Transarterial embolization with n-butyl-2-cyanoacrylate for a ruptured aneurysm at the artery of Davidoff and Schechter.

The artery of Davidoff and Schechter (ADS) is mostly identified in pathological conditions such as dural arteriovenous fistulas and brain tumors. Herein, we report a rare case of a ruptured aneurysm of the ADS, which was one of the feeders of a falcotentorial dural arteriovenous fistula. We performed endovascular embolization of the aneurysm and parent artery using n-butyl-2-cyanoacrylate. Complete occlusion of the fistula was achieved by another feeder after the acute phase. To our best knowledge, only a few reports on embolization of ruptured ADS aneurysms exist. Furthermore, this is the first report on the embolization of a ruptured ADS aneurysm using n-butyl-2-cyanoacrylate. This case highlights that endovascular n-butyl-2-cyanoacrylate embolization could be a useful treatment for a ruptured ADS aneurysm.

Surgically treated intracranial arteriovenous fistulas with hemorrhage, resulting in complete obliteration: illustrative cases.

BACKGROUND: Intracranial arteriovenous fistula (AVF) is a rare disease, defined as anastomoses between cerebral or meningeal arteries and dural venous sinuses or cortical veins. With the development of new agents and devices, endovascular embolization has been considered safe and effective in a majority of cases. However, cases that require direct surgery do exist. Herein, the authors present 3 cases of intracranial AVFs that presented with hemorrhage and were treated with direct surgery, achieving complete obliteration and favorable outcomes. OBSERVATIONS: Intracranial AVFs that present with hemorrhage require immediate and complete obliteration. When AVFs involve the dural sinus, transvenous embolization is usually the first choice of treatment. AVFs with single cortical venous drainage are best treated with interruption of the draining vein close to the fistula. Transarterial embolization can be a curative treatment if there are no branches supplying cranial nerves or an association with pial feeders. In cases in which endovascular treatment is technically challenging or has resulted in incomplete occlusion, surgical treatment is indicated. LESSONS: Despite the recent rise in endovascular treatment, it is important to recognize situations in which such treatment is not suitable for intracranial AVFs. Direct surgery is effective in such cases to offer the best possible outcome.

Recurrent glioblastoma metastatic to the lumbar vertebra: A case report and literature review: Surgical oncology.

Background: Glioblastoma is a malignant tumor, and its prognosis is as poor as 1.5 to 2 years. Most cases recur within one year even under the standard treatment. The majority of recurrences are local, and in rare cases, metastasize mostly within the centra nervous system. Extradural metastasis of glioma is exceedingly rare. Here, we present a case of vertebral metastasis of glioblastoma. Case presentation: We present a 21-year-old man post total resection of the right parietal glioblastoma, diagnosed with lumbar metastasis. He originally presented with impaired consciousness and left hemiplegia and underwent gross total resection of the tumor. Given the diagnosis of glioblastoma, he was treated with radiotherapy combined with concurrent and adjuvant temozolomide. Six months after tumor resection, the patient presented with severe back pain, and was diagnosed as metastatic glioblastoma on the first lumbar vertebrae. Posterior decompression with fixation and postoperative radiotherapy were conducted. He went on to receive temozolomide and bevacizumab. However, at 3 months after the diagnosis of lumbar metastasis, further disease progression was noted, and his care was transitioned to best supportive care. Comparison on copy number status between primary and metastatic lesions on methylation array analysis revealed more enhanced chromosomal instability including 7p loss, 7q gain and 8 gain in the metastatic lesion. Conclusion: Based upon the literature review and our case, younger age of initial presentation, multiple surgical interventions, and long overall survival seem to be the risk factors of vertebral metastasis. As the prognosis of glioblastoma improves over time, its vertebral metastasis is seemingly more common. Therefore, extradural metastasis should be kept in mind in the treatment of glioblastoma. Further, detailed genomic analysis on multiple paired specimens is mandated to elucidate the molecular mechanisms of vertebral metastasis.

Solitary fibrous tumor with aneurysmal bone cyst-like change in the middle cranial fossa: illustrative case.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor known for its propensity for recurrence and metastasis. Furthermore, aneurysmal bone cyst (ABC) is a benign osteolytic lesion. ABC-like areas can be seen in bone tumors that have undergone hemorrhagic cystic change. They are formed by disruptions in the osseous circulation caused by the associated lesion. The most common associated lesions are giant cell tumor, chondroblastoma, osteoblastoma, osteosarcoma, chondromyxoid fibroma, and fibrous dysplasia. There has been no reported case of SFT being the associated lesion. OBSERVATIONS: A 42-year-old woman presented with a 6-month history of headache and impaired memory. Radiological examinations revealed a 50-mm cystic lesion with multiple fluid levels arising from the left temporal bone. Total resection of the tumor was conducted, and postoperative course was uneventful. Histopathological examination was consistent with SFT with ABC-like change. LESSONS: This is the first documented case of SFT with ABC-like change in the cranial fossa. This should be considered a differential diagnosis when treating a lesion in the cranial fossa, such as in this case, to achieve complete resection of the tumor and have close follow-up postoperatively.

Incremental changes in interhemispheric functional connectivity after two-stage corpus callosotomy in a patient with subcortical band heterotopia.

Corpus callosotomy (CC) has been reported to be effective in reducing generalized seizures in patients with drug-resistant epilepsies. However, efficacy is measured only by seizure frequency, without any electrophysiological guidance. Herein, we conducted a quantitative analysis of interhemispheric functional connectivity using inter-electrode coherence of scalp electroencephalogram (EEG) in a clinical case of subcortical band heterotopia to evaluate its relationship with seizure frequency. In our case, seizure frequency decreased significantly after posterior CC but not after anterior CC. Inter-electrode coherence also decreased after posterior CC, suggesting it correlated with seizure frequency. This case study supports the use of inter-electrode coherence as an electrophysiological tool that is useful as predictive factor in evaluating the effectiveness of CC.

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.

Pediatric nodular fasciitis at the roof of the optic canal causing decreased vision: case report and review of the literature.

We present a 3-year-old girl with decreased visual acuity of the left eye. Radiological studies revealed a mass lesion at the roof of the left optic canal with bony erosion, which compressed the left optic nerve. Gross total resection of the mass and decompression of the optic canal were performed. Histopathological study was consistent with nodular fasciitis. This is the first report of nodular fasciitis at the roof of the optic canal in a young child. Nodular fasciitis compressing the optic nerve should be included in differential diagnoses of optic neuropathy in young children.

Critical Role of SREBP-1c Large-VLDL Pathway in Environment-Induced Hypertriglyceridemia of Apo AV Deficiency.

Objective- APOA5 variants are strongly associated with hypertriglyceridemia, as well as increased risks of cardiovascular disease and acute pancreatitis. Hypertriglyceridemia in apo AV dysfunction often aggravates by environmental factors such as high-carbohydrate diets or aging. To date, the molecular mechanisms by which these environmental factors induce hypertriglyceridemia are poorly defined, leaving the high-risk hypertriglyceridemia condition undertreated. Previously, we reported that LXR (liver X receptor)-SREBP (sterol regulatory element-binding protein)-1c pathway regulates large-VLDL (very low-density lipoprotein) production induced by LXR agonist. However, the pathophysiological relevance of the finding remains unknown. Approach and Results- Here, we reconstitute the environment-induced hypertriglyceridemia phenotype of human APOA5 deficiency in Apoa5-/- mice and delineate the role of SREBP-1c in vivo by generating Apoa5-/- ;Srebp-1c-/- mice. The Apoa5-/- mice, which showed moderate hypertriglyceridemia on a chow diet, developed severe hypertriglyceridemia on high-carbohydrate feeding or aging as seen in patients with human apo AV deficiency. These responses were nearly completely abolished in the Apoa5-/- ;Srebp-1c-/- mice. Further mechanistic studies revealed that in response to these environmental factors, SREBP-1c was activated to increase triglyceride synthesis and to permit the incorporation of triglyceride into abnormally large-VLDL particles, which require apo AV for efficient clearance. Conclusions- Severe hypertriglyceridemia develops only when genetic factors (apo AV deficiency) and environmental effects (SREBP-1c activation) coexist. We demonstrate that the regulated production of large-sized VLDL particles via SREBP-1c determines plasma triglyceride levels in apo AV deficiency. Our findings explain the long-standing enigma of the late-onset hypertriglyceridemia phenotype of apo AV deficiency and suggest a new approach to treat hypertriglyceridemia by targeting genes that mediate environmental effects.

Cerebral Hyperperfusion Syndrome After a Burr Hole Drainage Surgery for Chronic Subdural Hematoma.

BACKGROUND: Although chronic subdural hematoma (CSDH) has a good prognosis after classical minimally-invasive drainage surgery, severe complications still occur at a substantial rate. Cerebral hyperperfusion syndrome (CHS), which is a common severe complication after carotid endarterectomy or carotid artery stenting for cervical carotid artery stenosis, is rare after drainage surgery for a CSDH. CASE DESCRIPTION: We describe the case of an 82-year-old woman who presented with ipsilesional symptoms including contralateral hemiparesis and dysarthria, progressively worsening consciousness, and status epilepticus after a burr hole drainage surgery for CSDH. Magnetic resonance fluid-attenuated inversion recovery imaging showed diffuse subcortical low intensity in the ipsilesional hemisphere almost simultaneously with the appearance of the symptoms. Arterial spin labeling magnetic resonance perfusion imaging showed the abnormal increase of cerebral blood flow in the hemisphere. Continuous propofol administration and blood pressure management improved the symptoms. CONCLUSIONS: CHS can cause severe postoperative complications after drainage surgery for CSDH. Subcortical low-intensity fluid-attenuated inversion recovery imaging is a useful investigation for early detection of CHS in CSDH, and arterial spin labeling imaging is an effective minimally-invasive modality for confirming the diagnosis.

Large Cell Neuroendocrine Carcinoma of the Mediastinum Successfully Treated with Systemic Chemotherapy after Palliative Radiotherapy.

Large cell neuroendocrine carcinoma (LCNEC) is a highly malignant cancer originally found in lung in 1991. In extremely rare occasions, primary LCNEC is found in the mediastinum; approximately 40 of such cases have been reported. Due to the limited number of reported cases, a standardized treatment protocol has yet to be established. We report a case of a 66-year-old woman with primary mediastinal LCNEC who presented with superior vena cava syndrome. Emergent radiotherapy was performed, followed by systemic chemotherapy with cisplatin and etoposide, which resulted in a dramatic tumor reduction. This is the first report describing the achievement of a complete response after systemic chemotherapy in a patient with primary LCNEC.

Transcription factor YY1 contributes to tumor growth by stabilizing hypoxia factor HIF-1α in a p53-independent manner.

In response to hypoxic stress, hypoxia-inducible factor (HIF)-1α is a critical transcription factor regulating fundamental cellular processes, and its elevated expression level and activity are associated with poor outcomes in most malignancies. The transcription factor Yin Yang 1 (YY1) is an important negative regulator of the tumor suppressor factor p53. However, the role of YY1 under tumor hypoxic condition is poorly understood. Herein, we show that inhibition of YY1 reduced the accumulation of HIF-1α and its activity under hypoxic condition, and consequently downregulated the expression of HIF-1α target genes. Interestingly, our results revealed that the downregulation of HIF-1α by inhibiting YY1 is p53-independent. Functionally, the in vivo experiments revealed that inhibition of YY1 significantly suppressed growth of metastatic cancer cells and lung colonization and also attenuated angiogenesis in a p53-null tumor. Collectively, our findings unraveled a novel mechanism by which YY1 inhibition disrupts hypoxia-stimulated HIF-1α stabilization in a p53-independent manner. Therefore, YY1 inhibition could be considered as a potential tumor therapeutic strategy to give consistent clinical outcomes independent of p53 status.