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Cavernous malformations (CMs) or hemangiomas arising from within the dural sinuses are rare entities that differ from their parenchymal counterparts in that they are highly vascular lesions. While parenchymal CMs are typically angiographically occult, intrasinus malformations may have large, dural-based arterial feeding vessels that are amenable to preoperative embolization. The novel liquid embolic Onyx (ev3, Inc.) is an ideal agent for the embolization of these lesions. The authors present the first known case of a giant intrasinus CM embolized with Onyx before gross-total resection. The authors report the case of a 9-year-old boy with brief apneic episodes in whom MR imaging revealed a giant CM arising from within the right transverse and sigmoid sinuses and infiltrating the right tentorium cerebelli. At another institution, the patient had undergone 1 prior embolization and 2 unsuccessful attempts at resection. Both surgeries had been complicated by massive blood loss and were aborted. Under the authors' care, the patient underwent preoperative transarterial embolization with Onyx during which a substantial volume of the mass lesion was filled with embolisate. Subsequently, complete circumferential excision of the mass from the tentorium was accomplished with minimal intraoperative blood loss.

Cerebral cavernous malformations are rarely described in children. Giant cavernous malformations are exceptional in this group of patients. The authors reports a case of an eleven months boy, who begins at the sixth month of life with epilepsy. Medical therapy was advised and imagenological studies were carried out. An IRM was performed, and shows a giant cavernous malformation in the right parietal lobe. Surgery was carried out, with complete resection of the lesion. In the postoperative period and at more than a year of follow up, he was free of crisis. We report the case because of the rarity of a giant cavernous angioma in a patient less than a year old.

This 15-year-old boy with a tentorial cavernous angioma reported occasional headache and scintillation in his left visual field. Magnetic resonance imaging revealed a well-demarcated, homogeneously enhanced tumor originating from the right cerebellar tentorium and extending into both the supratentorial and infratentorial spaces. Although a meningioma was suspected, vertebral artery angiography revealed a thickened meningeal branch originating from the right posterior inferior cerebellar artery and flecked tumor stain with pooling of contrast medium until the late venous phase. A cavernous angioma of the tentorium was suspected based on this finding, and as expected from the radiological findings, profuse bleeding was encountered during tumor removal. The histological diagnosis was a cavernous angioma. A cavernous angioma of the tentorium is extremely rare but should be differentiated from a meningioma preoperatively given that a cavernous angioma of dural origin tends to bleed massively during removal.

Diffuse neonatal hemangiomatosis presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. Described here is a case of diffuse neonatal hemangiomatosis with predominant central nervous system involvement. The early appearance of central nervous system lesions on imaging can overlap with that of cavernous malformations, confounding diagnosis; however, rapid growth, response to steroids, cystic appearance with sedimentation levels of the mature lesions, and involvement of other visceral organs can help confirm the diagnosis.

A clinical-neuropsychological study of 25 patients with hematomas, cavernomas and arteriovenous malformations of different brain stem regions has been performed. Patients with hydrocephalic-hypertensive and dislocation syndromes as well as a history of neurological diseases were excluded from the study. All patients had hemorrhages in different brain stem regions, most of which had occurred several weeks ago. Hematomas were located in different regions of the pons (14 patients) and midbrain (7 patients) and spread to both regions in 4 patients. All patients underwent MRI study of the brain and complex neuropsychological investigation using the A.R. Luria's method. Neuropsychological symptoms before the surgery were found in 20 patients. Cognitive disturbances similar by the lesion of frontal lobes, in particular the promoter zone, that manifested themselves in disturbances of dynamic praxis, writing, verbal memory, were observed most often. Cognitive disturbances similar by the lesion of occipital hemisphere regions, i.e. disturbances of visual gnosis and spatial defects, were found less often. The most severe symptoms were observed in the lesion of the midbrain and upper regions of the pons.

Objective: To investigate the clinical features and treatment outcome of pediatric cavernous malformation (CM) in the central nervous system., Patients and Methods: Retrospective review of clinical data and treatment outcome for 66 pediatric patients under the age of 18 treated for CM at Huashan Hospital within the past 10 years from January 1998 to December 2007., Results: In our consecutive series, the mean age at onset of the CM was 11.6 years (age from 15 months to 17.8 years), and the boy-to-girl ratio was 1.54:1 (40:26). Main initial clinical manifestations in cerebral CM (CCM) included: seizures (47.7%, 31/65), headache (46.2%, 30/65), intracranial hemorrhage (20%, 13/65), and focal neurological deficits (12.3%, 8/65). There were 59 children with single CM and 7 children with multiple CMs: 55 (83.3%) with supratentorial CM, 6 (9.1%) with infratentorial CM, 4 (6.1%) with supra- and infratentorial CM, and 1 (1.5%) with intramedullary CM. Sixty-two (93.9%; 62/64) children underwent microsurgical operations, and with the help of neuronavigation (19 cases), intraoperative ultrasonography (6 cases), and neuronavigation combined with intraoperative ultrasonography (3 cases). And 4 (6.1%; 4/66) children did not undergo any surgical intervention. There was no major morbidity or mortality from surgical procedures. Follow-up data of 50 children was available with a mean follow-up time of 39.1 months (range from 5 to 112 months), including 46 operated children and 4 children managed without operation. In the 46 operated children, the overall long-term post-treatment results were satisfactory: 73.9% no sign or symptom associated with CMs, 19.6% improved obviously, only 1 (2.2%) boy with unrestored paraplegia incurred by spinal cord CM, and 2 boys (4.3%) with controllable seizures occurring after initial 5 symptom-free years (one without need of antiepileptic drugs, AEDs). Of 24 children followed-up with preoperative seizures, only 1 boy needed therapeutic AEDs and the others did not need any after resections of the CMs. Three children managed without operations (2 with headache and 1 with seizures) had spontaneous disappearance of symptoms and stable intracerebral lesions at follow-up MRI images., Conclusions: For symptomatic solitary CM, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation, and the risk of operation can be decreased to lower level with the help of neuronavigation and intraoperative ultrasonography. For multiple CM, the treatment modalities must be considered cautiously. A much longer follow-up remains mandatory for appropriate treatment strategies., (Copyright (c) 2009 S. Karger AG, Basel.)

Objective: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs., Materials: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS., Results: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas., Conclusion: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy., (Copyright (c) 2009 S. Karger AG, Basel.)

A clinical-neuropsychological study using Luria's method has been conducted in 13 patients with arteriovenous malformations (AVM) and 12 patients with cerebellar cavernomas and hematomas. The syndromes were similar to lesions of the large hemisphere cortex, more often of premoter regions and less often of the cortex of posterior hemispheric regions. After a surgery on the right cerebellar hemisphere, some patients had perseverations and micrographia in writing. These syndromes were principal similar with findings described in the literature. However there were differences including the absence of marked cognitive disturbances even after the surgery. The disturbances of verbal memory were found in the lesion of both right and left cerebellar hemispheres that is the functional asymmetry of cerebellar hemispheres was not pronounced. The inactivity of speech and agrammatism in writing, even in patients with large duration of hemorrhage, were not observed. In some patients with the lesion of right cerebellar hemisphere, the disinhibition of speech, similar to that in patients with the lesion of right frontal lobe, was found. There were no marked mental disturbances. The most distinct cognitive disturbances were found after surgeries of the superior cerebellar artery. Possible mechanisms of the cerebellum's role in cognitive functions are discussed.

Cavernous haemangiomas are benign vascular lesions of the central nervous system. Their size varies from a few millimetres to several centimetres. Giant and paediatric cases are rare. This report presents a 10-month-old baby with a giant multilobular cavernous haemangioma in the left parietal lobe who presented with seizures.

Objective: We sought to assess the appearance of cerebral cavernous malformations (CCM) on magnetic resonance imaging (MRI) scans in murine Ccm1 and Ccm2 gene knockout models and to develop a technique of lesion localization for correlative pathobiological studies, Methods: Brains from 18 CCM mutant mice (Ccm1 Trp53 and Ccm2 Trp53) and 28 control animals were imaged by gradient recalled echo (T2*)-weighted MRI scans at 4.7- and 14.1-T in vivo and/or ex vivo. After MRI scanning, the brains were removed and stained with hematoxylin and eosin, and cells were laser-microdissected for molecular biological studies., Results: T2*-weighted MRI scans of brains in vivo and ex vivo revealed lesions similar to human CCMs in mutant mice, but not in control animals. Stereotactic localization and hematoxylin and eosin staining of correlative tissue sections confirmed lesion histology and revealed other areas of dilated capillaries in the same brains. Some lesions were identified by MRI scans at 14.1-T, but not at 4.7-T. Polymerase chain reaction amplification from Ccm1 and beta-actin genes was demonstrated from nucleic acids extracted from laser microdissected lesional and perilesional cells., Conclusion: The high-field MRI techniques offer new opportunities for further investigation of disease pathogenesis in vivo, and the localization, staging, and histobiological dissection of lesions, including the presumed earliest stages of CCM lesion development.

Vocal cord palsy may result from varied causes. However, cord palsy resulting from a brain-stem cavernoma has never been reported. We report a patient with vocal cord palsy in a 30-year-old male resulting from the brain-stem lesion. The patient became symptomatic each time the lesion bled and improved gradually when the bleeding resolved. Repeated insults on the brain-stem produced permanent cord palsy. Although a rare presentation, brain-stem cavernoma may be considered in the aetiology of cord palsy.

Cavernous angiomas comprise 5-10% of all vascular malformations in the central nervous system, occurring most frequently in the supratentorial region, and 20% of them in the brain stem. According to literature, brain stem cavernous angiomas occur most frequently in the pons (60%), and equally in the mesencephalon (20%) and in medulla oblongata. In clinical evaluation the authors describe the successful removal of a mesencephalic cavernous angioma causing progressive neurological deficits and symptoms. The authors present a case of a 51 year old female, who had developed 1 year prior to her admittance: fatigue, weakness in the right upper limb and fingers, right lower limb ataxia. One month later, her lower right limb developed sensory deficits. The first neurological exploration indicated dysarthria, moderate facial and right hemiparesis, hemihypaesthesia and ataxia. CT and MR imaging indicated multilobulated cavernomas in the mesencephalon. After conservative treatment the patient became almost symptom free, and thus neurosurgical treatment was not discussed. Later on her symptoms fluctuated, but after 6 month she suddenly developed progressive right hemiparesis, right facial weakness, serious dysphasia, and emotional incontinence combined with continuous spastic sobbings. The control MRI showed enlargement of the cavernomas and new extravasation. Surgery was indicated for removing the cavernomas. The left infratentorial, supracerebellar approach revealed a blood engorged cavernoma in the center of the mesencephalon, almost dividing it. The cavernomas and accompanying haematoma was extirpated. The patient's neurological symptoms rapidly improved after surgery, her dysphasia as well as motor weakness have disappeared. Six days after surgery, we discharged a neurologically symptomless and self-supporting patient. The literature and the presented case indicates that the correct timing and proper surgery allows brain stem cavernomas to be safely removed, or significantly bated, which results in the massive regression of neurological symptoms.

Optimal management of cavernous sinus hemangiomas remains unclear. Total microsurgical removal of these neoplasms may be extremely difficult due to their rich vascularization. Three cases of cavernous sinus hemangioma treated with low-dose Gamma Knife radiosurgery are presented. Marginal dose varied from 10 to 13 Gy. Treatment planning and radiation dosimetry were done with a goal of conformal and selective coverage of the lesion with 50% prescription isodose line using multiisocenter technique. In all cases significant shrinkage of the neoplasm was marked at 3 months after treatment. Mean volume reduction at 12 months after radiosurgery was 60% (range: 45-75%). In all patients the shrinkage of the neoplasm was accompanied by notable improvement of the preexistent oculomotor nerve palsy. No radiosurgery-related complications were met during follow-up. In conclusion, low-dose Gamma Knife radiosurgery seems to be very effective for management of cavernous sinus hemangiomas, and can be considered as a treatment modality of choice for these lesions.

Background and Purpose: Radiotherapy is an integral part of various therapeutic regimens in pediatric and adult oncology. Endocrine dysfunction, neurologic and psychiatric deficits, secondary malignancies and radiation-induced necrosis are well-known possible late effects of cranial irradiation. However, only sporadic cases of radiation-induced cavernous hemangiomas (RICH) have been reported so far., Patients and Methods: Pediatric patients who underwent cranial radiation therapy for malignant diseases between January 1980 and December 2003 were retrospectively analyzed. After the end of therapy they entered a detailed follow-up program., Results: Of 171 patients, eight (three patients with medulloblastoma, three patients with acute lymphoblastic leukemia, and one patient each with ependymoma and craniopharyngioma) developed intracerebral cavernoma 2.9-18.4 years after irradiation representing a cumulative incidence (according to the Kaplan-Meier method) of 2.24%, 3.86%, 4.95%, and 6.74% within 5, 10, 15, and 20 years following radiation therapy, respectively. In patients treated in the first 10 years of life, RICH occurred with shorter latency and significantly more often (p = 0.044) resulting in an even higher cumulative incidence., Conclusion: These findings and previously published cases show that cavernous hemangiomas may occur after irradiation of the brain several years after the end of therapy irrespective of the radiation dose and type of malignancy. Particularly children < 10 years of age at the time of irradiation are at higher risk. Since patients with RICH frequently do not show symptoms but hemorrhage is a possible severe complication, imaging of the central nervous system should be performed routinely for longer follow- ups, particularly in patients who were treated as young children.

A decade of empirical work in brain imaging, genomics, and other areas of research has yielded new knowledge about the frequency of incidental findings, investigator responsibility, and risks and benefits of disclosure. Straightforward guidance for handling such findings of possible clinical significance, however, has been elusive. In early work focusing on imaging studies of the brain, we suggested that investigators and institutional review boards must anticipate and articulate plans for handling incidental findings. Here we provide a detailed analysis of different approaches to the problem and evaluate their merits in the context of the goals and setting of the research and the involvement of neurologists, radiologists, and other physicians. Protecting subject welfare and privacy, as well as ensuring scientific integrity, are the highest priorities in making choices about how to handle incidental findings. Forethought and clarity will enable these goals without overburdening research conducted within or outside the medical setting.

A 41-year-old female presented with repeated pontine hemorrhage. Magnetic resonance imaging showed the pontine hemorrhage as a heterogeneously enhanced mass mimicking cavernous angioma. The mass lesion was removed via a midline suboccipital approach. Histological examination showed malignant melanoma cells. No cutaneous lesion was found and positron emission tomography found no abnormalities. Our presumptive diagnosis was primary intra-axial brainstem malignant melanoma. The possibility of malignant melanoma should be considered in patients with intra-axial brainstem lesion associated with repeated hemorrhages.

Results of our series of 10 operated patients who had cavernous malformation of the brain stem, in accordance with the findings of other authors, have confirmed that surgical removal of cavernomas is an absolutely appropriate approach as regards the prevention of repeated and disabling hemorrhages, also from the point of view of the recovery of the neurological deficit. In patients with disturbed vital functions (breathing, swallowing) evacuation of hematomas and removal of the malformation eliminate life endangering compressive effect on the vital structures of the brain stem. It is not disputable that patients with superficially localized cavernomas and progressing neurological deficit should be subjected to surgical treatment. In cases of deep lying lesions that are covered by a considerable layer of nervous parenchyma, it is indispensable to have a very careful preoperative planning of a safe entry into the brain stem, without damage to the vital functional structures. In case of a complete neurological recovery after a single hemorrhagic episode, the decision to perform surgery is delicate and demands individual appraisal of each patient. As regards the procedure with incidentally found and clinically asymptomatic lesions, regardless of the MR-signs of hemorrhage in the form of the hemosiderin ring around the lesion, we support the view of those authors who think that such lesions should not be operated but should be subjected to clinical treatment and MR with periodical MR check-ups.

Among cavernomas of the central nervous system, spinal ones are rare. The true incidence of spinal cavernomas is unclear, but with widespread use of magnetic resonance imaging the number of cases is increasing. Furthermore, cavernomas represent only 5-12% of all vascular anomalies of the spinal cord, with a mere 3% reported to be intradural and intramedullary in location. Cervical spine intradural extramedullary cavernomas are very seldom seen, and only 4 cases have been reported in world literature previously. In this report, a unique case of an intradural extramedullary spinal cavernoma was surgically treated in a patient who presented only with an intramedullary hemorrhage.

Cerebral cavernous malformations (CCM) are common hamartous dysplasias characterized by abnormally dilated vascular channels. CCM mostly occur sporadically, and multiple occurrence of CCM is highly suggestive of a genetic origin of the disorder. Typical clinical symptoms are seizures, hemorrhages, focal neurological deficits, and headaches. Three genes have so far been described that are responsible for most cases of familial CCM and more than half of the sporadic cases with multiple CCM (CCM1-3). The coincidence of CCM and other vascular anomalies has been described before. The present review discusses the association of CCM with mesenchymal anomalies, with special emphasis on the possible common pathogenetic pathway for CCM and atrial myxomas. An illustrative case is presented in which CCM occurred together with different dysplasias (multiple CCM, liver cavernoma, and cardiac atrial myxoma), which are all thought to arise from abnormal mesenchymal cell differentiation processes.

Although, for a long time, they have been known as a kind of vascular lesion, cavernomas have mostly been incidental or causative autopsy finding or have subsequently been histologically confirmed after surgical interventions undertaken due to intracerebral and spinal spontaneous hematoma of various localization. The aim of this study was to establish on the basis of the outcome of the operative treatment, whether the surgical interventions were justified and to systematize indications for surgical treatment of the cerebral cavernoma that initially manifested by hemorrhage. The subject of the study was a group of 38 patients who had been operated at the Institute for Neurosurgery during a ten-year period, from 1990 until 2000. The study group consisted of 16 male and 22 female patients. All operated patients had cyst intraparenchymal lesions. In our group of surgically treated patients three had been treated urgently due to spontaneous intracerebral hematoma, and intraoperatively taken material after inspection of the cavum pointed to the fact that cavernoma had been the cause of hemorrhage. All the others, after postoperatively done MRI of the brain, in some even DSA, were completely evaluated, and histologically confirmed. Not one hemorrhaging cavernoma showed signs of subarachnoid hemorrhage, although 60% of operated patients had cortically localized lesion. Most of the operated patients, except for the three mentioned because of urgent intervention did not give massive intraparenchymal lesion that, in the clinical picture, would lead to the change of the state of consciousness. Focal neurologic deficit was a dominant clinical presentation. On the basis of the analysis of the clinically pathologic correlations and direct and longstanding operative results of the surgical treatment of cavernoma, operative treatment is indicated in all superficial lobar lesions as well as in those that are localized in the brain chambers and pineal region regardless of the type of the clinical presentation.

The aim of this study is to present the principal clinical manifestations and neurosurgical results of the treatment of patients with supratentorial cavernoma and epilepsy. The retrospective study included 14 patients with supratentorial lesion on MRI and CT scan of the brain that manifested with epileptic seizures. All patients were surgically treated and pathophysiologic evaluation in all patients confirmed that cavernoma was in question. Results were analyzed and compared with data from the literature. Epileptic seizures are the most frequent clinical manifestation found in supratentorial cavernoma and neurosurgical treatment gives excellent result as regards the control of epilepsy, with a very low incidence of morbidity and mortality. A complete resection of all cavernomas in this study was confirmed by postoperative neurosurgical diagnosing. 12 patients did not have any more epi seizures in the postoperative period, and in 2 patients we found decrease in the occurrence of epilepsy seizures.

Purpose: In the context of stem cell transplantation (SCT), we often observe neurological complications as a consequence of immune system suppression, conditioning therapy or prophylaxis and treatment of graft-versus-host disease. Furthermore, cerebral lesions in existence prior to transplantation can be found. The aim of this study was to evaluate the benefit of cerebral magnetic resonance imaging (MRI) prior to stem cell transplantation., Patients and Method: Cerebral MR examinations of 116 children and adolescents were performed before SCT. Patients ranged in age from 1.1 to 21.4 years (mean 12.6 years). All MR images were obtained by a 1.5 T system. The predefined short protocol included an axial T 1-weighted SE sequence and a coronary T 2-weighted TSE sequence. We evaluated existing cerebral lesions, the diameter of the ventricular system, and the paranasal sinuses. In the case of pathological findings, the short examination protocol was expanded., Results: In 5 of 116 children (4.3 %) we observed prior to SCT findings requiring immediate treatment although the patients did not show any clinical symptoms (1 x aspergilloma, 1 x hemorrhage of vascular anomaly). An increased risk of bleeding caused by cavernoma or another vascular anomaly without hemorrhage also had to be taken into account. 32 of 116 patients (37.1 %) showed atrophic lesions. In 42 children (36.2 %), we observed affections of the paranasal sinuses., Conclusion: The imaging findings requiring immediate treatment even though the children did not show any clinical signs, justify cerebral MR examinations prior to stem cell transplantation.

Introduction: To report a retrospective series of 84 cerebral developmental venous anomalies (DVAs), focusing on associated parenchymal abnormalities within the drainage territory of the DVA., Methods: DVAs were identified during routine diagnostic radiological work-up based on magnetic resonance imaging (MRI) (60 cases), computed tomography (CT) (62 cases) or both (36 cases). Regional parenchymal modifications within the drainage territory of the DVA, such as cortical or subcortical atrophy, white matter density or signal alterations, dystrophic calcifications, presence of haemorrhage or a cavernous-like vascular malformation (CVM), were noted. A stenosis of the collecting vein of the DVA was also sought for., Results: Brain abnormalities within the drainage territory of a DVA were encountered in 65.4% of the cases. Locoregional brain atrophy occurred in 29.7% of the cases, followed by white matter lesions in 28.3% of MRI investigations and 19.3% of CT investigations, CVMs in 13.3% of MRI investigations and dystrophic calcification in 9.6% of CT investigations. An intracranial haemorrhage possibly related to a DVA occurred in 2.4% cases, and a stenosis on the collecting vein was documented in 13.1% of cases. Parenchymal abnormalities were identified for all DVA sizes., Conclusion: Brain parenchymal abnormalities were associated with DVAs in close to two thirds of the cases evaluated. These abnormalities are thought to occur secondarily, likely during post-natal life, as a result of chronic venous hypertension. Outflow obstruction, progressive thickening of the walls of the DVA and their morphological organization into a venous convergence zone are thought to contribute to the development of venous hypertension in DVA.

Objective: A causal association of brain lesion to the physiopathology of anorexia nervosa will be discussed., Method: The authors report the case of a female patient who developed anorexia nervosa. A cavernoma, located on the frontal side of the right sylvian, was discovered by chance after a seizure., Results: Surgical treatment of the lesion resulted in complete remission of the eating disorder at two years follow-up., Conclusion: Evidence for organic brain contribution to anorexia nervosa is strong and can be illustrated by this case report of anorexia nervosa associated with cerebral tumour., ((c) 2007 by Wiley Periodicals, Inc.)

Background: Although patients with MCMs have increasingly been found in clinics, little has been focused on them. Thus, we intended to investigate these patients' clinical presentations, family history, radiological characters, and treatment strategy., Methods: A retrospective review of the files and family investigations were conducted for 30 patients with MCMs. All patients underwent MRI examination. Symptomatic patients underwent the surgical treatment with image-guided technique., Results: There were 19 male and 11 female patients with a total 79 lesions. The common presentations were seizures, hemorrhages, or focal neurological deficits. Nine patients had positive or doubtful family history. The FLAIR sequence of MRI showed the highest sensitivity in the detection of CM lesions. In 27 symptomatic patients with 69 lesions, total removal was achieved in 19 patients with 48 lesions. In the other 8 patients with 21 lesions, 13 lesions were removed. Preoperative symptoms were improved in 21 patients and unchanged in 5. Preoperative neurological deficits temporarily worsened in one, and a new onset of seizure occurred in other one; but both gradually improved during the follow-up period. Among 3 patients with asymptomatic MCMs, one patient had hemorrhage during the follow-up period and underwent surgical operation., Conclusions: Because a high frequency of family CM occurs in MCMs, a detailed family investigation is mandatory for each patient with MCM. Selection of higher sensitive MRI sequence would contribute to detection of more CM lesions. Microsurgery assisted with the neuroimaging techniques is the treatment of choice for symptomatic MCMs.

Background: Diffusion tensor imaging (DTI) and white matter tractography (WMT) are promising techniques for estimating the course, extent, and connectivity patterns of the white matter (WM) structures in the human brain. In this study, we investigated the ability of DTI and WMT to visualize white matter tract involvement for the preoperative surgical planning and postoperative assessment of brainstem lesions., Methods: Preoperative and postoperative DTI data (echo-planar, 1.5T) were retrospectively analyzed in 10 patients with brainstem lesions (3 diffuse, 7 focal). WMT applying a tensor deflection algorithm was used to reconstruct WM tracts adjacent to the lesions. Reconstructed tracts included corticospinal tracts and medial lemnisci. The clinical and imaging follow-up data were also compared and analyzed., Findings: WMT revealed a series of tract alteration patterns including deviation, deformation, infiltration, and apparent tract interruption. WMT reconstructions showed that the major WM tracts were preserved during surgery and improved in position and appearance postoperatively. These findings correlated with the improvement or preservation of neurological function as determined by clinical assessment., Conclusions: Compared with the information provided by conventional MR imaging, DTI and WMT provided superior quantification and visualization of lesion involvement in eloquent fibre tracts of the brainstem. Moreover, DTI and WMT were found to be beneficial for white matter recognition in the neurosurgical planning and postoperative assessment of brainstem lesions.

The objective of this study was to retrospectively investigate the surgical indications, operation methods, and postoperative results of patients with cavernous malformations (CMs) and the use of neuronavigation. Seventy patients with CMs were operated on in our hospital. The initial symptoms were hemorrhage in 31 cases, seizures in 29 cases, focal neurological deficit in 4 cases, and headache in 5 cases. Only one asymptomatic case was incidentally identified by radiological examination. The size of CMs ranged from 5 to 50 mm (mean: 21 mm). The CMs in 39 patients were located within the white matter of the hemispheres, 11 within the basal ganglia or thalamus, 5 within the cerebellum, and 6 in multiple locations. Prior to the operation, five fiducial markers were placed on the scalp and magnetic resonance imaging (MRI) was performed. Data were transferred to the neuronavigation workstation, a profile of tumors was drawn up, and a three-dimensional reconstruction was completed. The foci were separated and resected under a microscope through a sulci and fissures approach. All the lesions were resected totally. No residual foci were found by postoperative computed tomography (CT)/MRI examination, and there was no operative death. The disability rate was 8.6%; four patients had a new transitory neurological deficit within 1 month after the operation, and one patient with thalamic CMs developed facial paralysis. All patients, however, recovered in 6 months. Twenty-four patients with preoperative epilepsy were followed up for 6-24 months (mean: 19.4 months). After the operation, seizures disappeared in 19 patients and symptoms improved in 5. CMs can be determined in patients even if patients are asymptomatic. Surgical treatment should be considered if bleeding occurs. An operation with the guidance of neuronavigation is safe and can decrease the occurrence of disability following the procedure.

Cavernomas of the CNS may involve brain parenchyma as well as the spinal cord and belong to those cerebrovascular malformations that have no arteriovenous shunt and thus are generally angiographically occult. Contrary to computed tomography (CT), which is generally suited to reveal calcifications or acute bleeding, magnetic resonance imaging (MRI) enables detection of even small cavernomas. A frequently associated venous anomaly is helpful for correct diagnosis.

The treatment of an intrinsic midbrain tumour has always been controversial due to the precarious nature of its location. We report a rare example of a cavernoma located in the ventral mesencephalon and presented clinically as Holmes' tremor. In order to access this vital portion of midbrain, we chose a fronto-temporal trans-sylvian route via an orbito-zygomatic craniotomy. The cavernoma was removed en bloc with a longitudinal pial incision between the fronto-pontine fibers and the pyramidal tracts in the peduncle. After the operation, the tremor dramatically disappeared. Through a review and analysis of the literature, we discuss the surgical strategy for management of an anterior midbrain cavernoma. With an optimal surgical approach, fairly safe entry zones on the anterior face of the rostral brainstem may be accessible, which makes it possible for successful resection of a ventral mesencephalic cavernoma without postoperative complications.

As congenital anatomic variants of venous drainage, developmental venous anomalies (DVA) represent up to 60% of all cerebral vascular malformations. The prior term "venous angioma" is a misnomer implicating an abnormal vascular structure with an increased bleeding risk. They are often found incidentally and are hardly ever symptomatic. Their morphologic characteristics are dilated vessels in the white matter, which converge on a greater collector vein, forming the typical caput medusae. They drain into the superficial or deep venous system. The frequent association with other, potentially bleeding-prone vascular malformations is clinically relevant, in particular cavernous angioma, which might require therapeutic action. Therefore, coincident vascular lesions need to be actively sought by appropriate additional imaging techniques.

Study Design: Case report and review of the literature., Objective: To report an unusual case of a spinal intradural extramedullary cavernous angioma (CA), located at the cauda equina, and to compare it with the previously published 22 surgically treated cases in the literature., Setting: Ankara, Turkey., Method: A 67-year-old man presented with a 4-month history of back pain and sciatica and a 4-week history of progressive lower extremity paresthesia, difficulty walking, and loss of bladder and bowel sphincter control. Neurological examination revealed bilateral hypoesthesia below the T12 dermatome with spastic paraparesis. Magnetic resonance imaging (MRI) of the cauda equina revealed a heterogeneous enhancing intradural extramedullary mass obliterating the spinal canal and expanding the spinal cord. T12 laminectomy and total tumor removal were performed without additional neurological deficit. Pathological examination confirmed the diagnosis of a CA., Result: The patient's neurological status improved during postoperative recovery. He was ambulatory without assistance and regained full sphincter control on the eighth postoperative week., Conclusion: CAs of the spinal cord are extremely rare lesions. Typically, they present with low back pain and sciatica, neurological deficits, or as a subarachnoid hemorrhage. These lesions have characteristic features on MRI and should be considered in the differential diagnosis of intradural spinal lesions. Following a thorough literature review of reported cases, the authors propose that for patients presenting with severe preoperative neurological signs, immediate microsurgical tumor excision or decompression increases the chance of neurological improvement.

MRI is the best radiological technique to explore cavernomas, vascular malformations affecting the entire central nervous system. The presence of blood degradation products produces a specific aspect which enables excellent contrast resolution. Certain diagnosis can be established with MRI which can also be used to follow growth and modifications, particularly in familial forms. In the emergency setting, the first exam is often a CT-scan for patients presenting acute neurological sign(s) and/or with a clinical suspicion of hemorrhagic stroke. Angiography is generally not contributive because cavernomas are occult vascular malformations. Nevertheless, this exam is often necessary when an associated vascular abnormality is suspected, particularly a developmental venous abnormality.

Background: Cerebral cavernous malformations (CCMs) are congenital vascular anomalies of the central nervous system that can result in seizures, hemorrhage, recurrent headaches, and focal neurologic deficits. These CCMs can occur as sporadic or autosomal dominant conditions, although with incomplete penetrance and variable clinical expression. Three CCM loci have been identified, on chromosomes 7q21-22 (CCM1; Online Mendelian Inheritance in Man [OMIM] 116860), 7p13-15 (CCM2; OMIM 603284), and 3q25.2-27 (CCM3; OMIM 603285), and 3 genes have been cloned, KRIT1 on CCM1, MGC4607 on CCM2, and PDCD10 on CCM3. Mutations in KRIT1 account for more than 40% of CCMs., Objective: To describe the results of a comprehensive evaluation of 5 Italian families affected with CCM., Design: Clinical, magnetic resonance imaging, and KRIT1 gene analysis., Setting: University academic teaching hospitals., Patients: Fifteen patients with CCM diagnosed according to defined criteria and 45 at-risk, symptom-free relatives., Results: Three novel and 2 described mutations were found in KRIT1. The families included 33 KRIT1 mutation carriers, 57.6% of whom had no symptoms. Magnetic resonance imaging revealed CCM lesions in 82.3% of symptom-free mutation carriers., Conclusions: The data confirm both incomplete clinical and neuroimaging penetrance in families with the KRIT1 mutation. This consideration is important in genetic counseling. Moreover, the data emphasize both the importance of magnetic resonance imaging in the diagnosis of CCM and the potential for DNA-based diagnosis to identify subjects at risk.

Ten percent of all cavernomas are familial forms. 300 independent families have been identified in France since 1995. Clinical manifestations are more frequent in familial (50%) than in sporadic forms (5%). The symptoms are the same in both forms: epilepsy, hemorrhages, neurological focal deficits and headache, but hemorrhages are more frequent and the age of revelation is younger, before 30 years. It is also frequent to observe extraneural location, cutaneous and retinal. On MRI, four types of lesional aspects were described and lesions are multiple in all cases with numerous "de novo" cavernomas. The prognostic does not depend on the number of lesions, but on their topography, especially in the brain stem. Familial forms may be considered not only as a neurological but as a systemic disease for which global management with a genetic counseling should be considered. Gene therapy is not today available, but perhaps in the future.

Introduction: Intracraneal vascular malformations are congenital lesions due to alterations in the development of arteriocapillary network. Cavernomas are present in 0.4% of people, and represent 5-13% of all cerebrovascular malformations. They are multilobulated clearly delimited lesions that contain blood at different evolution. Cavernomas can be single or multiple, and sporadic or familial. Inheritance pattern in familial cases is autosomal dominant and three involved genes have been described., Case Reports: We made a retrospective review of clinical histories of two patients diagnosed of multiple familial cavernomatosis. First patient's onset was with partial seizures. Magnetic resonance (MR) showed a frontal cavernoma probable responsible of seizures, and other lesions in frontal and parietal lobes. Second patient consulted for psychomotor delay and behaviour disorder. MR showed multiple cavernomas. In the first patient, one lesion was surgically removed. In second patient, the attitude was expectant. In both cases familial study was done and multiple cavernomas were found in both parents., Conclusions: Cavernomas are a type of vascular malformations with specific histological features. Usual clinical characteristics are seizures and parenchymatous bleeding. The appearance of MR has permitted the diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for this purpose. In familial cases, multiple lesions are found in 84%, often in association with family history of seizures. Surgical treatment must be considered in patients with symptomatic or progressive lesions that are accessible. All cases must be clinically and MR followed.

Intramedullary cavernomas are rare, but with routinely use of MRI detection has improved, raising the problem of choosing the adequate management approach: conservative or surgical. Cavernomas are vascular malformations, but, as hemangioblastomas they appear as vascular tumors of the spinal cord. They can be durably asymptomatic. The symptoms are a progressive clinical deterioration or acute spinal dysfunction (tetra or paraplegia) in case of hemorrhage. Cavernomas have a typical aspect with MRI in contrast with intramedullary gliomas. The lesion is often superficial, covered by the pia-mater, visible immediately after opening the dura, the approach is direct; but in few cases the cavernoma is deep seated in the spinal cord and not visible, the approach is through the midline. It is recommended to perform a complete "en bloc" resection. A yearly MRI control is necessary to search possible "de novo" cases.

Background: Intrinsic midbrain tumor has been one of the most challenging therapeutic tasks in neurosurgery because of its prognosis and risks associated with surgical procedures. We encountered a rare case of cavernoma located in the anterior-mesencephalic region presented as parkinsonism tremor., Case Description: A 42-year-old woman had a resting tremor for 10 years. This tremor involved the left shoulder and the proximal and distal limb, which was exacerbated by any attempted movement and became grossly uncontrollable. She showed a partial right ophthalmoplegia with mydriasis. Her right upper and lower extremities had normal strength, but her left extremities had three-fifth strength. Her sensation was intact. Magnetic resonance imaging demonstrated a popcorn-like rounded lesion in the right ventral midbrain adjacent to the medial cerebral peduncle. To access this anterior-medial portion of the midbrain, we chose a frontotemporal transsylvian route via an orbitozygomatic craniotomy. With a longitudinal pial incision between the frontopontine fibers and the pyramidal tracts in the peduncle, the cavernoma was totally removed en bloc. After the operation, the tremor dramatically disappeared. The muscle strength of her left lower extremity improved to four fifths, whereas the upper extremity was still the same. The preoperative left oculomotor palsy seemed to have no improvement., Conclusions: Clinically manifested cavernoma due to repeated hemorrhage needs surgical intervention. With an optimal surgical approach, fairly safe entry zones on the anterior face of the rostral brainstem may be accessible, which provides a successful resection of a mesencephalic cavernoma without postoperative complications.