Search

Objective: To evaluate the demographic, clinical, radiological features, and surgical outcomes of 31 patients who underwent surgery for cranial cavernous malformations (CCM). Methods: A retrospective analysis was conducted on 31 patients who underwent CCM between July 2020 and January 2024. Data included demographic and clinical, radiological, intraoperative, and histopathological findings, and postoperative complications. Detailed neurological examinations were performed before and after surgery. All patients underwent preoperative computed tomography and magnetic resonance imaging (MRI), and the lesions were evaluated using the Zabramski classification. Surgeries aimed at total resection using neuronavigation, intraoperative MRI, and ultrasonography when needed. The average follow-up duration was 25.3 months. Results: Thirty-three transcranial microsurgical excisions were performed in 31 patients (48% male, 52% female; mean age 30.8 years). Lesions were most commonly parietal (39%) and frontal (26%). Clinical findings included epilepsy (62%), headache (20%), and focal neurological deficits (6%). According to Zabramski, 42% were type I, 52% were type II, and 6% were type III. Total excision was achieved in 94% of the patients, with 6% requiring a second operation. Postoperative seizures were absent in 74% of patients with epilepsy. The average length of hospital stay was 5.7 days, with no permanent neurological deterioration or mortality. Conclusion: Surgical resection is effective for treating symptomatic CCM. Total resection cases should be closely monitored due to the risk of recurrence. A conservative approach is recommended for asymptomatic, deep-seated, or eloquent lesions, and radiosurgery is considered for high surgical risk cases. Multidisciplinary and personalized treatment protocols can improve outcomes in patients with CCM. [ABSTRACT FROM AUTHOR]

Background Intracranial cavernous malformations (CMs), commonly known as cavernomas or cavernous angiomas, are low-flow, well-circumscribed vascular lesions composed of sinusoidal spaces lined by a single layer of endothelium and separated by a collagenous matrix without elastin, smooth muscle, or other vascular wall elements. A diameter greater than 3 cm for a CM is unlikely. These lesions may have atypical appearances on magnetic resonance imaging (MRI). MRI with advanced techniques such as a susceptibility-weighted image or T2-gradient echo, a diffusion-weighted image and corresponding apparent diffusion coefficient map, and diffusion tensor tractography have revolutionized the diagnostic approach to these lesions.

Cavernous angiomas (CA) affecting cranial nerves (CN) are exceptionally rare. Most reported cases involve the optic nerves, with even fewer occurrences in other CN. In this report, the authors describe a case of an extra-axial CA affecting the oculomotor nerve, which was managed conservatively. The authors also review the outcomes of other therapeutic options based on cases documented in the existing literature. Case report: 36-year-old woman presented to the Emergency Department with a headache in the left temporal region, predominantly at night, along with mild left palpebral ptosis and binocular diplopia of 2 months duration. Brain MRI revealed a lesion exhibiting typical characteristics of CA in the left lateral region of the interpeduncular cistern, in close contact with the left oculomotor nerve. After considering treatment options and in consultation with the patient, a conservative management plan with periodic MRI follow-up was chosen. After 2 years of follow-up, the patient showed favorable progress. Although exceptional, CA should be considered in the differential diagnosis of other more common extra-axial lesions involving CN. The therapeutic management of a CA of the oculomotor nerve remains controversial due to the limited number of cases described in the literature. The authors suggest that for individuals with asymptomatic or mildly symptomatic CA affecting the oculomotor nerve, a conservative treatment approach is the most suitable choice to preserve neurological function. In cases characterized by progressive symptoms, a history of recurrent bleeding, or evidence of lesion enlargement on sequential imaging assessments, total microsurgical resection should be considered. [ABSTRACT FROM AUTHOR]

Background Intracranial cavernous malformations (CMs), commonly known as cavernomas or cavernous angiomas, are low-flow, well-circumscribed vascular lesions composed of sinusoidal spaces lined by a single layer of endothelium and separated by a collagenous matrix without elastin, smooth muscle, or other vascular wall elements. A diameter greater than 3 cm for a CM is unlikely. These lesions may have atypical appearances on magnetic resonance imaging (MRI). MRI with advanced techniques such as a susceptibility-weighted image or T2-gradient echo, a diffusion-weighted image and corresponding apparent diffusion coefficient map, and diffusion tensor tractography have revolutionized the diagnostic approach to these lesions. Materials and Method The present study reviews the etiopathogenesis, clinical manifestations, MRI strategy, and MRI appearances of the CMs, with a few examples of the giant CMs from our archive. Results Intracranial giant CMs may have unexpected locations, sizes, numbers, and varied imaging appearances due to repeated hemorrhages, unusual enhancement patterns, intense perifocal edema, and unusual associations, making the differential diagnosis difficult. Conclusion Familiarity with the MRI appearances of the giant intracranial CMs and the differential diagnosis improves diagnostic accuracy and patient management. [ABSTRACT FROM AUTHOR]

Abstract Background Cavernous malformations can occur throughout the cerebral nervous system, most commonly located in the supratentorial compartment. The intramedullary location is rare and accounts for approximately 2.4% to 5% of all spinal vascular tumors. The cervical cord location and its clinical manifestations are underreported. Methods The authors report a case of spinal cord compression at the C5–C6 level by a cervical intramedullary cavernoma with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and meta-analysis guidelines. Relevant studies (1980 to 2023) that reported patients with cervical intramedullary cavernoma were identified from PubMed databases. Results A total of 29 studies reporting 423 patients were included in this study with a mean age of 40.5 ± 6.3 years old. The overall male-to-female ratio was 1:2.5 with a median duration of follow-up of 46 months [18.5–63.9]. The type of resection as well as the patient’s outcome was also reported. Conclusion Intramedullary cavernous angiomas are rare. Early surgical total resection of the symptomatic lesions in adequate conditions is crucial for good outcomes.

Background: Cavernous malformations can occur throughout the cerebral nervous system, most commonly located in the supratentorial compartment. The intramedullary location is rare and accounts for approximately 2.4% to 5% of all spinal vascular tumors. The cervical cord location and its clinical manifestations are underreported. Methods: The authors report a case of spinal cord compression at the C5–C6 level by a cervical intramedullary cavernoma with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and meta-analysis guidelines. Relevant studies (1980 to 2023) that reported patients with cervical intramedullary cavernoma were identified from PubMed databases. Results: A total of 29 studies reporting 423 patients were included in this study with a mean age of 40.5 ± 6.3 years old. The overall male-to-female ratio was 1:2.5 with a median duration of follow-up of 46 months [18.5–63.9]. The type of resection as well as the patient's outcome was also reported. Conclusion: Intramedullary cavernous angiomas are rare. Early surgical total resection of the symptomatic lesions in adequate conditions is crucial for good outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Deep cavernomas of eloquent areas, located in the region of the basal nuclei and thalamus, account for 9 to 36% of these encephalic vascular malformations. Internal capsule cavernomas are particularly challenging, as they are surrounded by important projection fibers and their manipulation can lead to permanent deficits. To demonstrate through surgical cases that cavernomas of the internal capsule can be approached by frontal craniotomy, via the superior frontal sulcus, in a curative manner and with low morbidity. Methods: We presented two cases of cavernomas of the internal capsule operated, whose treatment was microsurgical resection via frontal craniotomy and access to the lesion via the superior frontal sulcus, described step-by-step. To elucidate the rationale behind the decision, we used preoperative images with an emphasis on the patients’ tractography and the importance of comparing these images with anatomical specimens dissected in the neuroanatomy and microsurgery laboratory. Results: The two cases of internal capsule cavernomas, one in the anterior limb and the other in the posterior limb, were treated surgically via the superior frontal sulcus. Discussion: Both patients showed radiological cure and clinical improvement in the post-operative segment. The patient consented to the procedure and to the publication of his/her image. Treatment of internal capsule cavernomas via the superior frontal sulcus has proven to be a safe and effective option. [ABSTRACT FROM AUTHOR]

Background: Patients with supratentorial cavernous malformations (SCMs) commonly present with seizures. First-line treatments for cavernoma-related epilepsy (CRE) include conservative management (antiepileptic drugs (AEDs)) and surgery. We compared seizure outcomes of CRE patients after early (≤6 months) vs. delayed (>6 months) surgery. Methods: We compared outcomes of CRE patients with SCMs surgically treated at our large-volume cerebrovascular center (1 January 2010–31 July 2020). Patients with 1 sporadic SCM and ≥1-year follow-up were included. Primary outcomes were International League Against Epilepsy (ILAE) class 1 seizure freedom and AED independence. Results: Of 63 CRE patients (26 women, 37 men; mean ± SD age, 36.1 ± 14.6 years), 48 (76%) vs. 15 (24%) underwent early (mean ± SD, 2.1 ± 1.7 months) vs. delayed (mean ± SD, 6.2 ± 7.1 years) surgery. Most (32 (67%)) with early surgery presented after 1 seizure; all with delayed surgery had ≥2 seizures. Seven (47%) with delayed surgery had drug-resistant epilepsy. At follow-up (mean ± SD, 5.4 ± 3.3 years), CRE patients with early surgery were more likely to have ILAE class 1 seizure freedom and AED independence than those with delayed surgery (92% (44/48) vs. 53% (8/15), p = 0.002; and 65% (31/48) vs. 33% (5/15), p = 0.03, respectively). Conclusions: Early CRE surgery demonstrated better seizure outcomes than delayed surgery. Multicenter prospective studies are needed to validate these findings. [ABSTRACT FROM AUTHOR]

Cavernous angiomas (CAs) are benign vascular malformations predominantly seen in the brain parenchyma and therefore referred to as intra-axial. Extra-axial dural-based cavernous angiomas, on the other hand, are rare vascular lesions found outside of the brain parenchyma. They occur in the middle fossa and may be easily misdiagnosed as meningiomas due to their extra-axial location. In addition, CAs that are located outside the middle fossa, such as in the convexity, have a better prognosis since they are more surgically accessible. Surgical resection is the main treatment of choice in CAs. However, other options, such as embolization and radiotherapy, may also be considered therapeutic choices or additive treatment options. The pathogenesis of CA and the involvement of other factors (genetics or environmental factors) are still unknown and require further investigation. We are presenting a young man who presented for evaluation of seizure-like events without any family history of neurologic conditions. The physical examination was unremarkable except for a slightly antalgic gait. Imaging studies showed an extra-axial left tentorial mass suggestive of a meningioma, hemangiopericytoma, or other extra-axial lesions. The lesion was resected where its vascular nature was mentioned initially, and the histology proved the diagnosis of cavernous angioma. Here we give an overview of the known pathogenesis, causes, clinical features, and diagnostic and therapeutic options in CA. Better knowledge about CA, its causes, clinical features, and treatment options would help clinicians in early diagnosis and patient management.

Cavernous angiomas (CAs) are benign vascular malformations predominantly seen in the brain parenchyma and therefore referred to as intra-axial. Extra-axial dural-based cavernous angiomas, on the other hand, are rare vascular lesions found outside of the brain parenchyma. They occur in the middle fossa and may be easily misdiagnosed as meningiomas due to their extra-axial location. In addition, CAs that are located outside the middle fossa, such as in the convexity, have a better prognosis since they are more surgically accessible. Surgical resection is the main treatment of choice in CAs. However, other options, such as embolization and radiotherapy, may also be considered therapeutic choices or additive treatment options. The pathogenesis of CA and the involvement of other factors (genetics or environmental factors) are still unknown and require further investigation. We are presenting a young man who presented for evaluation of seizure-like events without any family history of neurologic conditions. The physical examination was unremarkable except for a slightly antalgic gait. Imaging studies showed an extra-axial left tentorial mass suggestive of a meningioma, hemangiopericytoma, or other extra-axial lesions. The lesion was resected where its vascular nature was mentioned initially, and the histology proved the diagnosis of cavernous angioma. Here we give an overview of the known pathogenesis, causes, clinical features, and diagnostic and therapeutic options in CA. Better knowledge about CA, its causes, clinical features, and treatment options would help clinicians in early diagnosis and patient management. [ABSTRACT FROM AUTHOR]

Cerebral cavernous malformations (CCMs) are vascular lesions associated with seizures, hemorrhage, and neurologic deficits. The familial form of CCM constitutes ≈20% of cases and presents with multifocal lesions in the brain and spinal cord, whereas the more common sporadic form typically involves a single lesion. Treatments of CCM include surgical resection and stereotactic radiosurgery, as well as management of symptoms (eg, seizures). Surgical resection or irradiation of lesions in eloquent areas requires careful consideration because of the potential for morbidity and mortality, and these treatments are not advised for asymptomatic lesions. The purpose of this narrative review is to describe the current state of treatments for CCM, with an emphasis on potential clinically relevant pharmacologic treatments aimed at targeting aberrant molecular signaling associated with CCM. Literature was identified through PubMed using search terms related to treatments of CCMs. In endothelial cells, overactivation of RhoA/Rho‐associated kinase contributes to disruption of cell‐cell junctions and a shift to a senescence‐associated secretory phenotype, which leads to inflammation, migration, and invasiveness of mutant endothelial cells. Specific (NRL‐1049) and nonspecific (fasudil, statins) inhibition of Rho‐associated kinase has shown effectiveness to reduce lesion burden in mouse models of CCM. A phase 1/2 clinical trial is currently underway to investigate the efficacy of atorvastatin in patients with CCM, and a first‐in‐human clinical trial to evaluate safety, tolerability, and pharmacokinetic parameters of NRL‐1049 began in 2023. The β‐blocker propranolol and the superoxide dismutase mimetic REC‐994 have also shown effectiveness in attenuating lesion burden in preclinical studies. Results from a pilot phase 2 clinical trial of propranolol support further investigation in an adequately powered trial, and the safety, pharmacokinetics, and potential efficacy of REC‐994 are currently being evaluated in a phase 2 clinical trial. Additional agents have been used solely in preclinical models and require clinical evaluation.

IntroductionDeep cavernomas of eloquent areas, located in the region of the basal nuclei and thalamus, account for 9 to 36% of these encephalic vascular malformations. Internal capsule cavernomas are particularly challenging, as they are surrounded by important projection fibers and their manipulation can lead to permanent deficits. To demonstrate through surgical cases that cavernomas of the internal capsule can be approached by frontal craniotomy, via the superior frontal sulcus, in a curative manner and with low morbidity.MethodsWe presented two cases of cavernomas of the internal capsule operated, whose treatment was microsurgical resection via frontal craniotomy and access to the lesion via the superior frontal sulcus, described step-by-step. To elucidate the rationale behind the decision, we used preoperative images with an emphasis on the patients’ tractography and the importance of comparing these images with anatomical specimens dissected in the neuroanatomy and microsurgery laboratory.ResultsThe two cases of internal capsule cavernomas, one in the anterior limb and the other in the posterior limb, were treated surgically via the superior frontal sulcus.DiscussionBoth patients showed radiological cure and clinical improvement in the post-operative segment. The patient consented to the procedure and to the publication of his/her image. Treatment of internal capsule cavernomas via the superior frontal sulcus has proven to be a safe and effective option.

Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels, lined by a single layer of endothelium. Acquired form of these lesions was related to previous radiation-therapy, and might be related to its pathophysiology, because the vascular endothelial growth factor has higher expression in the immature brain of the child. Consequently, although it occurs mainly in the paediatric population, it can happen in adults. We report a case of radiation-induced CCM in an adult patient presenting with aggressive behaviour and cerebral haemorrhage. [ABSTRACT FROM AUTHOR]

BackgroundCerebral cavernous angioma (CA) is a capillary microangiopathy predisposing more than a million Americans to premature risk of brain hemorrhage. CA with recent symptomatic hemorrhage (SH), most likely to re-bleed with serious clinical sequelae, is the primary focus of therapeutic development. Signaling aberrations in CA include proliferative dysangiogenesis, blood-brain barrier hyperpermeability, inflammatory/immune processes, and anticoagulant vascular domain. Plasma levels of molecules reflecting these mechanisms and measures of vascular permeability and iron deposition on magnetic resonance imaging are biomarkers that have been correlated with CA hemorrhage.ObjectiveTo optimize these biomarkers to accurately diagnose cavernous angioma with symptomatic hemorrhage (CASH), prognosticate the risk of future SH, and monitor cases after a bleed and in response to therapy.MethodsAdditional candidate biomarkers, emerging from ongoing mechanistic and differential transcriptome studies, would further enhance the sensitivity and specificity of diagnosis and prediction of CASH. Integrative combinations of levels of plasma proteins and characteristic micro-ribonucleic acids may further strengthen biomarker associations. We will deploy advanced statistical and machine learning approaches for the integration of novel candidate biomarkers, rejecting noncorrelated candidates, and determining the best clustering and weighing of combined biomarker contributions.Expected outcomesWith the expertise of leading CA researchers, this project anticipates the development of future blood tests for the diagnosis and prediction of CASH to clinically advance towards precision medicine.DiscussionThe project tests a novel integrational approach of biomarker development in a mechanistically defined cerebrovascular disease with a relevant context of use, with an approach applicable to other neurological diseases with similar pathobiologic features.

Background: Patients with supratentorial cavernous malformations (SCMs) commonly present with seizures. First-line treatments for cavernoma-related epilepsy (CRE) include conservative management (antiepileptic drugs (AEDs)) and surgery. We compared seizure outcomes of CRE patients after early (≤6 months) vs. delayed (>6 months) surgery. Methods: We compared outcomes of CRE patients with SCMs surgically treated at our large-volume cerebrovascular center (1 January 2010–31 July 2020). Patients with 1 sporadic SCM and ≥1-year follow-up were included. Primary outcomes were International League Against Epilepsy (ILAE) class 1 seizure freedom and AED independence. Results: Of 63 CRE patients (26 women, 37 men; mean ± SD age, 36.1 ± 14.6 years), 48 (76%) vs. 15 (24%) underwent early (mean ± SD, 2.1 ± 1.7 months) vs. delayed (mean ± SD, 6.2 ± 7.1 years) surgery. Most (32 (67%)) with early surgery presented after 1 seizure; all with delayed surgery had ≥2 seizures. Seven (47%) with delayed surgery had drug-resistant epilepsy. At follow-up (mean ± SD, 5.4 ± 3.3 years), CRE patients with early surgery were more likely to have ILAE class 1 seizure freedom and AED independence than those with delayed surgery (92% (44/48) vs. 53% (8/15), p = 0.002; and 65% (31/48) vs. 33% (5/15), p = 0.03, respectively). Conclusions: Early CRE surgery demonstrated better seizure outcomes than delayed surgery. Multicenter prospective studies are needed to validate these findings.

Vascular malformations of the brain are aberrant vascular connections that are most likely congenital. Cerebral vascular malformations are the umbrella term for multiple conditions, each with different symptoms, signs, and imaging characteristics. These conditions are (1) Arteriovenous malformations, abnormal arteries, and veins; (2) Dural arteriovenous fistula; (3) Developmental venous anomalies; (4) Cavernous malformations, enlarged blood-filled spaces; (5) Cavernous angiomas, abnormal veins; (6) capillary telangiectasias, enlarged capillary-sized vessels; (7) vein of Galen malformations; and (8) mixed malformations. It is important to study the complications of each and their mimics to make an accurate diagnosis. Various imaging features of different vascular malformations seen on MRI are discussed that which would aid in diagnosis and planning management. [ABSTRACT FROM AUTHOR]

Background and purpose: Language reorganization has been described in brain lesions with respect to their location and timing, but little is known with respect to their etiology. We used fMRI to investigate the effects of different types of left hemisphere lesions (GL = gliomas, TLE = temporal lobe epilepsy and CA = cavernous angioma) on the topographic intra-hemispheric language plasticity, also considering their location. Methods: Forty-seven right-handed patients with 3 different left hemisphere lesions (16 GL, 15 TLE and 16 CA) and 17 healthy controls underwent BOLD fMRI with a verb-generation task. Euclidean distance was used to measure activation peak shifts among groups with respect to reference Tailarach coordinates of Inferior Frontal Gyrus, Superior Temporal Sulcus and Temporo-Parietal Junction. Mixed-model ANOVAs were used to test for differences in activation peak shifts. Results: Significant activation peak shifts were found in GL patients with respect both to HC and other groups (TLA and CA). In addition, in the same group of patients a significant effect of tumor location (anterior or posterior) was detected. Conclusions: We demonstrated that intra-hemispheric language plasticity is influenced by the type of lesion affecting the left hemisphere and that fMRI is especially valuable in the preoperative assessment of such reorganization in glioma patients. [ABSTRACT FROM AUTHOR]

Background Spinal intramedullary cavernous angioma is a rare form of spinal cord tumor that is associated with myelopathy and significant morbidity and surgical treatment is almost always required. Case We report a case of spinal intramedullary cavernous angioma in a pain clinic with initial symptoms of unilateral interdigital space sensory change. Morton’s neuroma and piriformis syndrome were clinically suspected, however, symptoms acutely aggravated, and paraplegia developed following the patient’s COVID-19 vaccination. Vaccine-associated side effects were ruled out and spinal intramedullary cavernous angioma was confirmed through magnetic resonance image. The patient underwent surgery for complete mass excision. Conclusions Recent reports of COVID-19 vaccine-associated side effects have raised sensitive concerns to both health care providers and the public, that in some cases when the symptoms coincide with vaccination history, it may delay time-sensitive diagnosis and treatment and spend unnecessary costs.

Background and purpose: We aimed to assess the course and predictors of functional outcome after single and multiple intracerebral hemorrhage (ICH) in pediatric patients with cerebral cavernous malformations (CCMs) and to conduct a risk assessment of a third bleed during the first follow‐up year after second ICH. Methods: We included patients aged ≤18 years with complete baseline characteristics, a magnetic resonance imaging dataset, ≥1 CCM‐related ICH and ≥1 follow‐up examination, who were treated between 2003 and 2021. Neurological functional status was obtained using modified Rankin Scale scores at diagnosis, before and after each ICH, and at last follow‐up. Kaplan–Meier analysis was performed to determine the cumulative 1‐year risk of third ICH. Results: A total of 55 pediatric patients (median [interquartile range] age 12 [11] years) were analyzed. Univariate analysis identified brainstem cavernous malformation (BSCM; p = 0.019) as a statistically significant predictor for unfavorable outcome after second ICH. Outcome after second ICH was significantly worse in 12 patients (42.9%; p = 0.030) than after first ICH and in five patients (55.6%; p = 0.038) after a third ICH compared to a second ICH. Cumulative 12‐month risk of rebleeding during the first year after a second ICH was 10.7% (95% confidence interval 2.8%–29.37%). Conclusions: Pediatric patients with a BSCM have a higher risk of worse outcome after second ICH. Functional outcome improves over time after an ICH but worsens following each ICH compared to baseline or previous ICH. Second bleed was associated with neurological deterioration compared to initial ICH, and this deteriorated further after a third ICH. [ABSTRACT FROM AUTHOR]

Purpose: Spinal cavernous malformations (SCM) present a risk for intramedullary hemorrhage (IMH), which can cause severe neurologic deficits. Patient selection and time of surgery have not been clearly defined. Methods: This observational study included SCM patients who underwent surgery in our department between 2003 and 2021. Inclusion required baseline clinical factors, magnetic resonance imaging studies, and follow-up examination. Functional outcome was assessed using the Modified McCormick scale score. Results: Thirty-five patients met the inclusion criteria. The mean age was 44.7 ± 14.5 years, and 60% of the patients were male. In univariate analysis, the unfavorable outcome was significantly associated with multiple bleeding events (p =.031), ventral location of the SCM (p =.046), and incomplete resection (p =.028). The time between IMH and surgery correlated with postoperative outcomes (p =.004), and early surgery within 3 months from IMH was associated with favorable outcomes (p =.033). This association remained significant in multivariate logistic regression analysis (p =.041). Conclusions: Removal of symptomatic SCM should be performed within 3 months after IMH when gross total resection is feasible. Patients with ventrally located lesions might be at increased risk for postoperative deficits. [ABSTRACT FROM AUTHOR]

Introduction: Developmental venous anomalies (DVAs) are considered variants of normal transmedullary veins. Their association with cavernous malformations is reported to increase the risk of hemorrhage. Expert consensus recommends meticulous planning with MR imaging, use of anatomical "safe zones", intraoperative monitoring of long tracts and cranial nerve nuclei, and preservation of theDVA as key to avoiding complications in brainstemcavernomamicrosurgery. Symptomatic outflow restriction of DVA is rare, with the few reported cases in the literature restricted to DVAs in the supratentorial compartment. Case: We present a case report of the resection of a pontine cavernoma complicated by delayed outflow obstruction of the associated DVA. A female patient in her 20's presented with progressive left-sided hemisensory disturbance and mild hemiparesis. MRI revealed two pontine cavernomas associated with interconnected DVA and hematoma. The symptomatic cavernoma was resected via the infrafacial corridor. Despite the preservation of the DVA, the patient developed delayed deterioration secondary to venous hemorrhagic infarction. We discuss the imaging and surgical anatomy pertinent to brainstem cavernoma surgery, as well as the literature exploring the management of symptomatic infratentorial DVA occlusion. Conclusion: Delayed symptomatic pontine venous congestive edema is extremely rare following cavernoma surgery. DVA outflow restriction from a post-operative cavity, intraoperativemanipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstemvenous anatomy, and "safe entry zones" will further elucidate the etiology of and the effcacious treatment for this complication. [ABSTRACT FROM AUTHOR]

Symptoms, imaging features, and potential biopsy of intra-axial brain lesions are typically used to guide management and potentially render it more effective. Yet some cases exhibit atypical features that elude classification even in the light of histopathology. We present a case of a middle-aged man with a subcortical left frontal lesion with associated new seizures and lesional progression. The patient underwent biopsy demonstrating hyalinized vessels, chronic hemorrhage, and gliosis without significant proliferative activity. Molecular profiling was negative for BRAF and other gene variants associated with pilocytic astrocytoma, and PIK3CA variants described in cerebral cavernous malformations. Findings were not typical for any known vascular or neoplastic lesion. Lesion was treated with image guided thermal ablation appropriate for vascular or neoplastic etiology, with regression on imaging and no symptomatic recurrence. This illustrates a pragmatic approach to tumor like vascular anomalies, even in the absence of specific diagnostic classification.

BACKGROUND:Brain cavernous angiomas with symptomatic hemorrhage (CASH) are uncommon but exact a heavy burden of neurological disability from recurrent bleeding, for which there is no proven therapy. Candidate drugs to stabilize the CASH lesion and prevent rebleeding will ultimately require testing of safety and efficacy in multisite clinical trials. Much progress has been made in understanding the epidemiology of CASH, and novel biomarkers have been linked to the biological mechanisms and clinical activity in lesions. Yet, the ability to enroll and risk-stratify CASH subjects has never been assessed prospectively at multiple sites. Biomarkers and other outcomes have not been evaluated for their sensitivity and reliability, nor have they been harmonized across sites. OBJECTIVE:To address knowledge gaps and establish a research network as infrastructure for future clinical trials, through the Trial Readiness grant mechanism, funded by National Institute of Neurological Disorders and Stroke/National Institutes of Health. METHODS:This project includes an observational cohort study to assess (1) the feasibility of screening, enrollment rates, baseline disease categorization, and follow-up of CASH using common data elements at multiple sites, (2) the reliability of imaging biomarkers including quantitative susceptibility mapping and permeability measures that have been shown to correlate with lesion activity, and (3) the rates of recurrent hemorrhage and change in functional status and biomarker measurements during prospective follow-up. EXPECTED OUTCOMES:We propose a harmonized multisite assessment of enrollment rates of CASH, baseline features relevant to stratification in clinical trials, and follow-up assessments of functional outcomes in relation to clinical bleeds. We introduce novel biomarkers of vascular leak and hemorrhage, with firm mechanistic foundations, which have been linked to clinical disease activity. We shall test their reliability and validity at multiple sites, and assess their changes over time, with and without clinical rebleeds, hence their fitness as outcome instruments in clinical trials. DISCUSSION:The timing cannot be more opportune, with therapeutic targets identified, exceptional collaboration among researchers and the patient community, along with several drugs ready to benefit from development of a path to clinical testing using this network in the next 5 years.

Objective We performed a retrospective observational study to demonstrate the surgical risks and long-term prognoses of intramedullary tumors in Japan using a multicenter registry authorized by the Neurospinal Society of Japan. Methods Data from 1,033 consecutive patients with intramedullary tumors, treated between 2009 and 2020, were collected from 58 centers. Patients with spinal lipomas or myxopapillary ependymomas were excluded. Patient characteristics, clinical presentations, imaging characteristics, treatments, and outcomes were analyzed. The modified McCormick scale was used to classify functional status. Survival was described using Kaplan-Meier curves, and multivariable logistic regression analyses were performed. Results The mean age of the patients was 48.4 years. Data of 361 ependymomas, 196 hemangioblastomas, 168 astrocytic tumors, 160 cavernous malformations, and the remaining 126 cases including subependymomas, metastases, schwannomas, capillary hemangiomas, and intravascular B-cell lymphomas were analyzed. Twenty-two patients were undiagnosed. The mean follow-up duration was 46.1 ± 38.5 months. Gross total tumor removal was achieved in 672 tumors (65.1%). On the modified McCormick scale, 234 patients (22.7%) had worse postoperative grades at the time of discharge. However, neurological status gradually improved. At 6 months postoperatively, 251 (27.5%), 500 (54.9%), and 160 patients (17.6%) had improved, unchanged, and worsened grades, respectively. Preoperative functional status, gross total tumor removal, and histopathological type were significantly associated with mortality and functional outcomes. Conclusion Our findings demonstrate better postoperative functional outcomes in patients with fewer preoperative neurological deficits. Degree of resection, postoperative treatments, and prognoses are closely related to the histology of intramedullary tumors.

IntroductionDevelopmental venous anomalies (DVAs) are considered variants of normal transmedullary veins. Their association with cavernous malformations is reported to increase the risk of hemorrhage. Expert consensus recommends meticulous planning with MR imaging, use of anatomical “safe zones”, intraoperative monitoring of long tracts and cranial nerve nuclei, and preservation of the DVA as key to avoiding complications in brainstem cavernoma microsurgery. Symptomatic outflow restriction of DVA is rare, with the few reported cases in the literature restricted to DVAs in the supratentorial compartment.CaseWe present a case report of the resection of a pontine cavernoma complicated by delayed outflow obstruction of the associated DVA. A female patient in her 20's presented with progressive left-sided hemisensory disturbance and mild hemiparesis. MRI revealed two pontine cavernomas associated with interconnected DVA and hematoma. The symptomatic cavernoma was resected via the infrafacial corridor. Despite the preservation of the DVA, the patient developed delayed deterioration secondary to venous hemorrhagic infarction. We discuss the imaging and surgical anatomy pertinent to brainstem cavernoma surgery, as well as the literature exploring the management of symptomatic infratentorial DVA occlusion.ConclusionDelayed symptomatic pontine venous congestive edema is extremely rare following cavernoma surgery. DVA outflow restriction from a post-operative cavity, intraoperative manipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstem venous anatomy, and “safe entry zones” will further elucidate the etiology of and the efficacious treatment for this complication.

Background and purpose: The purpose of this study was to investigate the 5‐year risk of a third bleeding event in cavernous malformations (CMs) of the central nervous system. Methods: Patients with cerebral or spinal CMs treated between 2003 and 2021 were screened using our institutional database. Patients with a complete magnetic resonance imaging dataset, clinical baseline characteristics, and history of two bleeding events were included. Patients who underwent surgical CM removal were excluded. Neurological functional status was obtained using the modified Rankin Scale score at the second and third bleeding. Kaplan–Meier and Cox regression analyses were performed to determine the cumulative 5‐year risk for a third haemorrhage. Results: Forty‐two patients were included. Cox regression analysis adjusted for age and sex did not identify risk factors for a third haemorrhage. 37% of patients experienced neurological deterioration after the third haemorrhage (p = 0.019). The cumulative 5‐year risk of a third bleeding was 66.7% (95% confidence interval [CI] 50.4%–80%) for the whole cohort, 65.9% (95% CI 49.3%–79.5%) for patients with bleeding at initial diagnosis, 72.7% (95% CI 39.3%–92.7%) for patients with a developmental venous anomaly, 76.9% (95% CI 55.9%–90.3%) for patients with CM localization to the brainstem and 75% (95% CI 50.6%–90.4%) for patients suffering from familial CM disease. Conclusions: During an untreated 5‐year follow‐up after a second haemorrhage, a significantly increased risk of a third haemorrhage compared to the known risk of a first and second bleeding event was identified. The third bleeding was significantly associated with neurological deterioration. These findings may justify a surgical treatment after a second bleeding event. [ABSTRACT FROM AUTHOR]

Spinal cavernous angiomas are uncommon vascular malformations in the spine accounting for 5%-12% of all spinal vascular lesions. When present in an intradural extra-medullary location, these usually present with radicular pain and neurological deficits due to mass effect (myelopathy). Herein, we present an atypical presentation of cavernous angioma in a 54-year-old man with tinnitus, headache and sensorineural hearing loss. We have also reviewed 51 cases of intradural extramedullary cavernous angiomas including our case with respect to demographic and clinical profile. A 54-year-old man presented with tinnitus in the left ear and occipital headache with neck pain and slight weakness of left-hand grip along with atrophy of thenar muscles. His pure tone audiometry (PTA) test reveled mild left sensorineural hearing loss. Magnetic resonance imaging (MRI) of cervical spine showed T2WI heterogeneously hyperintense left intradural extramedullary lesion at C7 vertebral body level. It was avidly enhancing with contrast. The patient underwent C7 laminectomy with a midline durotomy and complete excision of the lesion under neuromonitoring with sacrifice of the C8 sensory root. His symptoms improved following the surgery. The diagnosis of a cavernoma in an unusual location in the presence of cranial nerve dysfunction needs a high degree of diagnostic suspicion. Most of these cavernomas have a nerve root origin or attachment. The optimal treatment is microsurgical en bloc resection which leads to an effective resolution of both the symptoms.

Objective: Cerebral cavernous malformations (CMs) presenting with focal neurological symptoms or mass effects require surgical removal. In recent years, cylindrical retractors have been widely utilized for the removal of deep-seated lesions during both microscopic and endoscopic surgery. In the present study, we evaluated the efficacy and safety of endoscopic transcylinder removal of CMs using a novel wet-field technique. Methods: We included 13 patients with supratentorial CMs who had undergone endoscopic transcylinder surgery between April 2013 and March 2022. One patient experienced recurrence of the CM and underwent a second endoscopic transcylinder surgery. Therefore, we retrospectively evaluated 14 procedures. The surgical field was continuously irrigated with artificial cerebrospinal fluid to maintain expansion and visualization of the tumor bed. We termed this method as the "wet-field technique." Patient characteristics, symptoms, and pre- and postoperative magnetic resonance imaging results were obtained from medical records. Results: The average maximum CM diameter was 35.3 mm (range: 10–65 mm). Cylinder diameters were 6 mm in eight procedures, 10 mm in four procedures, and 17 mm in one procedure. Wet-field technique was applied in all cases. The endoscope provided a bright field of view even under water. Continuous water irrigation made it easier to observe the entire tumor bed which naturally expanded by water pressure. Gross total resection was achieved in 13 procedures, while subtotal resection was achieved in one procedure. No surgical complications were observed. Conclusions: The endoscopic transcylinder removal using wet-field technique is safe and effective for the removal of symptomatic intracranial supratentorial CMs. [ABSTRACT FROM AUTHOR]

Background: Dural hemangiomas are a relatively rare form of intracranial mass, as hemangiomas tend to present in bone or as intraparenchymal lesions and only around 5%-13% have been reported to originate from the dura mater. Here, the authors present the case of a 46-year-old female who underwent craniotomy for a suspected convexity meningioma resection, which was unexpectedly found to be a dural capillary hemangioma., Observations: The patient was a 46-year-old female who presented with a left frontal intracranial mass found incidentally and showed significant growth over 4 years. The mass was suspected to be a meningioma as it was homogeneously enhancing, extra-axial, and exhibited findings of a broad base with a dural tail along the dura of the superior frontal lobe. This was excised en bloc and found to be a purplish-red mass firmly attached to the dura, which was diagnosed as a capillary hemangioma on histopathology., Lessons: Dural hemangiomas, particularly at the convexity, are quite rare and can mimic meningiomas on imaging, sometimes even demonstrating homogeneous enhancement with a broad base and dural tail. These are an important part of the differential diagnosis when diagnosing dural-based masses and should be considered when planning operative or radiation treatment. https://thejns.org/doi/10.3171/CASE24476.

Introduction: There is uncertainty whether patients with a cerebral cavernous malformation (CCM) should undergo conservative or surgical treatment, resulting in practice variation among hospitals. Our objective was to report clinical outcomes of patients with primarily conservatively managed CCMs., Patients and Methods: This single-center cohort study included consecutive adult CCM patients, diagnosed in 2000-2023, who underwent conservative management as primary treatment strategy. Data were extracted from medical records, and we systematically conducted telephone and questionnaire follow-up. Functional status was assessed on the modified Rankin Scale (mRS)., Results: Of 345 patients, we included 265 patients with a CCM (median age 46 years; 45% male). At baseline, 131 (49%) patients presented with symptomatic hemorrhage (SH), and 134 (51%) with other symptoms or asymptomatically. During 58 months (IQR 35-94) median follow-up, 51 (19%) patients experienced a SH, 33 (12%) a seizure, and 13 (5%) focal neurological deficits. Fourteen (5%) patients underwent intervention (surgery n  = 11, radiosurgery n  = 4). Presentation with SH was associated with higher annual bleeding rates (6.0% vs 1.5%, p  < 0.001), and higher cumulative 5-/10-year bleeding risks (31%/41% vs 7%, p  < 0.001). Brainstem CCM was associated with higher cumulative 5-/10-year bleeding risks (27%/38% vs 17%/21%, p  = 0.038). Nineteen (7%) patients died; two (0.8%) directly attributable to CCM. Of 246 surviving patients, 205 (83%) completed the questionnaire. At follow-up, 172/224 (77%) patients were functionally independent (mRS score ⩽2)., Discussion and Conclusion: The majority of conservatively managed CCM patients remained free of a SH during follow-up. Few patients required intervention, and death attributable to the CCM was rare. These data may help patient counseling and treatment decisions., Competing Interests: Declaration of conflicting interestThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: JMC received financial support from the Dutch Heart Foundation, Medtronic, Bayer and Boehringer (all fees were paid to his employer), and is co-founder and shareholder of TrianecT. RvdB has a non-personal hospital consultancy at Johnson & Johnson (CERENOVUS). All other authors declare that there is no conflict of interest.

BACKGROUND Structural damage to the brain substance in adults is one of the most common causes of epilepsy. Currently, such patients are prescribed drug therapy and/or surgery. With this approach, epileptic symptoms can persist in a significant proportion of patients (up to 30%, depending on the disease).AIM OF STUDY To clarify the efficacy of stereotactic radiosurgery (STS) as part of the complex treatment of patients suffering from pharmacoresistant symptomatic epilepsy with hippocampal sclerosis, vascular malformations, and some benign tumors of the brain and its membranes.MATERIAl AND METHODS Examination and radiosurgical treatment were carried out in 45 patients with various brain diseases accompanied by symptomatic epilepsy, provided that the previous conservative or surgical treatment was insufficiently effective. During the observation period, the frequency of seizures was assessed and the presence and nature of postoperative complications were recorded.RESUlTS With a median follow-up of 30 months, good outcomes (class I–II according to J. Engel’s scale) were observed in 5 out of 8 patients with hippocampal sclerosis who underwent STS after amygdaloghippocamplobectomy; seizure frequency decreased in all patients. Complications of STS were noted in 2 patients and had a transient nature. In the group of patients with meningiomas, in 4 out of 8 patients, seizures completely stopped, in 3 patients the frequency of seizures decreased by more than 90%, in another 1 the frequency of seizures decreased by 60%. There were no complications of STS during the observation period. Inintracerebral tumors (diffuse astrocytoma), Engel Ia outcome was observed in both patients. In the group of patients with cavernous angiomas, there were good outcomes (class I–II according to J. Engel’s scale) in 17 patients (85%). Deterioration (Engel IVc outcome) was observed in 1 patient (5%), 2 patients (10%) had complications of STS. In the group of patients with arteriovenous malformations, good outcomes (classes I–II according to J. Engel’s scale) were found in all 7 patients. The complication after STS was revealed in 1 patient.CONClUSION The presented results confirm the high efficiency and low risk of side effects when using stereotactic radiosurgery in the complex treatment of patients with epilepsy associated with common structural brain lesions.

Introduction: Pediatric cerebral cavernous malformations (CCMs) are commonly treated vascular anomalies with different clinical characteristics than their adult counterparts. Outcomes of Gamma Knife Radiosurgery (GKRS) for pediatric CCMs have not explicitly been reported. This paper reports our experience in managing pediatric CCMs with GKRS. Methods: We retrospectively reviewed the clinical features, GKRS parameters, and clinical and radiological outcomes of 46 children with 64 CCMs. Results: A total of 46 children, including 19 girls and 27 boys, with a median age of 16 years (3–17 years), were enrolled in the study. The median age at first CCM diagnosis was 13 years (range, 2–17 years). Twenty-two patients (47.8%) had more than one neurological symptom at the time of diagnosis, and the most common presenting symptom was seizure (28.3%). The most common location was supratentorial superficial (53.1%), and 17.4% of patients had multiple CCMs. A developmental venous anomaly was detected in 5 patients (10.9%). During a total of 52.4 retrospective patient-years in patients with > 1 hemorrhage episode, the calculated annual hemorrhage rate was 40.1%. The median post-GKRS follow-up was 79 months (range, 19–175 months), with an overall 306.2 prospective patient years. The annual hemorrhage rate (AHR) during the first 2 years after GKRS and after the initial 2 years was 1.11% and 0.46%, respectively. Regarding clinical factors and GKRS parameters, univariate analysis revealed a significant association with post-GKRS AHR and volume (p = 0.023) only. Patients with pre-GKRS seizures showed favorable seizure control (Engel class I and II) in 8 children (61.5%). There was no mortality in our series. Conclusion: Low AHR following GKRS with no radiation-induced toxicity makes GKRS a therapeutic alternative for pediatric CCMs. [ABSTRACT FROM AUTHOR]

Background and Purpose: Analyze and compare the natural course of confirmed familial cerebral cavernous malformation (FCCM), assumed FCCM and non‐familial multiple cerebral cavernous malformation (CCM) disease over a 5‐year period. Methods: Our institutional database was screened for patients with CCM admitted between 2003 and 2020. Patients with complete magnetic resonance imaging dataset, evidence of multiple CCM, clinical baseline characteristics, and follow‐up examination were included. Patients were separated into confirmed familial cases, assumed familial cases or non‐familial multiple cavernous malformations. Kaplan–Meier and Cox regression analyses were performed to determine the cumulative 5‐year risk for hemorrhage and recurrent hemorrhage. Results: A total of 238 patients with multiple CCM were analyzed; 90 individuals had a confirmed FCCM disease, 115 an assumed FCCM, and 33 were allocated to the non‐FCCM group. Univariate Cox regression analysis identified intracerebral hemorrhage (ICH) as mode of presentation (p = 0.001) as a predictor for occurrence of recurrent hemorrhage during the 5‐year follow‐up (FU). The cumulative 5‐year risk of (re)bleeding was 21.6% for the entire cohort, 30.7% for patients with ICH at diagnosis, 22.1% for those patients with a confirmed diagnosis of FCCM, 23.5% for those with an assumed FCCM, and 21% for the non‐FCCM cases. Conclusions: FCCM patients with ICH at diagnosis are prone to develop rebleeding. During untreated 5‐year FU, FCCM patients and patients with sporadic multiple CCM reveal an almost equal susceptibility for (re)hemorrhage. Moreover, confirmed, assumed and non‐FCCM patients showed an equal cumulative 5‐year risk of symptomatic ICH. The probability of hemorrhage tends to increase over time, particularly in cases with ICH at presentation. [ABSTRACT FROM AUTHOR]

Background: Cerebral cavernous angioma (CA) is a capillary vasculopathy affecting more than a million Americans with a small fraction of cases demonstrating lesional bleed or growth with major clinical sequelae. Perfusion and permeability are fundamental features of CA pathophysiology, but their role as prognostic biomarkers is unclear. Purpose: To investigate whether perfusion or permeability lesional descriptors derived from dynamic contrast‐enhanced quantitative perfusion (DCEQP) magnetic resonance imaging (MRI) can predict subsequent lesional bleed/growth in the year following imaging. Study Type: Single‐site case‐controlled study. Subjects: Two hundred and five consecutively enrolled patients (63.4% female). Field Strength/Sequence: Three‐Tesla/T1‐mapping with contrast‐enhanced dynamic two‐dimensional (2D) spoiled gradient recalled acquisition (SPGR) sequences. Assessment: Prognostic associations with bleed/growth (present or absent) in the following year were assessed in 745 CA lesions evaluated by DCEQP in the 205 patients in relation to lesional descriptors calculated from permeability and perfusion maps. A subgroup of 30 cases also underwent peripheral blood collection at the time of DCEQP scans and assays of plasma levels of soluble CD14, IL‐1β, VEGF, and soluble ROBO4 proteins, whose weighted combination had been previously reported in association with future CA bleeding. Statistical Tests: Mann–Whitney U‐test for univariate analyses. Logistic regression models minimizing the Bayesian information criterion (BIC), testing sensitivity and specificity (receiver operating characteristic curves) of weighted combinations of parameters. Results: The best prognostic biomarker for lesional bleed or growth included brainstem lesion location, mean lesional permeability, and low‐value perfusion cluster mean (BIC = 201.5, sensitivity = 77%, specificity = 72%, P < 0.05). Adding a previously published prognostic plasma protein biomarker improved the performance of the imaging model (sensitivity = 100%, specificity = 88%, P < 0.05). Data Conclusion: A combination of MRI‐based descriptors reflecting higher lesional permeability and lower perfusion cluster may potentially predict future bleed/growth in CAs. The sensitivity and specificity of the prognostic imaging biomarker can be enhanced when combined with brainstem lesion location and a plasma protein biomarker of CA hemorrhage. Level of Evidence: 2 Technical Efficacy: Stage 5 [ABSTRACT FROM AUTHOR]

Background: The present study hypothesized that some factors may distinguish between patients with a brain cavernous angioma (BCA), who were free from anti-seizure medication (ASM), and patients who still required ASMs postoperatively. The purpose of the study was thus to identify factors associated with ceasing ASMs for patients with drug-resistant epilepsy secondary to BCA, who underwent BCA removal surgery. Methods: We divided patients into those with drug-resistant epilepsy secondary to BCA who achieved complete seizure freedom without ASMs a year after surgery (No-ASM group) (International League Against Epilepsy (ILAE) classification class I with no epileptiform discharges), and others (ASM group) (ILAE classification ≤ II and/or epileptiform discharges). We statistically compared groups in terms of: (1) age at operation; (2) history of epilepsy; (3) size of BCA; and (4) location of BCA. Results: Overall, a year after the surgery, the No-ASM group comprised 12 patients (48%), and the ASM group comprised 13 patients (52%). In both multi- and univariate logistic regression analyses, age at BCA removal surgery correlated significantly with the No-ASM group (p = 0.043, p = 0.019), but history of epilepsy did not (p = 0.581, p = 0.585). Conclusions: Earlier age at surgery for patients with drug-resistant epilepsy is encouraged to achieve complete seizure freedom without the need for ASMs when the cause of epilepsy is BCA. [ABSTRACT FROM AUTHOR]

Acute onset of neurological deficit is highly suggestive of stroke; in such cases, computed tomography (CT) brain is the initial choice of investigation. While CT brain can differentiate between hemorrhagic and ischemic infarct, more often than not, it is unable to detect the underlying etiology of intracerebral hemorrhage. In these situations, magnetic resonance imaging (MRI) brain is crucial in determining the exact etiology and helps us tailor the specific management best suited for our patient. The case under discussion is of a 48-year-old male who presented with multiple cranial nerve palsies and ipsilateral cerebellar involvement in whom CT brain revealed a hemorrhage involving left hemipons and left middle cerebellar peduncle while an MRI brain revealed an unexpected cavernous angioma which changed the management and prognosis of the patient, proving its superiority over CT brain., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Karthikeyan Meenakshi et al.)

The insular cortex is considered one of the most complex regions of the brain, defined as the "hub" of somatosensory areas. Here, we examine the case of a surgically treated haemorrhagic cavernoma involving the middle and posterior insular cortex, presenting both sensory, gustative and speech symptoms. By reviewing the recent findings in humans' and primates' basic research, we illustrated clinical and radiological correlations of the reported case, confirming insular role in sensitive and gustatory functions. [ABSTRACT FROM AUTHOR]

Background: Cerebral cavernous malformations (CCMs), otherwise known as cavernous hemangiomas/cavernomas, are a type of vascular malformation. It is the third most common cerebral vascular malformation, histologically characterized by ectatic, fibrous, blood filled "caverns" with thin-walled vasculature without intervening normal brain parenchyma. Case Description: Herein, we present a case of an original, spontaneous hemorrhage from a sporadic form of CCM without associated gross developmental venous anomaly in an 11-year-old child, which is an extremely rare occurrence, with the special emphasis on the demographic data of the affected population, risk factors associated with hemorrhage, and correlation of histopathological and radiological findings with an in-depth literature review. Conclusion: The significant majority of the CCM are clinically occult. Hence, the development of risk assessment tools and guidelines for timely neurosurgical intervention poses a greater clinical challenge for medical experts rendering the management of the affected individuals with CCM in an anecdotal situation. Presentation of life-threatening rebleeds and neurological deficits in the diagnosed population albeit uncommon is possibly preventable outcomes. [ABSTRACT FROM AUTHOR]

Abstract Cerebral cavernous malformations (CCMs) are dilated capillaries causing epilepsy and stroke. Inheritance of a heterozygous mutation in CCM3/PDCD10 is responsible for the most aggressive familial form of the disease. Here we studied the differences and commonalities between the transcriptomes of microdissected lesional neurovascular units (NVUs) from acute and chronic in vivo Ccm3/Pdcd10 ECKO mice, and cultured brain microvascular endothelial cells (BMECs) Ccm3/Pdcd10 ECKO . We identified 2409 differentially expressed genes (DEGs) in acute and 2962 in chronic in vivo NVUs compared to microdissected brain capillaries, as well as 121 in in vitro BMECs with and without Ccm3/Pdcd10 loss (fold change ≥ |2.0|; p