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This study aims to elucidate the clinical and molecular characteristics, treatment outcomes and prognostic factors of patients with histone H3 K27-mutant diffuse midline glioma. We retrospectively analyzed 93 patients with diffuse midline glioma (47 thalamus, 24 brainstem, 12 spinal cord and 10 other midline locations) treated at 24 affiliated hospitals in the Kansai Molecular Diagnosis Network for CNS Tumors. Considering the term "midline" areas, which had been confused in previous reports, we classified four midline locations based on previous reports and anatomical findings. Clinical and molecular characteristics of the study cohort included: age 4-78 years, female sex (41%), lower-grade histology (56%), preoperative Karnofsky performance status (KPS) scores ≥ 80 (49%), resection (36%), adjuvant radiation plus chemotherapy (83%), temozolomide therapy (76%), bevacizumab therapy (42%), HIST1H3B p.K27M mutation (2%), TERT promoter mutation (3%), MGMT promoter methylation (9%), BRAF p.V600E mutation (1%), FGFR1 mutation (14%) and EGFR mutation (3%). Median progression-free and overall survival time was 9.9 ± 1.0 (7.9-11.9, 95% CI) and 16.6 ± 1.4 (13.9-19.3, 95% CI) months, respectively. Female sex, preoperative KPS score ≥ 80, adjuvant radiation + temozolomide and radiation ≥ 50 Gy were associated with favorable prognosis. Female sex and preoperative KPS score ≥ 80 were identified as independent good prognostic factors. This study demonstrated the current state of clinical practice for patients with diffuse midline glioma and molecular analyses of diffuse midline glioma in real-world settings. Further investigation in a larger population would contribute to better understanding of the pathology of diffuse midline glioma., (© 2024. The Author(s).)

Background: Both filum terminale arteriovenous fistulas (FTAVFs) and filum terminale lipomas (FTLs) are rare. Because of this rarity, there is a paucity of data regarding concomitant FTAVF and FTL, and the optimal treatment remains to be defined. The authors describe a patient with coexisting FTAVF and FTL treated with single-stage surgery., Observations: A man in his 70s was referred to the authors' department because of a suspected spinal vascular malformation seen on magnetic resonance imaging that was performed to investigate lower limb weakness, intermittent claudication, and urinary incontinence. Previous imaging had shown a terminal lipoma with an internal flow void. Computed tomography angiography and digital subtraction angiography revealed an FTAVF, the feeder being a lateral sacral artery. The patient was treated surgically with curative intent. The FTL and tethered cord that had been identified by imaging were treated in the same procedure. Postoperative digital subtraction angiography showed absence of the abnormal vessels. The patient was discharged home on the 19th postoperative day., Lessons: When considering treatment, it is important to determine whether symptoms are attributable mainly to FTL, tethered cord, or FTAVF. One-stage treatment is useful because it eliminates both the FTAVF and the factors that led to its development.

Diffuse idiopathic skeletal hyperostosis (DISH) is a condition in which minor trauma can cause extremely unstable vertebral fractures. Spinal fractures associated with DISH are prone to instability due to the large moment of lever arm and secondary neurological deterioration; hence, surgical internal fixation is considered necessary. On the other hand, some reports suggest that patients with DISH have a high osteogenic potential. In this report, we describe three patients with DISH. These patients had spinal injuries that resulted in a large gap, for which anterior fixation with bone graft would generally be considered due to comminuted fractures. However, we achieved good bony fusion with posterior fixation alone, without forcible correction., Competing Interests: Authors have no conflict of interest in this research., (© 2022 The Japan Neurosurgical Society.)

Syringomyelia is often associated with Chiari malformation, trauma, infection, and spinal cord tumor. Although they are relatively rare diseases, arachnoid cysts and its related pathology, "arachnoid web" can sometimes lead to syrinx formation at the thoracic vertebral level. However, syrinx formation caused by degenerative spinal disorders, particularly at the thoracic vertebral levels, has rarely been reported. Herein, we present a case of syringomyelia with thoracic ossified yellow ligament (OYL) in a 79-year-old man, who underwent initial posterior decompression followed by arachnoid web removal. Posterior decompression via laminectomy of thoracic vertebra 2 and removal of the OYL improved the syrinx partially, but dorsal indentation of the spinal cord and a remnant syrinx were observed in post-operative magnetic resonance images, subsequent to the second surgery's successful removal of the arachnoid web, which had dorsally compressed the spinal cord. After the second operation, the syrinx shrunk further, and the patient could walk independently at 5 months after the operations. In our case, both the OYL and arachnoid web were responsible for syrinx formation. Therefore, the coincidence of degenerative vertebral diseases with a syrinx might indicate the coexistence of an underlying lesion. Furthermore, the arachnoid web in this case might have formed due to the denaturation of the arachnoid cyst triggered by the OYL., Competing Interests: Authors have no conflicts of interest in this research., (© 2022 The Japan Neurosurgical Society.)

This study aims to present our surgical technique of lateral lumbar interbody fusion (LLIF) without corpectomy for lumbar vertebral fracture (LVF) associated radiculopathy. This study includes three patients treated with LLIF (mean age of 77.3 years, Group L) and three patients treated with PLIF (mean age of 75.7 years, Group P) to compare the surgical outcomes. The cartilage on the fractured vertebrae was aggressively resected with attention to avoid injury to the ring apophysis. The central cavity of the fractured endplate was filled with a bone graft substitute made of hydroxyapatite and collagen composite, followed by interbody fusion achieved by utilizing of a cage with sufficient length spanning the bilateral edges of the fractured vertebra. PLIF was performed with a standard technique using two interbody cages, and vertebroplasty was combined in one patient. Comparing to PLIF, LLIF could be performed with less estimated blood loss in shorter surgical time. Local kyphotic angle improved in all cases of Group L immediately after the surgery, but correction loss was observed at the final examination. The lordotic angle was lost in Group P postoperatively. Arthrodesis was achieved in all the cases. The mean VAS score for leg pain was 85.3 mm in Group L and 82.0 mm in Group P at preoperation and decreased to 8.7 mm and 11.3 mm, respectively, at postoperation. LLIF is an effective surgical option that enables stabilization of the fractured vertebra and reduces radicular pain by indirect neural decompression.

Objective: This retrospective study aimed to compare vertebral endplate cyst formation (VECF), an early predictor for pseudoarthrosis, in different types of interbody cages., Methods: We reviewed 84 cases treated with single-level posterior/transforaminal lumbar interbody fusion. We utilized a polyetheretherketone cage in 20 cases (group P), a titanium cage in 16 cases (group Ti), a titanium-coating polyetheretherketone cage in 13 cases (group TiP) and a porous tantalum cage in 35 cases (group Tn). VECF was evaluated comparing the computed tomography scans taken at day 0 and 6-month postoperation. We defined VECF (+) as enlargement of a pre-existing cyst or de novo formation of a cyst with the diameter over 2 mm. We calculated the adjusted odds ratio (OR) and 95% confidence intervals (CIs) as an indicator of association between different types of cages and VECF using a logistic regression model., Results: VECF was observed in 13 (65%), 7 (44%), 9 (69%), and 8 (23%) cases in groups P, Ti, TiP and Tn, respectively. VECF correlated with the type of cage (p = 0.04). In comparison with group P, the proportion of VECF (+) cases was lower in group Tn (OR, 0.16; 95% CI, 0.04-0.60) but not different in group Ti (OR, 0.47; 95% CI, 0.10-2.20) and group TiP (OR, 1.06; 95% CI, 0.21-5.28). No patient underwent additional surgery for the fused spinal level during the follow-up periods (average, 37.9 months; range, 6-76 months)., Conclusion: VECF was the least in the porous Tn cage, suggesting its potential superiority for initial stability.

We report a case of post-traumatic syringomyelia (PTS), which developed 2 months after spinal cord injury (SCI). A 20-year-old man who was involved in a motorcycle accident sustained a complete SCI resulting from a burst fracture of the T5 vertebral body. He underwent posterior fixation with decompression at another hospital 2 days after the injury. Postoperative imaging suggested that spinal stenosis endured at the T4 level and swelling of the spinal cord above that level. Two months later, he felt dysesthetic pain in his forearms and hands, but the cause of the pain was not examined in detail. Four months after the injury, he presented with motor weakness in the upper extremities. Magnetic resonance imaging (MRI) showed syringomyelia ascending from the T3 level to the C1 level, and he was referred to our hospital immediately. The imaging studies suggested that PTS was caused by congestion of the cerebrospinal fluid (CSF) at the T3 level. The patient was treated with syringosubarachnoid (SS) shunt at the T1-T2 level, whereby neurological symptoms of the upper extremities were immediately relieved. Postoperative MRI showed shrinkage of the syrinx. At the latest follow-up 2 years postoperatively, there was no sign of recurrence. It is noteworthy that PTS potentially occurs in the early phase after severe SCI. We discuss relevant pathology and surgical treatment through a review of previous literature., Competing Interests: Conflicts of Interest Disclosure None of the authors have any conflicts of interest to disclose., (© 2020 The Japan Neurosurgical Society.)

Atlantoaxial rotatory fixation (AARF) occurs commonly in children who have undergone trauma. It is usually corrected with conservative therapy. In this report, however, the patient was an adult with AARF who was treated surgically. A 64-year-old woman presented with a 1-year history of spastic gait and hand clumsiness. Imaging studies revealed the presence of AARF, os odontoideum, and severe spinal cord compression at that spinal level. As the AARF had not been responded to head traction with a halo device, we decided to treat the patient with in situ posterior fixation. Because the rigid dislocation was present between the atlas and the axis, we were forced to make an unusual instrumentation construct. Neurological symptoms other than hand numbness diminished after the surgery, and arthrodesis was obtained between the occiput and the axis. It should be noted that surgical planning for posterior instrumentation construct is required when rigid AARF is treated surgically., Competing Interests: Conflicts of Interest Disclosure None of the authors has any conflicts of interest to disclosure.

Olfactory stem cells are generated from olfactory mucosa. Various culture conditions generate olfactory stem cells that differ according to species and developmental stage and have different progenitor or stem cell characteristics. Olfactory spheres (OSs) are clusters of progenitor or stem cells generated from olfactory mucosa in suspension culture. In this study, adult human OSs were generated and their characteristics analyzed. Human OSs were adequately produced from olfactory mucosa with area over 40 mm(2). Immunocytochemistry (ICC) and fluorescence-activated cell sorting showed that human OSs were AN2 and A2B5-positive. Immunofluorescence analysis of cell type-specific ICC indicated that the number of Tuj1-positive OS cells was significantly elevated. Tuj1-positive cells displayed typical neuronal soma and dendritic morphology. Human OS cells were also immunopositive for MAP2. By contrast, few RIP-, O4-, and GFAP-positive cells were present. These RIP, O4, and GFAP-positive cells did not resemble bona fide oligodendrocytes and astrocytes morphologically. In culture to induce differentiation of oligodendrocytes, human OS cells also expressed neuronal markers, but neither oligodendrocyte or astrocyte markers. These findings suggest that human OS cells autonomously differentiate into neurons in our culture condition and have potential to be used as a cell source of neural progenitors for their own regenerative grafts, avoiding the need for immunosuppression and ethical controversies., (Copyright © 2015. Published by Elsevier B.V.)

The olfactory epithelial layer contains multipotent horizontal basal cells (HBCs) that differentiate into olfactory sensory neurons. Here, we show that rat HBCs express oligodendrocyte progenitor cell (OPC) and astrocyte markers. We generated olfactory sphere (OS) cells in cultures that were derived from adult rat olfactory mucosa. Fluorescence-activated cell sorting and immunofluorescence analyses showed that OS cells also express OPC and astrocyte markers. Interestingly, OS cells underwent oligodendrocyte differentiation in vitro. To study oligodendrocyte differentiation in vivo, OS cells were transplanted into injured rat spinal cords. The transplanted cells integrated into host tissue and differentiated into oligodendrocytes. When transected saphenous nerve ends were encased in collagen-containing silicone tubes with or without OS cells, the transplanted OS cells differentiated into Schwann cells. Our data provide new insights into of the stemness of OS cells., (© 2013.)

Intradural extramedullary spinal ependymomas are extremely rare. Herein, we describe a lesion-type spinal ependymoma that followed a malignant course, and discuss its clinical presentation, etiopathogenesis, and treatment. We present a patient who was diagnosed with an intradural extramedullary spinal tumor at T4-T6. The patient underwent gross total resection of the tumor without damage to the spinal cord. Histological examination, classified the lesion as a World Health Organization (WHO)-grade 2 ependymoma. One and a half years later, magnetic resonance imaging detected a recurring tumor at T4-T5. The tumor was removed and classified as a WHO-grade 3 anaplastic ependymoma. The patient was started on a course of regional spinal cord radiotherapy. The patient achieved tumoral control and clinical stabilization after the recurrence. We must consider the differential diagnosis of intradural extramedullary spinal tumors. The best treatment for this lesion is gross total resection and adjunctive radiotherapy is necessary in cases of malignant-change.

Bow hunter's stroke is typically due to mechanical compression or stretching of the dominant vertebral artery (VA) during contralateral head rotation against the bony elements of the atlas and axis. We report a case of vertebrobasilar insufficiency due to bilateral vertebral artery occlusion at the left C3-4 and the right C1-2 junction on rightward head rotation. A 64-year-old man experienced ischemic symptoms during 90° head rotation to the right with complete resolution of symptoms after returning his head to the neutral position. Dynamic cervical angiography with rightward head rotation showed severe compression of the right VA at the transverse foramen of C3-4 and mechanical stenosis of the left VA at the C1-2 level. During head rotation, the flow of the right VA was decreased more than the left side. Cervical 3-D computed tomography (CT) on rightward head rotation demonstrated displacement of the uncovertebral C3-4 joint, with excessive rotation of the C3 vertebral body. Based on these findings, instability at C3-4 was suspected to be the main cause of the vertebrobasilar insufficiency. Anterior discectomy and fusion at the C3/4 level were performed. Postoperatively, the patient experienced complete resolution of symptoms, and dynamic cervical angiography showed disappearance of the compression of the right VA. To our knowledge, this is the first reported case of bow hunter's stroke diagnosed by dynamic cerebral angiography and cervical 3-D CT without angiography, and treated by anterior decompression and fusion without decompression of the VA.