Shorter survival time of adolescents and young adult patients than older adults with spinal cord glioblastoma: a multicenter study.

OBJECTIVE: Cancers in adolescents and young adults (AYAs) (age 15-39 years) often present with unique characteristics and poor outcomes. To date, spinal cord glioblastoma, a rare tumor, remains poorly understood across all age groups, including AYAs. This comparative study aimed to investigate the clinical characteristics and outcomes of spinal cord glioblastoma in AYAs and older adults (age 40-74 years), given the limited availability of studies focusing on AYAs. METHODS: Data from the Neurospinal Society of Japan's retrospective intramedullary tumor registry (2009-2020) were analyzed. Patients were dichotomized on the basis of age into AYAs and older adults. Univariate and multivariate Cox proportional hazards regression models were utilized to explore risk factors for overall survival (OS). RESULTS: A total of 32 patients were included in the study, with a median (range) age of 43 (15-74) years. Of these, 14 (43.8%) were AYAs and 18 (56.2%) were older adults. The median OS was 11.0 months in AYAs and 32.0 months in older adults, and the 1-year OS rates were 42.9% and 66.7%, respectively, with AYAs having a significantly worse prognosis (p = 0.017). AYAs had worse preoperative Karnofsky Performance Status (KPS) than older patients (p = 0.037). Furthermore, AYAs had larger intramedullary tumors on admission (p = 0.027) and a significantly higher frequency of intracranial dissemination during the clinical course (p = 0.048). However, there were no significant differences in the degrees of surgical removal or postoperative radiochemotherapy between groups. The Cox proportional hazards regression model showed that AYAs (HR 3.53, 95% CI 1.17-10.64), intracranial dissemination (HR 4.30, 95% CI 1.29-14.36), and no radiation therapy (HR 57.34, 95% CI 6.73-488.39) were risk factors for mortality for patients of all ages. Worse preoperative KPS did not predict mortality in AYAs but did in older adults. The high incidence of intracranial dissemination may play an important role in the poor prognosis of AYAs, but further studies are needed. CONCLUSIONS: The clinical characteristics of AYAs with spinal cord glioblastoma differ from those of older adults. The prognosis of AYAs was clearly worse than that of older adults. The devastating clinical course of spinal glioblastoma in AYAs was in line with those of other cancers in this age group.

Epidemiology and Comparative Analysis of Outcomes of Intramedullary Spinal Cord Tumor Between Pediatric and Adult Patients.

STUDY DESIGN: This was as clinical retrospective study. OBJECTIVES: We sought to evaluate the characteristics of Pediatric intramedullary spinal cord tumors (PISCTs) and to identify differences between pediatric and adult intramedullary spinal cord tumors. SUMMARY OF BACKGROUND DATA: PISCTs represent a rare clinical entity with limited evidence-base in the literature. METHODS: This study is a subanalysis of the retrospective multicenter observational study authorized by the Neurospinal Society of Japan, including consecutive patients with spinal intramedullary tumors treated surgically at 58 institutions between 2009 and 2020. Data on 1080 intramedullary spinal cord tumors were obtained, consisting of 91 pediatric and 939 adult patients. Survival was compared using Cox hazard regression while clinical differences were evaluated using multivariable logistic regression that controlled for confounders. RESULTS: Pediatric patients had a shorter overall, and progression-free, survival than adults. Pediatric patients with ISCTs were likely to have scoliosis [odds ratio (OR) = 6.49, 95% CI: 2.26-18.7], short preoperative symptom duration (OR = 0.99, 95% CI: 0.98-0.99), lower incidence of paresthesia (OR = 0.41, 95% CI: 0.22-0.77), higher incidence of paresis (OR = 2.10, 95% CI: 1.01-4.35), histopathology of astrocytoma (OR = 2.97, 95% CI: 1.19-7.43), and postoperative functional deterioration upon discharge (OR = 2.83, 95% CI: 1.43-5.58). Age was not a statistically significant prognostic factor of overall survival among the pediatric cohort. CONCLUSION: We found that the clinical characteristics of ISCTs differed between pediatric and adult patients. In terms of histopathological types, astrocytoma was most common in pediatric patients. ISCT occurring at an early age may not be an indicator for poor prognosis.

Intravenous Administration of Human Muse Cells Ameliorates Deficits in a Rat Model of Subacute Spinal Cord Injury.

Multilineage-differentiating stress-enduring (Muse) cells are newly established pluripotent stem cells. The aim of the present study was to examine the potential of the systemic administration of Muse cells as an effective treatment for subacute SCI. We intravenously administered the clinical product "CL2020" containing Muse cells to a rat model two weeks after mid-thoracic spinal cord contusion. Eight experimental animals received CL2020, and twelve received the vehicle. Behavioral analyses were conducted over 20 weeks. Histological evaluations were performed. After 20 weeks of observation, diphtheria toxin was administered to three CL2020-treated animals to selectively ablate human cell functions. Hindlimb motor functions significantly improved from 6 to 20 weeks after the administration of CL2020. The cystic cavity was smaller in the CL2020 group. Furthermore, larger numbers of descending 5-HT fibers were preserved in the distal spinal cord. Muse cells in CL2020 were considered to have differentiated into neuronal and neural cells in the injured spinal cord. Neuronal and neural cells were identified in the gray and white matter, respectively. Importantly, these effects were reversed by the selective ablation of human cells by diphtheria toxin. Intravenously administered Muse cells facilitated the therapeutic potential of CL2020 for severe subacute spinal cord injury.

The Impact of Adjuvant Radiotherapy on Clinical Performance Status in Patients With Grade II Spinal Cord Astrocytoma - A Nationwide Analysis by the Neurospinal Society of Japan.

OBJECTIVE: The impact of adjuvant radiotherapy on overall survival (OS) and progression-free survival (PFS) of patients with grade II spinal cord astrocytomas remains controversial. Additionally, the relationship between progression and clinical deterioration after radiotherapy has not been well investigated. METHODS: This study included 53 patients with grade II intramedullary spinal cord astrocytomas treated by either subtotal, partial resection or open biopsy. Their clinical performance status was assessed immediately before operation and 1, 6, 12, 24, and 60 months after surgery by Karnofsky Performance Scale (KPS). Patients with and without adjuvant radiotherapy were compared. RESULTS: The groups with and without radiation comprised 23 and 30 patients with a mean age of 50.3 ± 22.6 years (range, 2-88 years). The mean overall disease progression rate was 47.1% during a mean follow-up period of 48.4 ± 39.8 months (range, 2.5-144.5 months). In the radiation group, 11 patients (47.8%) presented with progressive disease, whereas 14 patients (46.7%) presented with progressive disease in the group without radiation. There were no significant differences in OS or PFS among patients with or without adjuvant radiotherapy. KPS in both groups, especially radiation group, gradually decreased after operation and deteriorated before the confirmation of disease progression. CONCLUSION: Adjuvant radiotherapy did not show effectiveness regarding PFS or OS in patients with grade II spinal cord astrocytoma according to classical classification based on pathohistological findings.

Clinical Characteristics and Long-term Outcomes of Spinal Pilocytic Astrocytomas: A Multicenter Retrospective Study by the Neurospinal Society of Japan.

OBJECTIVE: The characteristics, imaging features, long-term surgical outcomes, and recurrence rates of primary spinal pilocytic astrocytomas (PAs) have not been clarified owing to their rarity and limited reports. Thus, this study aimed to analyze the clinical presentation, radiological features, pathological findings, and long-term outcomes of spinal PAs. METHODS: Eighteen patients with spinal PAs who were surgically treated between 2009 and 2020 at 58 institutions were included in this retrospective multicenter study. Patient data, including demographics, radiographic features, treatment modalities, and long-term outcomes, were evaluated. RESULTS: Among the 18 consecutive patients identified, 11 were women and 7 were men; the mean age at presentation was 31 years (3-73 years). Most PAs were located eccentrically, were solid or heterogeneous in appearance (cystic and solid), and had unclear margins. Gross total resection (GTR), subtotal resection (STR), partial resection (PR), and biopsy were performed in 28%, 33%, 33%, and 5% of cases, respectively. During a follow-up period of 65 ± 49 months, 4 patients developed a recurrence; however, the recurrence-free survival did not differ significantly between the GTR and non-GTR (STR, PR, and biopsy) groups. CONCLUSION: Primary spinal PAs are rare and present as eccentric and intermixed cystic and solid intramedullary cervical tumors. The imaging features of spinal PAs are nonspecific, and a definitive diagnosis requires pathological support. Surgical resection with prevention of neurological deterioration can serve as the first-line treatment; however, the resection rate does not affect recurrence-free survival. Investigation of relevant molecular biomarkers is required to elucidate the regrowth risk and prognostic factors.

Impact of surgical treatment for intramedullary spinal cord metastasis on neurological function and survival: A multicenter retrospective study by the Neurospinal Society of Japan.

BACKGROUND: This retrospective multicenter study aimed to analyze the characteristics and surgical outcomes of intramedullary spinal cord metastasis (ISCM) and to discuss the controversy regarding its surgical indications. METHODS: This study included 29 ISCM patients who underwent surgery between 2009 and 2020. Biopsy cases were excluded from analysis. For functional and neurological functional assessments, Karnofsky Performance Status (KPS, %) and modified McCormick Scale (MMS, 5-grade scale) scores were determined before and after surgery. Patients were divided into two groups: a mild-to-moderate disability group with preoperative MMS grades 1 to 3, and a severe disability group with preoperative MMS grades 4 to 5. RESULTS: The mean preoperative KPS was 45.9, and the mean duration from symptom onset to surgical intervention was 1 month. The ISCM was located in the cervical spine in 10 cases and the thoracic spine in 19 cases. The access route (myelotomy) for ISCM removal was via the posterior median sulcus in 9 cases and via the posterior lateral sulcus in 11 cases, while others were not recorded. The degree of removal was gross total resection in 20 patients (69%), subtotal resection in 0 patients, and partial removal in 9 patients (31%). No significant complications related to the surgical procedures were recorded. Postoperative adjuvant therapy included radiotherapy in 17 patients (58.6%) and chemotherapy or molecular targeted therapy in 13 patients (44.8%). Pathological findings of ISCM showed that colorectal cancer was the most common in 9 cases, followed by lung cancer in 7, renal cell carcinoma in 5, and breast cancer in 2. Twenty-one of the 29 patients (72.4%) were confirmed to have survived 6 months after surgery: 8 of the 10 patients (80%) in the mild-to-moderate disability group and 13 of the 19 patients (68.4%) in the severe disability group. At 6 months after surgery, 3 of the 8 patients (38%) in the mild-to-moderate group were able to maintain or improve their function. Eleven of the 13 patients (85%) in the severe disability group maintained their function despite being severely disabled. CONCLUSIONS: This study suggests that surgical treatment can maintain or improve neurological function in a limited number of patients with ISCM, although it had minimal impact on improving the survival rate after surgery.

Acceptance of Early Surgery for Treatment of Spinal Cord Cavernous Malformation in Contemporary Japan.

OBJECTIVE: Spinal cord cavernous malformation (CM) is an intramedullary vascular lesion that may present with progressive symptoms. Surgery is recommended for symptomatic patients, but optimal timing of surgery is debatable. Some advocate waiting until plateau of neurological recovery and others support emergency surgery. There is no statistic on how commonly these strategies are utilized. We aimed to find contemporary practice pattern among neurosurgical spine centers in Japan. METHODS: A database of intramedullary spinal cord tumors assembled by Neurospinal Society of Japan was surveyed and 160 patients with spinal cord CM were identified. Neurological function, disease duration, and number of days between presentation to hospitals and surgery were analyzed. RESULTS: Duration of disease before presentation to hospitals ranged from 0 to 336 months (median, 4 months). Number of days between patients' presentation and surgery ranged from 0 to 6,011 days (median, 32 days). Time from symptom onset to surgery ranged from 0 to 336.9 months (median, 6.6 months). Patients with severe preoperative neurological dysfunction had shorter duration of disease, fewer days between presentation and surgery, and shorter time between symptom onset and surgery. Patients with paraplegia or quadriplegia were more likely to improve when operated on within 3 months from onset. CONCLUSION: Timing of surgery for spinal cord CM in Japanese neurosurgical spine centers generally was early, with 50% of patients undergoing surgery within 32 days after presentation. Further study is needed to clarify optimal timing of surgery.

Outcomes of Intramedullary Spinal Cord Tumor Surgery in Older Versus Younger Adults: A Multicenter Subanalysis Study by the Neurospinal Society of Japan.

OBJECTIVE: Intramedullary spinal cord tumors (IMSCTs) are uncommon and difficult to treat. Studies examining the efficacy of rare IMSCT surgery in the elderly are limited. We conducted a subanalysis using multicenter retrospective-historical data provided by the Japan Neurospinal Society to compare surgical outcomes between older and younger adults with IMSCTs. METHODS: We classified patients with IMSCTs into younger (aged 18-64 years) or older ( ≥ 65 years) groups. The primary outcomes of "improved" or "worsened" from the preoperative period to 6 months after surgery were evaluated using the modified McCormick scale (mMCs). A favorable outcome was defined as an mMCs grade of I/II at 6 months. RESULTS: Among 841 patients registered, there were 658 younger (78.2%) and 183 older patients (21.8%) evaluated using mMCs at 6 months. Median preoperative mMCs grades were significantly worse in older patients than in younger patients. Neither the "improved" nor "worsened" rate differed significantly between the groups (28.1% vs. 25.1%; crude odds ratio [cOR], 0.86; 95% confidence interval [CI], 0.59-1.25; adjusted OR [aOR], 0.84; 95% CI, 0.55-1.28; 16.9% vs. 23.0%; cOR, 1.47; 95% CI, 0.98-2.20; aOR, 1.28; 95% CI, 0.83-1.97). Favorable outcomes were significantly less common among older adults in the univariate analysis but were not significant in the multivariate analysis (66.4% vs. 53.0%; cOR, 0.57; 95% CI, 0.41-0.80; aOR, 0.77; 95% CI, 0.50-1.19). In both younger and older patients, preoperative mMCs accurately predicted favorable outcomes. CONCLUSION: Age alone is not a sufficient reason to prohibit surgery for IMSCTs.

Usefulness of Transcranial Motor Evoked Potential in Clipping Surgery for Cerebral Aneurysms-Introduction of a New Protocol for Stable Monitoring.

The usefulness of transcranial motor evoked potentials (Tc-MEPs) in clipping surgery has been reported. However, numerous false positive and false negative cases were reported. We report the usefulness of a new protocol compared with direct cortical MEP (Dc-MEP).Materials were 351 patients who underwent aneurysmal clipping under simultaneous monitoring of Tc- and Dc-MEPs. A total of 337 patients without hemiparesis and 14 with hemiparesis were separately analyzed. Intraoperative changes of Tc-MEP thresholds were examined in the first 50 patients without hemiparesis. The stimulation strength of Tc-MEP was set at +20% of the stimulation threshold. As thresholds changed intraoperatively, thresholds were examined every 10 min and changed stimulation strength.Stimulation thresholds of Tc-MEP were significantly decreased after craniotomy and significantly increased after CSF aspiration. The recording ratios of Tc- and Dc-MEPs were 98.8% and 90.5%, respectively. Out of 304 patients without MEP change, 5 patients developed transient or mild hemiparesis with infarction of the territory of the perforating artery arising from the posterior communicating artery. Out of 31 patients whose MEP transiently disappeared, 3 patients developed transient or mild hemiparesis. The other two patients without MEP recovery manifested persistent hemiparesis. In 14 patients with preoperative hemiparesis, 3 patients whose healthy/affected ratio of Tc-MEP was large developed severe persistent hemiparesis.We clarified the intraoperative changes of Tc-MEP thresholds for the first time. A new protocol of Tc-MEP that followed thresholds and changed stimulation strength to +20% of thresholds is useful for stable monitoring. The usefulness of Tc-MEP is the same as that or better than that of Dc-MEP.

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.

Comparison of the Recurrence and Surgical Outcome of Spinal Hemangioblastoma in Sporadic and Von Hippel-Lindau Diseases: A Subanalysis of a Nationwide Study by the Neurospinal Society of Japan.

OBJECTIVE: This study aimed to clarify the relationship between recurrence and the extent of resection in surgery for intramedullary spinal hemangioblastoma (sHB) and its impact on von Hippel-Lindau (vHL) disease. METHODS: Data on sHB cases followed up for at least 6 months after surgery were extracted from a nationwide registry of 1,033 consecutive spinal intramedullary tumors surgically treated between 2009 and 2020, and were retrospectively categorized into a sporadic or vHL group. The diagnosis of vHL disease was made at each institution based on clinical findings. RESULTS: A total of 168 patients (sporadic group, 101; vHL group, 67) were included in the study. Compared with the sporadic group, the vHL group had a younger onset (45.4 ± 16.8 years vs. 39.6 ± 14.1 years, p = 0.02), more preoperative motor (47.5% vs. 68.7%, p < 0.01) and gait (37.6% vs. 61.2%, p < 0.01) impairments, and more patients with worsening neurological symptoms at discharge (p = 0.02). The gross total resection (GTR) rates and the recurrence rates were not statistically different between the sporadic and the vHL groups. GTR significantly improved recurrence-free survival compared to non-GTR in all patient analysis (p < 0.01) but this trend was not observed in the sporadic group. Physical functional improvement from discharge to 6 months after surgery was observed in the sporadic group (p < 0.01) but not in the vHL group. CONCLUSION: A high GTR rate may sufficiently decrease susceptibility to recurrence, especially in patients with sHB with vHL. In sporadic sHB, postoperative functional improvement can be expected, and the long-term functional prognosis is favorable.

Intramedullary Schwannoma of the Spinal Cord: A Nationwide Analysis by the Neurospinal Society of Japan.

OBJECTIVE: This study was aimed to report the clinical characteristics of intramedullary schwannomas and discuss imaging findings and treatment strategies. METHODS: The inclusion criterion was consecutive patients with intramedullary schwannomas who were surgically treated at 8 centers between 2009 and 2020. Clinical characteristics included age, sex, clinical presentation, disease duration, and follow-up period. The modified McCormick scale was used to compare the preoperative and postoperative conditions. Pre- and postoperative magnetic resonance images (MRI) of each case were analyzed. RESULTS: The mean age of the total 11 patients at the operation was 50.2 years. The mean duration of the symptoms was 23 months, with limb paresthesia being the most common clinical presentation. The cervical spine was the most common localization level of the tumor in 6 cases. The mean follow-up duration was 49.4 months. Gross total resection (GTR) and subtotal resection (STR) was achieved in 9 and 2 cases, respectively. According to the modified McCormick scale at 6 months postoperatively, 7 cases (63.6%) had improved and 4 cases (36.3%) had unchanged grades. Typical MRI findings of the intramedullary schwannoma included ring-like enhancement, syringomyelia, cystic formation, intramedullary edema, and hemosiderin deposition. Gadolinium enhancement was homogenous in 8 cases (72.7%). The tumor margins were well demarcated in all cases. CONCLUSION: Intramedullary schwannoma should be considered when sharp margins and well-enhanced tumors are present at the cervical spine level and the initial symptoms are relatively mild, such as dysesthesia. When GTR cannot be achieved, STR for tumor decompression is recommended.

Erratum to "Carotid computed tomography angiography after cobalt-based alloy carotid artery stenting using ultra-high-resolution computed tomography with model-based iterative reconstruction" [Radiol Case Rep 2021;16:3721-8].

[This corrects the article DOI: 10.1016/j.radcr.2021.09.003.].

Predictors of Progression-Free Survival in Patients With Spinal Intramedullary Ependymoma: A Multicenter Retrospective Study by the Neurospinal Society of Japan.

BACKGROUND AND OBJECTIVES: Ependymoma is the most common spinal intramedullary tumor. Although clinical outcomes have been described in the literature, most of the reports were based on limited numbers of cases or been confined to institutional experience. The objective of this study was to analyze more detailed characteristics of spinal intramedullary ependymoma (SIE) and provide clinical factors associated with progression-free survival (PFS). METHODS: This retrospective observational multicenter study included consecutive patients with SIE in the cervical or thoracic spine treated surgically at a total of 58 institutions between 2009 and 2020. The results of pathological diagnosis at each institute were confirmed, and patients with myxopapillary ependymoma, subependymoma, or unverified histopathology were strictly excluded from this study. Outcome measures included surgical data, surgery-related complications, postoperative systemic adverse events, postoperative adjuvant treatment, postoperative functional condition, and presence of recurrence. RESULTS: This study included 324 cases of World Health Organization grade II (96.4%) and 12 cases of World Health Organization grade III (3.6%). Gross total resection (GTR) was achieved in 76.5% of cases. Radiation therapy (RT) was applied after surgery in 16 cases (4.8%), all of which received local RT and 5 of which underwent chemotherapy in combination. Functional outcomes were significantly affected by preoperative neurological symptoms, tumor location, extent of tumor resection, and recurrence. Multivariate regression analysis suggested that limited extent of tumor resection or recurrence resulted in poor functional outcomes. Multiple comparisons among the groups undergoing GTR, subtotal resection and biopsy, or partial resection of the tumor showed that the probability of PFS differed significantly between GTR and other extents of resection. CONCLUSION: When GTR can be safely obtained in the surgery for SIE, functional maintenance and longer PFS can be expected.

[Settings and Decisions of Monitoring].

Since the beginning of the 21st century, as intraoperative monitoring has been steadily spreading in Japan and globally, the values of motor-evoked potentials, visual-evoked potentials, and cortical-evoked potentials have been described. There are a wide variety of monitoring methods; the diseases handled are not limited to brain lesions, but extend also to spinal cord and spinal lesions; and there are many problems that have not yet been solved. Possible precautions are indicated by means of a video of an actual case site. Considerations are presented regarding the setting of this monitoring method, utilized in relatively frequent diseases and associated intraoperative judgments.

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.

Principal component analysis of texture features for grading of meningioma: not effective from the peritumoral area but effective from the tumor area.

PURPOSE: To investigate whether texture features from tumor and peritumoral areas based on sequence combinations can differentiate between low- and non-low-grade meningiomas. METHODS: Consecutive patients diagnosed with meningioma by surgery (77 low-grade and 28 non-low-grade meningiomas) underwent preoperative magnetic resonance imaging including T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), diffusion-weighted imaging (DWI), and contrast-enhanced T1WI (CE-T1WI). Manual segmentation of the tumor area was performed to extract texture features. Segmentation of the peritumoral area was performed for peritumoral high-signal intensity (PHSI) on T2WI. Principal component analysis was performed to fuse the texture features to principal components (PCs), and PCs of each sequence of the tumor and peritumoral areas were compared between low- and non-low-grade meningiomas. Only PCs with statistical significance were used for the model construction using a support vector machine algorithm. k-fold cross-validation with receiver operating characteristic curve analysis was used to evaluate diagnostic performance. RESULTS: Two, one, and three PCs of T1WI, apparent diffusion coefficient (ADC), and CE-T1WI, respectively, for the tumor area, were significantly different between low- and non-low-grade meningiomas, while PCs of T2WI for the tumor area and PCs for the peritumoral area were not. No significant differences were observed in PHSI. Among models of sequence combination, the model with PCs of ADC and CE-T1WI for the tumor area showed the highest area under the curve (0.84). CONCLUSION: The model with PCs of ADC and CE-T1WI for the tumor area showed the highest diagnostic performance for differentiating between low- and non-low-grade meningiomas. Neither PHSI nor PCs in the peritumoral area showed added value.

Piezo Actuator-Driven Pulsed Water Jet for Neurosurgery: Laboratory Evaluation with the Swine Model and Implications of Mechanical Properties.

Object Pulsed water jet is an emerging surgical instrumentation intended to achieve both maximal lesion resection and functional maintenance through preservation of fine vessels and minimal damage to the surrounding tissue. The piezo actuator-driven pulsed water jet (ADPJ) is a new technology that can deliver a precisely controlled uniform and efficient pulsed water jet with minimum water flow. The present study evaluated the ADPJ system in preclinical animal studies in the swine brain, and investigated breaking strength, one of the parameters for mechanical properties, to elucidate the mechanism of tissue selectivity for tissue dissection by the water jet. Methods This system consisted of a pump chamber driven by a piezo actuator, a stainless steel tube, and a nozzle (internal diameter: 0.15 mm). The water was supplied at 6 ml/min. The relationship between input voltage (3-25 V at 400 Hz) and peak pressure was measured using a pressure sensor through a sensing hole. Temporal profile of dissection depth during moving application was evaluated using gelatin brain phantom and swine brain. The dissected specimens were evaluated histologically. The mechanical property (breaking strength) of swine brain was measured by a compact table-top universal tester. Results Peak pressure increased linearly with increase in the input voltage, which reflected dissection depth in both the gelatin brain phantom and swine brain. Small arteries were preserved, and minimum damage to surrounding tissues occurred. The breaking strength of arachnoid membrane (0.12 ± 0.014 MPa) was significantly higher compared to gray matter (0.030 ± 0.010 MPa) and white matter (0.056 ± 0.009 MPa) (p < 0.05). The breaking strength of gray matter corresponded to that of 3 wt% gelatin, and that of white matter corresponded to a value between those of 3.5 and 4 wt% gelatin, and the dissection depth seemed to be estimated by 3-4 wt% gelatin. Conclusion The present study suggests that the ADPJ system has the potential to achieve accurate tissue dissection with preservation of blood vessels in neurosurgery. The difference in breaking strength may explain the tissue selectivity between brain parenchyma and tissue protected by the arachnoid membrane.