Relationships between the global symptom score and electrophysiological findings after surgical release for carpal tunnel syndrome: Indications and outcomes.

PURPOSE: Regarding surgical indications for carpal tunnel syndrome (CTS), the hypothesis that the recovery processes of subjective symptoms differ among pain, sensory, and motor symptoms and correlate with recovery in objective nerve conduction studies was examined in the present study. METHODS: The global symptom score (GSS) is a method used to assess clinical outcomes and covers subjective symptoms, including pain (pain and nocturnal awakening), sensory (numbness and paresthesia), and motor (weakness/clumsiness) symptoms. The relationships between long-term changes in GSS and recovery in nerve conduction studies were investigated. RESULTS: Forty patients (40 hands) were included (mean age 65 years; 80% female; 68% with moderate CTS: sensory nerve conduction velocity < 45 m/s and motor nerve distal latency > 4.5 ms). Pain and nocturnal awakening rapidly subsided within 1 month after surgery and did not recur in the long term (median 5.6 years). Paresthesia significantly decreased 3 months after surgery and in the long term thereafter. Weakness/clumsiness significantly decreased at 1 year. Sensory nerve distal latency, conduction velocity, and amplitude significantly improved 3 months and 1 year after surgery, and correlated with nocturnal awakening in the short term (3 months) in moderate CTS cases. The patient satisfaction rate was 91%. CONCLUSION: Rapid recovery was observed in pain and nocturnal awakening, of which nocturnal awakening correlated with the recovery of sensory nerve conduction velocity. Patients with pain symptoms due to moderate CTS may benefit from surgical release.

Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation.

Ependymomas encompass multiple clinically relevant tumor types based on localization and molecular profiles. Tumors of the methylation class "spinal ependymoma" (SP-EPN) represent the most common intramedullary neoplasms in children and adults. However, their developmental origin is ill-defined, molecular data are scarce, and the potential heterogeneity within SP-EPN remains unexplored. The only known recurrent genetic events in SP-EPN are loss of chromosome 22q and NF2 mutations, but neither types and frequency of these alterations nor their clinical relevance have been described in a large, epigenetically defined series. Transcriptomic (n = 72), epigenetic (n = 225), genetic (n = 134), and clinical data (n = 112) were integrated for a detailed molecular overview on SP-EPN. Additionally, we mapped SP-EPN transcriptomes to developmental atlases of the developing and adult spinal cord to uncover potential developmental origins of these tumors. The integration of transcriptomic ependymoma data with single-cell atlases of the spinal cord revealed that SP-EPN display the highest similarities to mature adult ependymal cells. Unsupervised hierarchical clustering of transcriptomic data together with integrated analysis of methylation profiles identified two molecular SP-EPN subtypes. Subtype A tumors primarily carried previously known germline or sporadic NF2 mutations together with 22q loss (bi-allelic NF2 loss), resulting in decreased NF2 expression. Furthermore, they more often presented as multilocular disease and demonstrated a significantly reduced progression-free survival as compared to SP-EP subtype B. In contrast, subtype B predominantly contained samples without NF2 mutation detected in sequencing together with 22q loss (monoallelic NF2 loss). These tumors showed regular NF2 expression but more extensive global copy number alterations. Based on integrated molecular profiling of a large multi-center cohort, we identified two distinct SP-EPN subtypes with important implications for genetic counseling, patient surveillance, and drug development priorities.

Angiographic challenges of spinal dural and epidural arteriovenous fistulas: report on 45 cases.

PURPOSE: The localization of the fistula level in spinal dural arteriovenous fistulas (dAVFs) and epidural arteriovenous fistulas (edAVFs) remains a diagnostic challenge. METHODS: Consecutive patients with spinal dAVFs and edAVFs in the thoracic, lumbar, and sacral regions were included. The primary endpoint was to describe the characteristics of patients who required angiography with multiple catheterizations of segmental arteries (10 or more). RESULTS: Forty-five patients (median age 69 years; male 89%; dAVFs, n = 31; edAVFs, n = 14) were included. Spinal dAVFs commonly developed in the thoracic region and edAVFs in the lumbosacral region. Fistulas were predicted at the correct level or plus/minus 2 level in less invasive examinations using multi-detector CT angiography (n = 28/36, 78%) and/or contrast-enhanced MR angiography (n = 9/14, 64%). We encountered diagnostic challenges in the localization of fistulas in 6 patients. They underwent angiography a median of 2 times. In each patient, spinal levels were examined at a median of 25 levels with a median radiation exposure of 3971 mGy and 257 ml of contrast. Fistulas were finally localized at the high thoracic region (T4-6) in 3 patients, the sacral region (S1-2) in 2, and the lumbar region (L3) in 1. Four patients were diagnosed with edAVFs and 2 with dAVFs. The correlation coefficient between the fistula level and the rostral end of the intramedullary T2 high-signal intensity on MRI was interpreted as none. CONCLUSION: In patients in whom less invasive examinations failed for fistula localization, high thoracic or sacral AVFs need to be considered.

Small Thoracic Disk Herniation without Spinal Stenosis Presenting with Acute Myelopathy: Three Case Reports.

We herein describe three patients with thoracic disk herniation (TDH) that presented with acute myelopathy at the Tokyo Metropolitan Neurological Hospital between 2014 and 2021 (age range, 45-76 years; male/female ratio = 1:2), with a focus on the mechanisms underlying their development. All patients had sudden-onset gait disturbance due to acute nontraumatic paraparesis. The specialties of the doctors at the first hospital were neurology and orthopedic surgery. TDH was overlooked at the first hospital, and the patients were referred to our hospital. The TDH in all cases was of the central type; however, since they were small, no spinal stenosis was observed. The key feature of all three cases is the small anterior deformation of the spinal cord, making a vascular etiology for the symptoms more plausible than a compressive etiology. After a follow-up of several months or years, two out of three patients underwent surgery with the use of the transfacet pedicle-sparing approach due to residual symptoms. Intraoperative ultrasonography showed that the spinal cord was anchored to TDH by the dural attachment of dentate ligaments. The physical relationship between the dentate ligaments and TDH may be associated with the vascular cause of the symptoms of small TDH.

Spinal Cord Subependymoma: A Subanalysis of the Neurospinal Society of Japan's Multicenter Study of Intramedullary Spinal Cord Tumors.

OBJECTIVE: This study aimed to analyze the clinical characteristics, treatment strategies, and surgical outcomes of subependymoma patients from the 2022 Neurospinal Society of Japan multicenter intramedullary spinal cord tumor study. METHODS: Twenty-six patients with spinal cord subependymoma who were included in the index study of 1,033 patients were retrospectively analyzed. RESULTS: Mean patient age was 49.4 years. Seventeen patients were men and 9 were women. Sensory disturbance was reported in 22 patients and motor weakness in 18. Median duration of symptoms was 24 months. The tumor was eccentrically located in 19 patients (73.1%) and unilateral in 17 (65.4%). Gross total resection was achieved in 6 patients (23.1%). The same rate for ependymoma patients in the index study was significantly higher (74.8%). Median follow-up was 40.5 months (interquartile range, 18-68 months). In 2 patients who underwent only partial resection, reoperation was required owing to progression 68 and 90 months after surgery, respectively. No recurrence occurred in patients who underwent gross total resection. Five patients experienced neurological worsening after surgery. CONCLUSION: Although spinal cord subependymoma can be difficult to distinguish from other intramedullary spinal cord lesions before surgery, it is characterized by an indolent clinical course and eccentric location. Surgical treatment should prioritize functional preservation because the prognosis is good even after subtotal resection.

Surgical and Endovascular Treatments for Asymptomatic Arteriovenous Fistulas at the Craniocervical Junction: A Multicenter Study.

OBJECTIVE: Asymptomatic craniocervical junction arteriovenous fistulas (CCJ AVFs) are rare and, thus, a consensus has not yet been reached regarding the indication of surgical interventions. This retrospective multicenter cohort study investigated the risks associated with surgery for asymptomatic CCJ AVFs and discussed the indication of surgical interventions. METHODS: Using data from 111 consecutive patients with CCJ AVFs registered with the Neurospinal Society of Japan between 2009 and 2019, we analyzed the treatment, complications, and outcomes of 18 patients with asymptomatic CCJ AVF. RESULTS: The median age of the patient cohort was 68 years (37-80 years), and there were 11 males and 7 females. Diagnoses were 14 patients with dural AVF, one perimedullary AVF, one radicular AVF, one epidural AVF, and one bilateral dural and epidural AVF. Initial treatment included direct surgery in 12 patients, endovascular treatment in four, and conservative treatment in two. Among 16 patients, three complications (18.7%) occurred: spinal cord infarction associated with the surgical procedure, cerebral infarction associated with intraoperative angiography, and mortal medullary hemorrhage after endovascular treatment followed by open surgery. Complete occlusion was achieved in all 12 patients in the direct surgery group and in one out of four in the endovascular treatment group. CONCLUSIONS: Given the risk of serious complications associated with asymptomatic CCJ AVF and the fact that no case of asymptomatic CCJ AVF became symptomatic in this study, prophylactic surgery for asymptomatic CCJ AVF should be carefully considered.

Congestive myelopathy due to craniocervical junction arteriovenous fistulas mimicking transverse myelitis: a multicenter study on 27 cases.

BACKGROUND: The purpose was to clarify diagnostic clues and pitfalls in cranio-cervical junction arteriovenous fistulas (CCJ AVFs) with congestive myelopathy. METHODS: In a multicenter observational study by the Neurospinal Society of Japan, we described the demographics, clinical courses, imaging findings, and outcomes of consecutive patients with CCJ AVFs presenting with congestive myelopathy between 2009 and 2019. RESULTS: Twenty-seven patients were included (mean age, 70 years; male, 96%). Progressive symptoms within one day to one month were more common (63%) than chronic symptoms. Myelopathic symptoms were characterized by ascending paralysis beginning from the legs, involving the trunk and arms, and sometimes ending in the brainstem. Fifteen patients (56%) received a misdiagnosis, including acute transverse myelitis. The most common MRI findings were venous congestive edema of the cervical cord (96%) and the brainstem (63%) and surrounding vascular flow voids (100%). The mean extension of congestive edema was 5.5 ± 2.9 vertebral segments. The most common angiographic findings were a dural AVF (78%) at the C1 level (81%) with descending venous drainage (85%). Seven patients (26%) were administered steroids, which resulted in neurological decline in 3. Neurosurgical obliteration of the AVF led to improvements in MRI findings in 75% and a functional status in 67%; however, 44% remained dependent. CONCLUSIONS: The myelopathy of CCJ AVFs was characterized by acute ascending paralysis in elderly men. A misdiagnosis was common because of the acute presentation due to a longitudinally extensive spinal cord lesion. Dilated vessels on MRI were a key finding for the correct diagnosis. What is already known on this topic? Slowly progressive myelopathy is a well-known symptom that results from impaired spinal venous drainage due to thoracolumbar AVFs. Although cranio-cervical junction arteriovenous fistulas (CCJ AVFs) constitute a treatable cause of congestive myelopathy, detailed information is not currently available due to their rarity. What does this study add? CCJ AVFs often presented with acute ascending myelopathy in elderly men due to a longitudinally extending cervical cord lesion with surrounding flow voids. Steroid pulse therapy was not effective or even harmful to congestive myelopathy, while neurosurgical treatment effectively obliterated AVFs. How might this study affect research, practice or policy? The results obtained revealed diagnostic clues and pitfalls from the largest dataset of patients with CCJ AVFs in a multicenter cohort.

Sacral myolipoma with involuntary contraction causing tethered cord syndrome: illustrative case.

BACKGROUND: Spinal lipomas sometimes involve various ectopic tissues originating from the ectoderm, mesoderm, and endoderm in the process of morphological development. OBSERVATIONS: A 29-year-old male patient with myolipoma of the conus medullaris at the S2 and S3 levels was described. The unusual finding, involuntary muscle contraction, was presented in an operative video and a literature review. In the present case, sacral myolipoma with involuntary contraction caused tethered cord syndrome in adulthood, and untethering surgery resolved continuous buttock and leg pain. LESSONS: This rare finding is considered a surgical indication for adult patients with myolipoma.

Ischemic complications in the neurosurgical and endovascular treatments of craniocervical junction arteriovenous fistulas: a multicenter study.

OBJECTIVE: A recent comparative analysis between neurosurgical and endovascular treatments for craniocervical junction (CCJ) arteriovenous fistulas (AVFs) revealed better treatment outcomes in the neurosurgery group than in the endovascular group. This finding was attributed to the higher than expected rate of ischemic complications in the endovascular group than in the neurosurgery group (26% vs 7.7%, p = 0.037). The aim of the present study was to describe ischemic complications associated with treatments for CCJ AVFs. METHODS: This descriptive study was authorized by the Neurospinal Society of Japan. Data from 97 consecutive patients with CCJ AVFs who underwent neurosurgical (n = 78) or endovascular (n = 19) treatment between 2009 and 2019 were collected from 29 centers. The primary endpoints were details on ischemic complications and their risk factors. Secondary endpoints were details on other complications. RESULTS: Among all major complications, ischemic complications were the most common (11% of 97 patients), followed by hemorrhagic complications (7.2%), hydrocephalus (2.1%), and CSF leakage (2.1%). Ischemic complications included 8 spinal, 2 brainstem, and 1 cerebellar infarctions. Iatrogenic occlusion of the anterior or posterior spinal artery from the radiculomedullary or radiculopial arteries caused these complications. Ischemic complications resulted in neurological deficits, including motor paresis, sensory disturbances, and brainstem dysfunction. The modified Rankin Scale score was 3 or higher in 36% of patients with ischemic complications at the final follow-up of 23 months. Risk factors associated with ischemic complications were endovascular treatment (OR 4.3, 95% CI 1.1-16) and spinal feeding arteries (OR 3.8, 95% CI 1.03-14). Most of the other complications were addressed by additional treatment without permanent neurological deficits. CONCLUSIONS: Among ischemic complications associated with treatments for CCJ AVFs, spinal infarctions were the most common and were mostly attributed to endovascular procedures for CCJ AVFs fed by spinal arteries. These results support the use of neurosurgery as the first-line treatment for CCJ AVFs.

Neurosurgical versus endovascular treatment of craniocervical junction arteriovenous fistulas: a multicenter cohort study of 97 patients.

OBJECTIVE: Craniocervical junction (CCJ) arteriovenous fistulas (AVFs) are treated using neurosurgical or endovascular options; however, there is still no consensus on the safest and most effective treatment. The present study compared the treatment results of neurosurgical and endovascular procedures for CCJ AVFs, specifically regarding retreatment, complications, and outcomes. METHODS: This was a multicenter cohort study authorized by the Neurospinal Society of Japan. Data on consecutive patients with CCJ AVFs who underwent neurosurgical or endovascular treatment between 2009 and 2019 at 29 centers were analyzed. The primary endpoint was the retreatment rate by procedure. Secondary endpoints were the overall complication rate, the ischemic complication rate, the mortality rate, posttreatment changes in the neurological status, independent risk factors for retreatment, and poor outcomes. RESULTS: Ninety-seven patients underwent neurosurgical (78 patients) or endovascular (19 patients) treatment. Retreatment rates were 2.6% (2/78 patients) in the neurosurgery group and 63% (12/19 patients) in the endovascular group (p < 0.001). Overall complication rates were 22% and 42% in the neurosurgery and endovascular groups, respectively (p = 0.084). Ischemic complication rates were 7.7% and 26% in the neurosurgery and endovascular groups, respectively (p = 0.037). Ischemic complications included 8 spinal infarctions, 2 brainstem infarctions, and 1 cerebellar infarction, which resulted in permanent neurological deficits. Mortality rates were 2.6% and 0% in the neurosurgery and endovascular groups, respectively (p > 0.99). Two patients died of systemic complications. The percentages of patients with improved modified Rankin Scale (mRS) scores were 60% and 37% in the neurosurgery and endovascular groups, respectively, with a median follow-up of 23 months (p = 0.043). Multivariate analysis identified endovascular treatment as an independent risk factor associated with retreatment (OR 54, 95% CI 9.9-300; p < 0.001). Independent risk factors associated with poor outcomes (a postoperative mRS score of 3 or greater) were a pretreatment mRS score of 3 or greater (OR 13, 95% CI 2.7-62; p = 0.001) and complications (OR 5.8; 95% CI 1.3-26; p = 0.020). CONCLUSIONS: Neurosurgical treatment was more effective and safer than endovascular treatment for patients with CCJ AVFs because of lower retreatment and ischemic complication rates and better outcomes.

Nonrheumatoid Retro-Odontoid Pseudotumors: Characteristics, Surgical Outcomes, and Time-Dependent Regression After Posterior Fixation.

OBJECTIVE: Although a retro-odontoid pseudotumor associated with rheumatoid arthritis is a well-known clinical entity, little is known about retro-odontoid pseudotumors not associated with rheumatoid arthritis due to their rarity. METHODS: Between 2006 and 2019, consecutive patients with nonrheumatoid pseudotumors were included and retrospectively compared with patients with rheumatoid pseudotumors. RESULTS: Nineteen patients had nonrheumatoid pseudotumors (mean age, 73 ± 6 years; male, 53%). All had cervical lesions including ossified anterior and posterior longitudinal ligaments with a history of cervical surgery in 5. The mean thickness of the pseudotumors at diagnosis was 8.1 mm (range, 4.2-17.2 mm). Pseudotumor thickness had a significant negative correlation with the atlantodental interval (p = 0.008) and the subaxial range of motion (p = 0.049). In comparison with 7 rheumatoid pseudotumor patients, nonrheumatoid pseudotumor patients were older (p = 0.042), had a higher proportion of males (p = 0.023), had a smaller atlantodental interval (p = 0.007), and had larger pseudotumors at diagnosis (p = 0.030). Of the 19 patients, 18 received posterior fixation with or without C1 laminectomy, while the other received C1 laminectomy alone. The percent pseudotumor thickness at follow-up to those at diagnosis was 91%, 77%, 68%, 46%, 58%, and 49% at 1, 3, 6, 12, 24, and 36 months after surgery, respectively. CONCLUSION: This study revealed markedly clinical and radiological differences between nonrheumatoid and rheumatoid pseudotumors. The main etiology for nonrheumatoid pseudotumors was subaxial cervical degeneration and ossified lesions. There were good outcomes following posterior fixation and time-dependent pseudotumor regression within 12 months.

Superficial siderosis of the central nervous system associated with ventral dural defects: bleeding from the epidural venous plexus.

OBJECTIVE: Superficial siderosis of the central nervous system is a rare intractable disease induced by chronic subarachnoid hemorrhage. Neurological deficits, such as cerebellar ataxia and hearing difficulties, gradually progress if left undiagnosed. Hemosiderin deposition is irreversible because standard medical treatment has not yet been established. Interventions at the source of bleeding may be the key to a preferable outcome of treatment for chronic subarachnoid hemorrhage; however, the source is not clear in many cases. METHODS: Among the consecutive cases diagnosed with a spontaneous cerebrospinal fluid (CSF) leak, cases of superficial siderosis associated with a CSF leak due to a ventral dural defect were retrospectively analyzed. RESULTS: Among 77 cases of a CSF leak, 7 cases (9%) of superficial siderosis were identified (median age of 59 years, male, 4 cases). Defects were diagnosed on 1-mm sliced fast imaging employing steady-state acquisition MRI (n = 5), conventional myelographic CT (n = 1), or dynamic myelographic CT (n = 1) at high thoracic levels (T1-T4). All defects were repaired by direct neurosurgery. During surgery, continuous bleeding from the epidural veins of the internal vertebral venous plexus was identified as the source of subarachnoid hemorrhage. Epidural CSF pulsations through the defect prevented clot formation by the epidural veins. Dural repair stopped free communication between the subarachnoid and epidural spaces, leading to the disappearance of chronic subarachnoid hemorrhage. CONCLUSION: Bleeding from the epidural venous plexus may be the cause of superficial siderosis associated with ventral dural defects. Neurosurgical repair may stop the progression of this condition.

Intraoperative Anatomical Findings in Pediatric Clear Cell Meningioma of the Lumbar Spine: Case Report and Literature Review.

Clear cell meningioma (CCM) is a WHO classification Grade II meningioma. It is a very rare disease, of which only 41 cases of spinal cord CCM in children have been reported to date. CCMs sometimes do not have the "dural attachment" that is usually found in meningiomas, and our understanding of the origin of CCMs is therefore controversial. We hereby present a case of pediatric CCM of the lumbar spine, in which we examined intraoperatively, the detailed anatomical location of the tumor. The case is a 10-year-old boy, who presented to our hospital with a 2-month history of lower back and bilateral lower extremity pain upon waking, which gradually worsened. Lumbar spine CT and MRI revealed an intradural extramedullary tumor at the L3 vertebral level, and surgery was performed to remove it. The tumor was in close contact with the dura mater, and also in contact with the cauda equina via the arachnoid. The tumor was likely located primarily between the dura mater and arachnoid. The pathological diagnosis was CCM, with an MIB-1 index of less than 1%. His back pain and bilateral lower extremity pain improved after surgery, and he was discharged from our hospital. Postoperative radiation therapy was not performed. Based on this case, we suggest that intraoperative examination of the anatomical location of these tumors and accumulation of relevant experience are important to elucidate the embryological mechanisms of this rare disease.

Microsurgical anatomy and treatment of dural defects in spontaneous spinal cerebrospinal fluid leaks.

OBJECTIVE: Spontaneous spinal CSF leaks are caused by abnormalities of the spinal dura mater. Although most cases are treated conservatively or with an epidural blood patch, some intractable cases require neurosurgical treatment. However, previous reports are limited to a small number of cases. Preoperative detection and localization of spinal dural defects are difficult, and surgical repair of these defects is technically challenging. The authors present the anatomical characteristics of dural defects and surgical techniques in treating spontaneous CSF leaks. METHODS: Among the consecutive patients who were diagnosed with spontaneous CSF leaks at the authors' institution between 2010 and 2020, those who required neurosurgical treatment were included in the study. All patients' clinical information, radiological studies, surgical notes, and outcomes were reviewed retrospectively. Outcomes of two different procedures in repairing dural defects were compared. RESULTS: Among 77 patients diagnosed with spontaneous CSF leaks, 21 patients (15 men; mean age 57 years) underwent neurosurgery. Dural defects were detected by FIESTA MRI in 7 patients, by CT myelography in 12, by digital subtraction myelography in 1, and by dynamic CT myelography in 1. The spinal levels of the defects were localized at the cervicothoracic junction in 16 patients (76%) and thoracolumbar junction in 4 (19%). Intraoperative findings revealed that the dural defects were small, circumscribed longitudinal slits located at the ventral aspect of the dura mater. The median dural defect size was 5 × 2 mm. The presence of dural defects at the thoracolumbar junction was associated with manifestation of an altered mental status, which was an unusual manifestation of CSF leaks (p = 0.003). Eight patients were treated via the posterior transdural approach with watertight primary sutures of the ventral defects, and 13 were treated with muscle or fat grafting. Regardless of the two different procedures, postoperative MRI showed either complete disappearance or significant reduction of the extradural CSF collection. No patient experienced postoperative neurological deficits. Clinical symptoms improved or stabilized in 20 patients with a median follow-up of 12 months. CONCLUSIONS: Dural defects in spontaneous CSF leaks were small, circumscribed longitudinal slits located ventral to the spinal cord at either the cervicothoracic or thoracolumbar junction. Muscle/fat grafting may be an alternative treatment to watertight primary sutures of ventral dural defects with a good outcome.

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.

The algorithm for diagnosis and management of intracranial hypotension with coma: Report of two cases.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is caused by spontaneous cerebrospinal fluid (CSF) leaks that can be treated in most cases with an epidural blood patch (EBP). However, some patients, who develop severe brain sagging, can neurologically deteriorate, and in occasional instances, which become comatose. Here, with the presentation of two cases, and a review of the literature, we have set guidelines for diagnosing SIH along with recommendations for its management. CASE DESCRIPTION: We reviewed two cases of SIH. Both patients became comatose due to a CSF leak associated with a tear in the spinal dura diagnosed on myelo-CT studies. As targeted EBP failed to achieve sustained improvement, direct operative repair of the dural tears was warranted (video presentation). CONCLUSION: After reviewing two cases of SIH and the literature, we developed an algorithm for the diagnosis and management of SIH. To avoid deterioration to a comatose status, we recommend the early performance of myelo- CT studies to identify the location of the dural leak, followed by early dural repair.

Retro-odontoid Pseudotumor: Two Cases of Intradural Ganglion Cysts Arising From the Odontoid Process with Syringobulbia.

BACKGROUND: Ganglion cysts mostly occur in the knuckles and wrists, but they rarely present in the odontoid process and can cause neurological symptoms by compressing the spinal cord. They are mostly localized in the epidural space, but may very rarely appear in the intradural space. There are no reports of cases of intradural ganglion cyst involving syringobulbia. CASE DESCRIPTION: We report the presentation and management of 2 cases of an intradural ganglion cyst of the odontoid process. Several treatment options for ganglion cysts of the odontoid process have been reported, such as rest and use of a neck collar, posterior decompression and fusion, and transoral anterior decompression. Because our 2 cases progressed rapidly and had severe neurological symptoms, surgical treatment was performed for rapid decompression and definitive pathological diagnosis. The mass was resected as much as possible using the lateral occipital fossa approach, and the operation was completed without dissection of the brain stem or manipulation of the syringobulbia. Postoperatively, neurological symptoms promptly improved, and the syringobulbia reduced. CONCLUSIONS: For intradural ganglion cysts with syringobulbia, we suggest relief of the compression by resection of the mass and treatment of the syringobulbia in 2 stages, if necessary, to avoid the risk of damage to the brainstem.

IVIG in childhood primary angiitis of the central nervous system: A case report.

Aggressive immunosuppressive therapies have been proposed to treat primary angiitis of the central nervous system (PACNS). Here, we report the first successfully stabilized case of childhood, small-vessel PACNS with intravenous immunoglobulin (IVIG) therapy. A 12-year-old boy was admitted to our hospital complaining of recurrent headaches and upper-left homonymous quadrantanopia, since the age of 11 years. Brain computed tomography scans revealed fine calcification in the right temporal and occipital lobes. Brain magnetic resonance imaging scans revealed white matter lesions, with gadolinium enhancement, which waxed, waned, and migrated for 1 year, without immunomodulatory therapies. A cerebrospinal fluid study showed pleocytosis (12 cells per µl). No clinical or serological findings suggested systemic inflammation or vasculitis. Brain angiography was unremarkable. Brain biopsy revealed thickened and hyalinized small vessels, with intramural infiltration of inflammatory cells, which confirmed the diagnosis of small-vessel PACNS. Because the patient developed surgical site infection following biopsy, the administration of monthly IVIG (2 g/kg) was prescribed, instead of immunosuppressive agents. After IVIG therapy, the patient remained stable, except for a single episode of mild radiological exacerbation at 16 months, which occurred when the IVIG interval was expanded. Oral prednisone was added and gradually tapered. At 50 months, his intellectual abilities and motor functions were normal, although he showed residual upper-left homonymous quadrantanopia and post-exercise headache. A temporary headache, associated with the immunoglobulin infusion, was resolved by slowing the infusion rate. PACNS should be treated aggressively to improve prognosis. However, when immunosuppressants are contraindicated, IVIG may be an alternative therapeutic option.

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.