysms, characterized by organized intraluminal thrombus and solid mass, are frequently in the large and giant size range, whereas complete thrombosis of non-giant MCA aneurysms is very rare. CASE PRESENTATION: We present the clinical case of a 53 years-old woman with a completely thrombosed medium distal MCA aneurysm mimicking a cavernous angioma. She came to our emergency department after the onset of tinnitus and persistent headache. Magnetic resonance imaging performed subsequently showed a nodular mass surrounded by edema located in the temporal lobe with a homogeneous peripheral contrast enhancement. Furthermore, angiography showed regular flow in the MCA and confirmed the diagnosis of cavernous angioma. The patient underwent surgery, and the lesion was found to be a thrombosed aneurysm originating from the distal temporal branch of the left MCA (M2 segment). CONCLUSIONS: To our knowledge, this is the first report of a thrombosed distal medium MCA aneurysm that mimicked a cavernous angioma. The completely thrombosed aneurysm can be confused with intracranial lesions or cavernous malformations, which can have similar radiographic features without angiographic anomalies, so it is mandatory to consider the possibility of a thrombosed aneurysm for a correct differential diagnosis.