Time course of tumorigenesis of a newly developed sporadic hemangioblastoma in an elderly patient: illustrative case.

BACKGROUND: von Hippel-Lindau disease-associated hemangioblastomas (HBs) account for 20%-30% of all HB cases, with the appearance of new lesions often observed in the natural course of the disease. By comparison, the development of new lesions is rare in patients with sporadic HB. OBSERVATIONS: A 65-year-old man underwent clipping for an unruptured aneurysm of the anterior communicating artery. Fourteen years later, follow-up magnetic resonance imaging (MRI) revealed a strongly enhanced mass in the right cerebellar hemisphere, diagnosed as a sporadic HB. A retrospective review of MRI studies obtained over the follow-up period revealed the gradual development of peritumoral edema and vascularization before mass formation. LESSONS: Newly appearing high-intensity T2 lesions in the cerebellum may represent a preliminary stage of tumorigenesis. Careful monitoring of these patients would be indicated, which could provide options for early treatment to improve patient outcomes.

Molecular insights and the role of 18F-FDG-PET/CT in the diagnosis of spinal gliomas.

BACKGROUND: In recent years, molecular findings on spinal gliomas have become increasingly important. This study aimed to investigate the role of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) in the diagnosis of spinal glioma. METHODS: This study included patients diagnosed with spinal cord glioma who underwent 18F-FDG-PET examination at the Department of Neurosurgery, Nagoya University Hospital between January 2016 and November 2023. The gliomas were divided into two groups, high-grade and low-grade, based on pathological and molecular studies. The maximum standardized uptake values (SUVmax) of the tumors were quantified and subsequently represented using receiver operating characteristic (ROC) curves. RESULTS: Eighteen participants were included in this study. Of the participants, seven had high-grade glioma with an SUVmax of 6.76 ± 0.72, and eleven had low-grade glioma with an SUVmax of 4.02 ± 1.78, and a statistically significant difference between the two groups. The ROC curve delineated an SUVmax cutoff value of 5.650, with an area under the curve (AUC) of approximately 0.909. Based on the cutoff value, the results of the diagnostic performance rendered a sensitivity and negative predictive value of 1.0, whereas the specificity and positive predictive value were 0.909 and 0.875, respectively. CONCLUSIONS: The present study shows that 18F-FDG-PET exhibits a markedly sensitive and negative predictive value in the assessment of spinal gliomas. Additionally, these findings have potential implications for the qualitative assessment of spinal gliomas using 18F-FDG-PET/CT. This imaging modality may be useful for making timely treatment decisions in situations where a detailed diagnosis by molecular analysis is not possible.

Comparative Study of Anterior Transvertebral Foraminotomy and Anterior Cervical Discectomy and Fusion for Unilateral Cervical Spondylotic Radiculopathy.

OBJECTIVE: To study the compared surgical and radiographic outcomes of Transvertebral foraminotomy (TVF) with anterior cervical discectomy and fusion (ACDF) in patients with unilateral cervical spondylotic radiculopathy (CSR). METHODS: We performed a retrospective comparative study of 72 consecutive patients with 1- or 2-level CSR treated with ACDF or TVF. 27 patients who underwent TVF (group T) and 45 patients who underwent ACDF (group A) with a minimum 2-year follow-up were enrolled. We evaluated clinical outcomes and radiological assessment. Clinical outcome included Visual analog scale (VAS) scores for axial, arm pain at preoperatively and final follow-up. VAS score for painful swallowing was also evaluated 1 week after surgery. Radiological assessment included C2-7 sagittal Cobb angle (C2-7 CA), range of motion (ROM) of C2-7 CA, the height, angle and ROM of the functional spinal unit (FSU), and tip of the spinous process of the operated segment. We also evaluated the disc height, FSU angle, and ROM of the FSU at the cranial adjacent segment. RESULTS: Both groups had good clinical outcomes. Soft tissue swelling was significantly less prominent in group T than that for group A. VAS scores for painful swallowing is lower in group T without significant difference. The ROM of C2-7 CA, FSU, and spinous processes demonstrated a significant reduction in group A compared with group T.(P < 0.05). Disc height at the cranial adjacent segment was maintained in group T. CONCLUSIONS: TVF is as effective as ACDF for unilateral CSR and preserves whole cervical spine and segmental alignment.

Spontaneous regression of asymptomatic tumefactive perivascular spaces in the anterior temporal lobe.

Perivascular spaces are fluid-filled spaces that surround the perforating vessels of the brain and are normal findings on brain imaging. These are usually asymptomatic and are considered a manifestation of aging. Perivascular spaces occasionally undergo significant enlargement and are referred to as tumefactive perivascular spaces, which are often indistinguishable from neoplastic lesions. Spontaneous regression of tumefactive perivascular spaces during follow-up is rare. We report the imaging findings and clinical course of a patient who showed spontaneous regression of tumefactive perivascular spaces in the anterior temporal lobe, together with a literature review and discussion regarding the characteristics and pathogenesis of spontaneous regression of tumefactive perivascular spaces. Most studies in the available literature report tumefactive perivascular spaces in the anterior temporal lobe; in our view, the characteristics of anterior temporal lobe tumefactive perivascular spaces may differ from those of tumefactive perivascular spaces that occur at other locations.

Pilocytic astrocytoma of the optic nerve with intracystic hemorrhage in an adult: illustrative case.

BACKGROUND: Optic pathway gliomas are uncommon, accounting for 3-5% of childhood brain tumors, and are mostly classified as pilocytic astrocytomas (PAs). PAs of the optic nerve are particularly rare in adults. OBSERVATIONS: The authors presented the case of PA of the left optic nerve in a 49-year-old woman along with detailed pathological and molecular analyses and sequential magnetic resonance imaging. The tumor had progressed during 5 years of follow-up along with cyst formation and intracystic hemorrhage; it had a thick capsule and contained xanthochromic fluid. The boundary between tumor and optic nerve was unclear. B-type Raf kinase (BRAF) V600E point mutations or translocations, IDH1-R132H mutations, loss of alpha-thalassemia/mental retardation X-linked, and 1p/19q codeletion were negative. LESSONS: BRAF alterations in pediatric PAs of the optic nerve are less frequent than those observed in PAs in other lesions; the same molecular pattern was observed in the adult case, without changes in BRAF. Surgical management should be indicated only in cases with severely impaired vision or disfigurement because there is no clear border between the tumor and optic nerve. Further discussion is needed to optimize the treatment for adult optic pathway gliomas, including radiotherapy, chemotherapy, and molecular-targeted therapies, in addition to surgical intervention.

Pure extraosseous spinal epidural cavernous hemangioma presenting with acute paraplegia: a case report.

INTRODUCTION: Spinal hemangiomas are benign vascular tumors that most commonly originate from the osseous structures of the spinal column. Epidural spinal hemangiomas without osseous involvement are uncommon and are classified as pure epidural spinal hemangiomas. Extraosseous spinal epidural cavernous hemangiomas are rarely described and among available reports; most patients present with slowly progressive neurological symptoms. Herein, we present a novel case of acute neurological dysfunction from a pure spinal epidural hemangioma that was managed through surgical resection with good neurological recovery at follow-up. CASE PRESENTATION: A 45-year-old previously healthy man presented to the emergency room with sudden inability to ambulate and was found to have bilateral lower extremity weakness. Magnetic resonance imaging of the spine demonstrated an epidural mass extending out of the right T5/6 neural foramen. The mass enhanced heterogeneously, and the preoperative diagnosis favored an atypical schwannoma. The lesion was surgically removed en-bloc through a midline posterior decompression with instrumentation. Histopathologic examination confirmed cavernous hemangioma pathology. Within 6 weeks of the surgical intervention, the patient had regained full sensorimotor function and these effects were durable through long term follow-up. DISCUSSION: Pure spinal epidural hemangiomas are rare and generally have an insidious clinical course. This case report highlights that these uncommon lesions may present with substantial and acute neurological dysfunction requiring urgent neurosurgical intervention. This should prompt clinicians to consider cavernous hemangioma in the differential diagnosis of patients presenting with acute neurological deterioration and an epidural spinal tumor.

Recent Molecular and Genetic Findings in Intramedullary Spinal Cord Tumors.

The study of genetic alterations and molecular biology in central nervous system (CNS) tumors has improved the accuracy of estimations of patient prognosis and tumor categorization. Therefore, the updated 2021 World Health Organization (WHO) classification includes various diagnostic genes, molecules, and pathways for diagnosis, as well as histological findings. These findings are expected both to have diagnostic applications and to facilitate new targeted therapies that target tumor-specific genetic changes and molecular biology. Intramedullary spinal cord tumors (IMSCTs) are rare CNS tumors that are difficult to treat because they occur in eloquent areas. Although the genetic underpinnings of IMSCTs remain unclear compared to their intracranial counterparts, the genetic characteristics of these tumors are gradually being revealed. Here, we describe the major changes in the new 2021 WHO classification and review the major types of IMSCTs, with an emphasis on their clinical features and genetic alterations.

Radiological Analysis of Minimally Invasive Microscopic Laminectomy for Lumbar Canal Stenosis with a Focus on Multilevel Stenosis and Spondylolisthesis.

OBJECTIVE: We retrospectively compared the radiological and clinical outcomes of two different surgical techniques (lumbar spinous process splitting laminectomy [LSPSL] and unilateral laminotomy for bilateral decompression [ULBD]) to treat lumbar spinal canal stenosis (LCS). METHODS: We performed a retrospective comparative study of 141 consecutive patients with an average age of 70.8 ± 9.4 years who had undergone LSPSL or ULBD for LCS between April 2015 and April 2019. None of the patients had developed remote fractures of the spinous processes using either technique. These cases were divided into 2 groups: group L, 73 patients who had undergone LSPSL from April 2015 to April 2017; and group U, 68 patients who had undergone ULBD from May 2017 to April 2019. The clinical and radiological outcomes and surgical complications at the 1-year postoperative follow-up period were evaluated. RESULTS: We found no significant differences in the operative time between the 2 groups. However, group U had had significantly less blood loss than group L. The facet joints were significantly well preserved in group U. We examined the multilevel and spondylolisthesis cases separately and found that both surgical procedures were equally effective and that the visual analog scale scores for back or leg pain and Japanese Orthopaedic Association scores had significantly improved postoperatively in each group. Group U showed better outcomes in terms of LCS recurrence, with 3 patients in the group L requiring repeat surgery. CONCLUSIONS: We found both ULBD and LSPSL to be safe and effective techniques for LCS, even for patients with spondylolisthesis and multilevel disease. ULBD was superior in terms of recurrence prevention, preservation of the facet joints, and less blood loss.

Piecemeal resection of aggressive vertebral hemangioma using real-time navigation-guided drilling technique.

Vertebral hemangiomas are the most common benign vertebral tumors and are usually asymptomatic. Aggressive subtypes of the tumor, called aggressive VHs (AVHs), can become symptomatic with extraosseous extensions and require surgical removal. We present a case of AVH in a 36-year-old man presenting with low back pain and right leg pain that persisted for three months. Imaging studies showed a Th12 vertebral tumor that extended into the spinal canal and was squeezing the spinal cord. Computed tomography (CT)-guided biopsy indicated vertebral hemangimoa. Following preoperative arterial embolization, piecemeal gross total resection was attained under navigation guidance. He was left with no neurological deficit and remained well at the 12-month postoperative folow-up. Since AVHs are benign tumor, piecemeal removal of the tumor can be selected. However, disadvantage of the approach include difficulty of making decision how much to remove the front part of the vertebral body close to thoracic descending aorta. Furthermore, when the tumor tissue is too hard to curett, manipulation in tight spaces near the spinal cord carries the risk of damaging it. Navigation-guided drill is highly helpful for real-time monitoring of ongoing tumor resection. It enables safely resection of the tumor especially in the anterior cortical surface of the vertebral body and easily resection even hard tumors. This method results in reducing residual tumor and maintaining safety resection.

Driver Genetic Mutations in Spinal Cord Gliomas Direct the Degree of Functional Impairment in Tumor-Associated Spinal Cord Injury.

Genetic analysis in glioma has been developed recently. Spinal cord glioma is less common than intracranial glioma. Thus, the clinical significance of genetic mutations in spinal cord gliomas remains unclear. Furthermore, because the spinal cord is an important communication channel between the brain and the rest of the body, increased attention should be paid to its functional prognosis. In this study, we investigated the functional prognosis and driver genetic mutations in eight patients with spinal cord gliomas (World Health Organization grade I, three cases; grade II, two cases; grade III/IV, three cases). IDH mutations were detected in all grade II cases and H3F3A mutations were detected in all grade III/IV cases. The functional status of grade I and II gliomas remained unchanged or improved 1 year after surgery, whereas grade III/IV gliomas remained unchanged or deteriorated. Spinal glioma progenitor cells with H3F3A mutations were associated with accelerated tumor-associated spinal cord injury, which led to functional impairment. Conversely, the presence of IDH mutations, which are rarely reported in spinal gliomas, indicated a relatively favorable functional prognosis.

Preoperative Intracranial Dissemination of Spinal Myxopapillary Ependymoma Attributed to Tumor Hemorrhage.

BACKGROUND: Spinal myxopapillary ependymoma (SME), generally considered a benign entity, can exhibit brain and whole-spine metastases as well as local recurrence after surgery. However, the presence of preoperative retrograde intracranial dissemination at the time of diagnosis is very rare. CASE DESCRIPTION: We report a case of SME in a 22-year-old man who presented with acute exacerbation of chronic back pain shooting down both thighs and weakness in both legs. Magnetic resonance imaging of the brain and whole spine showed an enhancing mass occupying the majority of the spinal canal at the L1-L2 level and multiple foci dissemination, including in the right pons, lateral midbrain, and occipital lobe, and at the C7, Th6, L4, and S2 levels of the spinal canal at the time of diagnosis. On gross total removal of the dominant tumor located at the L1-L2 level, severe intradural arachnoiditis and syrinx filled with xanthochromic cerebrospinal fluid was noted, indicating the presence of previous tumor hemorrhage. Histopathologic analysis of the tumor supported SME diagnosis, and <1% of cells showed Ki-67 expression. We speculated that distant retrograde dissemination could have been attributed to metastatic spread through cerebrospinal fluid caused by tumor hemorrhage, which may explain distant dissemination despite low expression of Ki-67. CONCLUSIONS: Screening of the whole brain and spine at the time of diagnosis is imperative when tumor is detected at any level of the neuraxis. The present case of SME with a preoperative intracranial lesion is the fifth case documented in the medical literature.

Clinical and Radiological Clues of Traumatic Craniocervical Junction Injuries Requiring Occipitocervical Fusion to Early Diagnosis.

OBJECTIVE: The purpose of this study is to find the clinical and radiographic characteristics of traumatic craniocervical junction (CCJ) injuries requiring occipitocervical fusion (OC fusion) for early diagnosis and surgical intervention. METHODS: We retrospectively reviewed 12 patients with CCJ injuries presenting to St. Michaels Hospital in Toronto who underwent OC fusion and looked into the following variables; (1) initial trauma data on emergency room arrival, (2) associated injuries, (3) imaging characteristics of computed tomography (CT) scan and magnetic resonance imaging (MRI), (4) surgical procedures, surgical complications, and neurological outcome. RESULTS: All patients were treated as acute spinal injuries and underwent OC fusion on an emergency basis. Patients consisted of 10 males and 2 females with an average age of 47 years (range, 18-82 years). All patients sustained high-energy injuries. Three patients out of 6 patients with normal BAI (basion-axial interval) and BDI (basion-dens interval) values showed visible CCJ injuries on CT scans. However, the remaining 3 patients had no clear evidence of occipitoatlantal instability on CT scans. MRI clearly described several findings indicating occipitoatlantal instability. The 8 patients with normal values of ADI (atlantodens interval interval) demonstrated atlantoaxial instability on CT scan, however, all MRI more clearly and reliably demonstrated C1/2 facet injury and/or cruciate ligament injury. CONCLUSION: We advocate measures to help recognize CCJ injury at an early stage in the present study. Occipitoatlantal instability needs to be carefully investigated on MRI in addition to CT scan with special attention to facet joint and ligament integrity.

Spontaneous Rupture of a Huge Presacral Tarlov Cyst Leading to Dramatic Neurologic Recovery.

BACKGROUND: This manuscript discusses the case of huge presacral Tarlov cysts (TCs) and the substantial neurologic recovery noted in the patient following spontaneous rupture of the most prominent cyst. Perineural or TCs are nerve root cysts, which are usually incidental findings on magnetic resonance imaging (MRI) and are most frequently observed in the sacral spine. Symptomatic lesions are rarely encountered. CASE DESCRIPTION: In this case, a 44-year-old woman presented with anal and vulva pain on the right side, and bladder and bowel dysfunction. MRI of the lumbosacral spine showed multiple huge bilateral TCs located within the presacral space from S1-3. There was a substantially large right-sided S3 cyst that was presumed to be responsible for her symptoms. Surgical intervention was considered; however, her symptoms improved significantly during the waiting period for surgery because of spontaneous rupture of the right-sided S3 cyst, as confirmed on follow-up MRI. On follow-up over a 1-year period, the patient had been very well with no recurrent symptoms. CONCLUSIONS: To our knowledge, this is the first report of spontaneous cyst rupture and resultant neurologic improvement in a case of symptomatic presacral TCs.

Neurosurgical versus endovascular treatment of spinal dural arteriovenous fistulas: a multicenter study of 195 patients.

OBJECTIVE: The purpose of the present study was to compare the treatment success rates of primary neurosurgical and endovascular treatments in patients with spinal dural arteriovenous fistulas (dAVFs). METHODS: Data from 199 consecutive patients with thoracic and lumbosacral spinal dAVFs were collected from 18 centers. Angiographic and clinical findings, the rate of initial treatment failure or recurrence by procedures, risk factors for treatment failure, complications, and neurological outcomes were statistically analyzed. RESULTS: Spinal dAVFs were frequently detected in the thoracic region (81%), fed by a single feeder (86%), and shunted into an intradural vein via the dura mater. The fistulous connection between the feeder(s) and intradural vein was located at a single spinal level in 195 patients (98%) and at 2 independent levels in 4 patients (2%). Among the neurosurgical (n = 145), and endovascular (n = 50) treatment groups of single dAVFs (n = 195), the rate of initial treatment failure or recurrence was significantly higher in the index endovascular treatment group (0.68% and 36%). A multivariate analysis identified endovascular treatment as an independent risk factor with significantly higher odds of initial treatment failure or recurrence (OR 69; 95% CI 8.7-546). The rate of complications did not significantly differ between the two treatment groups (4.1% for neurosurgical vs 4.0% for endovascular treatment). With a median follow-up of 26 months, improvements of ≥ 1 point in the modified Rankin Scale (mRS) score and Aminoff-Logue gait and Aminoff-Logue micturition grades were observed in 111 (56%), 121 (61%), and 79 (40%) patients, respectively. Independent risk factors for lack of improvement in the Aminoff-Logue gait grades were multiple treatments due to initial treatment failure or recurrence (OR 3.1) and symptom duration (OR 1.02). CONCLUSIONS: Based on data obtained from the largest and most recently assessed multicenter cohort, the present study shows that primary neurosurgery is superior to endovascular treatment for the complete obliteration of spinal dAVFs by a single procedure.

Thoracic Disc Herniation Manifesting as Abdominal Pain Alone Associated with Thoracic Radiculopathy.

Symptomatic thoracic disc herniation (TDH) with thoracic radiculopathy alone is an extremely rare condition. Here, we report a rare case of TDH in a 52-year-old man who presented with medically refractory severe right flank pain. Based on pain distribution, pain intensity changes according to truncal position, ineffectiveness of intercostal nerve block, and radiological findings, his pain was determined to be caused by TDH at T9-10 level. Symptomatic TDH often requires invasive surgery. However, TDH with radiculopathy alone can be treated via a posterior approach without spinal cord manipulation or spinal fusion. We could eliminate the pain by removing TDH with hemilaminectomy and microdiscectomy using an O-arm-based navigation system.

Possible Double Crush Syndrome Caused by Iatrogenic Acquired Lumbosacral Epidermoid Tumor and Concomitant Sacral Tarlov Cyst.

We describe a rare case of 42-year-old female who had possible double crush syndrome caused by iatrogenic spinal epidermoid tumor (ET) associated with lumbar puncture as well as concomitant sacral Tarlov cyst in close proximity. She presented with progressive left-sided perianal pain. She had a history of a Caesarean section with lumbar spinal anesthesia. Magnetic resonance imaging (MRI) demonstrated a relatively small intradural extramedullary solid lesion at L5/S1 level and cystic lesion at S2 level. We considered there were two different lesions, such as a tumor and Tarlov cyst; however, we could not rule out the possibility of a single lesion with two different components. Furthermore, there was a distinct compression at more than one locations along the course of the left S2 nerve root and we suspected possible double crush syndrome. We conducted tumor removal and the lesion turned out to be two different pathologies, such as an ET and Tarlov cyst. Both lesions were intraopertively pinching the left S2 nerve root at different sites as expected. The tumor was successfully removed and the cyst wall was imbricated and sutured. We need to take the possibility of ET into consideration if the patient underwent invasive spinal procedure previously. We also have to pay attention to the possibility of double crush syndrome if the nerve root possibly holding the responsibility for symptoms is compressed at two or more sites. This is the first report of possible double crush syndrome caused by acquired spinal tumor and congenital Tarlov cyst.

Intraosseous Melanotic Schwannoma in the Sacrum Mimicking Primary Bone Tumor.

Primary tumors of sacrum are rarely seen, and the differential diagnosis is extensive, such as chordomas, giant cell tumors, and schwannomas. Sacral intraosseous schwannomas (IOSs) are very rare and encompass approximately 1%-5% of all spinal schwannomas. Melanotic schwannomas (MSs) are categorized as an unusual variant of benign schwannomas; however, they sometimes follow a malignant course. The authors present a case of MS with intraosseous extension into sacrum in a 48-year-old male arising from the left S2 nerve root. Magnetic resonance imaging (MRI) and computed tomography (CT) scan demonstrated a destructive mass in the sacrum. He was made a diagnosis with MS by 18F-fluoro-deoxy-glucose positron-emission-tomography (18F-FDG PET) and open biopsy. The tumor was blackish-colored and vascular-rich fragile tumor covered by fibrous capsule. The floor of the tumor was not encapsulated and invading into the sacral bone. Total removal of the tumor together with the left S2 nerve of origin via posterior approach was achieved. The patient made dramatic recovery of neurological symptoms and tumor recurrence is not seen for 6-month follow-up period. MS is a benign tumor with potential for aggressive behavior and capacity to metastasize. Therefore, total removal of the tumor and careful postoperative follow-up are recommended. Postoperative spinopelvic stability also needs to be taken into consideration. The authors discuss our successful management with a focus on diagnostic process, surgical planning, and histological consideration to provide the most up-to-date guidance on managing this challenging tumor.

Microsurgical versus endovascular treatment of spinal epidural arteriovenous fistulas with intradural venous drainage: a multicenter study of 81 patients.

OBJECTIVE: Spinal arteriovenous shunts are rare vascular lesions and are classified into 4 types (types I-IV). Due to rapid advances in neuroimaging, spinal epidural AVFs (edAVFs), which are similar to type I spinal dural AVFs (dAVFs), have recently been increasingly reported. These 2 entities have several important differences that influence the treatment strategy selected. The purposes of the present study were to compare angiographic and clinical differences between edAVFs and dAVFs and to provide treatment strategies for edAVFs based on a multicenter cohort. METHODS: A total of 280 consecutive patients with thoracic and lumbosacral spinal dural arteriovenous fistulas (dAVFs) and edAVFs with intradural venous drainage were collected from 19 centers. After angiographic and clinical comparisons, the treatment failure rate by procedure, risk factors for treatment failure, and neurological outcomes were statistically analyzed in edAVF cases. RESULTS: Final diagnoses after an angiographic review included 199 dAVFs and 81 edAVFs. At individual centers, 29 patients (36%) with edAVFs were misdiagnosed with dAVFs. Spinal edAVFs were commonly fed by multiple feeding arteries (54%) shunted into a single or multiple intradural vein(s) (91% and 9%) through a dilated epidural venous plexus. Preoperative modified Rankin Scale (mRS) and Aminoff-Logue gait and micturition grades were worse in patients with edAVFs than in those with dAVFs. Among the microsurgical (n = 42), endovascular (n = 36), and combined (n = 3) treatment groups of edAVFs, the treatment failure rate was significantly higher in the index endovascular treatment group (7.5%, 31%, and 0%, respectively). Endovascular treatment was found to be associated with significantly higher odds of initial treatment failure (OR 5.72, 95% CI 1.45-22.6). In edAVFs, the independent risk factor for treatment failure after microsurgery was the number of intradural draining veins (OR 17.9, 95% CI 1.56-207), while that for treatment failure after the endovascular treatment was the number of feeders (OR 4.11, 95% CI 1.23-13.8). Postoperatively, mRS score and Aminoff-Logue gait and micturition grades significantly improved in edAVFs with a median follow-up of 31 months. CONCLUSIONS: Spinal epidural AVFs with intradural venous drainage are a distinct entity and may be classified as type V spinal vascular malformations. Based on the largest multicenter cohort, this study showed that primary microsurgery was superior to endovascular treatment for initial treatment success in patients with spinal edAVFs.

Arterial Spin Labeling Magnetic Resonance Imaging in the Assessment of Non-Convulsive Status Epilepticus in Alzheimer's Disease: A Report of Two Cases.

BACKGROUND The diagnosis of early non-convulsant status epilepticus (NCSE) can be challenging and can overlap with other critical conditions. Two patients with Alzheimer's disease are reported with clinically suspected NCSE presenting in the emergency setting who were diagnosed using arterial spin-labeling magnetic resonance imaging (ASL-MRI) sequences. CASE REPORT In Case 1, a 69-year-old woman with mild Alzheimer's disease and diabetes presented with acute worsening of cognitive status and fluctuating level of consciousness. In Case 2, a 70-year-old man with mild cognitive impairment due to Alzheimer's disease and hypertension presented with acute loss of consciousness and left hemiparesis, without evidence of hypoglycemia or a hypertensive crisis. In both cases, ASL-MRI perfusion images showed focal cerebral hyperperfusion in the posterior cingulate and parietal associative cortex, which involved neurodegenerative areas associated with epilepsy in early Alzheimer's disease. In both cases, the patients developed generalized tonic-clonic epileptic seizures that lasted for 5 minutes or more, which indicated the emergence of status epilepticus that developed from the initial presentation of NCSE. In both cases, electroencephalogram (EEG) findings confirmed that the seizures were controlled by intravenous administration of antiepileptic drugs. Both patients discharged home from the hospital without recurrence of seizures, between 10-12 days after the onset of symptoms. CONCLUSIONS These two cases have demonstrated that ASL-MRI is feasible as an emergency diagnostic tool in clinically suspected NCSE in patients with Alzheimer's disease.

Chiari Type 1 Malformation-induced Intracranial Hypertension with Diffuse Brain Edema Treated with Foramen Magnum Decompression: A Case Report.

Chiari type 1 malformation (CM1) rarely causes papilloedema, which is indicative of high intracranial pressure with or without ventricular dilatation. Furthermore, concomitant brain parenchymal abnormalities have not been reported to date. In this paper, the authors report on a young woman of CM1-induced intracranial hypertension (ICH) with diffuse brain edema with a focus on venous sinus assessment, and discuss the surgical strategy. A 24-year-old woman presented to Nagoya University Hospital complaining of 4-year history of severe occipital headache and blurry vision with slowly progressive worsening. Head and whole spine MRI showed a CM1 with diffuse white matter hyperintensities (WMH) on T2-weighted imaging and narrowed brain sulci without hydrocephalus. Lumbar puncture revealed extremely high opening pressure. Detailed blood examination and other radiographical imaging studies denied the presence of tumor, collagen disease, encephalitis and other entities. Head magnetic resonance venography and angiography demonstrated severe transverse sinus stenosis on both sides. Foramen magnum decompression was performed to alleviate the ICH by restoration of cerebrospinal fluid (CSF) stagnation at the foramen magnum with successful outcome. The patient completely recovered from preoperative symptoms immediately after surgery. The diffuse WMH and narrowing brain sulci have been resolving. The most feasible explanation for this complicated pathophysiology was ICH induced by CM1 led to transverse sinus collapse, resulting in diffuse WMH as a result of venous hypertension. This case report is the first illustration of successful surgical treatment of CM1 with diffuse brain edema with a focus on venous sinus assessment.