[A Case of Internal Jugular Arteriovenous Fistula Presenting as Lower Cranial Nerve Palsy].

INTRODUCTION: Although arteriovenous fistulas(AVFs)are typically located within the cranium, there are several published reports documenting rare cases of extracranial AVFs between the ascending pharyngeal artery(APA)and the internal jugular vein(IJV). Herein, we report the case of a patient with APA-IJV AVFs who presented with symptoms of lower cranial nerve palsy that was treated with transvenous embolization(TVE). CASE: A 53-year-old man presented with chief complaints of numbness in the left oral cavity and a temple headache. Magnetic resonance angiography showed an abnormal signal in the left jugular bulb. An AVF was suspected; digital subtraction angiography revealed the presence of a shunt from the jugular branch of the APA to the jugular bulb which was accompanied by regurgitation into the inferior petrosal sinus(IPS)and sigmoid sinus(SS). Numbness in the oral cavity was diagnosed as lower cranial nerve palsy associated with increased pressure within the jugular foramen. As the nature of the AVF(single or multi-hole)was uncertain, a therapeutic TVE was planned. Microcatheters were guided into the IPS and SS, and TVE was performed using a double-catheter technique. Regurgitation into the IPS resolved; embolization via the APA was not performed, and symptoms improved postoperatively. CONCLUSIONS: AVFs involving the APA and IJV are identified infrequently and there are only a few published case reports describing this vascular anomaly. Most reported cases were single-hole AVF and were treated with trans-arterial embolization via the APA. As noted in the present case, APA-IJV AVF can also be treated by TVE.

Status of In-Hospital Acute Ischemic Stroke Treated by Mechanical Thrombectomy.

Objective: To elucidate the current state of in-hospital acute ischemic stroke under the introduction of acute-phase mechanical thrombectomy. Methods: The study included 18 consecutive patients with in-hospital cerebral infarction who underwent thrombectomy between April 2014 and March 2020 at St. Marianna University School of Medicine Yokohama City Seibu Hospital. We analyzed the primary disease, department responsible for treatment, modified Rankin Scale (mRS) scores before onset and on discharge, status of onset, treatment course, and so on. Results: The mean age was 79.9 (66-93) years. There were nine females. The admission methods included scheduled admission in 5 patients and non-scheduled admission in 13 patients. The primary diseases consisted of malignant tumors in five patients and heart disease in four patients. The departments responsible for treatment consisted of the Department of Digestive Surgery for six patients and Department of Cardiology for three patients. The mRS score before admission was evaluated as 0-2 in 15 patients and 3-5 in 3 patients. The embolism was evaluated as cardiogenic in 14 patients. Antithrombotic therapy was discontinued before the onset of cerebral infarction in three patients. The mean interval from onset or last well known (LWK) until CT/MRI and puncture was 88.4 and 157.6 minutes. The median Alberta stroke program early CT score (ASPECTS; minimum-maximum) was 8 (2-10). Tissue plasminogen activator (t-PA) was administered to five patients. Concerning the degree of recanalization, the thrombolysis in cerebral infarction (TICI) grade was evaluated as 1 to 2a in 2 patients and 2b to 3 in 16. In the latter, the mean interval from onset or final onset-free confirmation until recanalization was 197.7 minutes. mRS score on discharge was evaluated as 0-2 in four patients, 3-5 in nine, and 6 in five patients. The mortality was related to a primary disease requiring admission in three patients. Conclusion: In-hospital onset cerebral infarction was markedly influenced by the primary disease requiring admission. Even when favorable recanalization was achieved, the number of patients with a favorable outcome was small.

[A Case of Primary Intrasellar Chondroid Chordoma].

OBJECTIVES: Intracranial chordomas are thought to arise from remnants of the notochord and usually occur at the parasellar region. We present a case of a primary intrasellar chondroid chordoma in a patient who was initially diagnosed with a pituitary adenoma. CASE: A 77-year-old woman had a history of two surgeries for a tumor in the sella turcica(17 months after the 1st surgery). On initial MRI, the intrasellar mass showed low signal intensity on T1WI, very high signal intensity on T2WI, and inhomogeneous enhancement. On bone reconstructive CT, the sellar floor was thin, and no abnormalities were observed at the top of the clivus. Transsphenoidal surgery was performed. The pathological diagnosis was pituitary adenoma in both cases. Seventy-two months after the 1st(31 months after the 2nd)surgery, she developed right-sided oculomotor and abducent nerve palsies again. Since recurrence occurred during the relatively short period, the surgical specimens obtained from the 1st and 2nd surgeries were reexamined. Reexamination of the previously obtained specimen demonstrated areas of chondroid tissue that were embedded in a mucoid stroma and tumor cells that were composed of round or pleomorphic nuclei with vacuolated cytoplasm(physaliphorus cells)that were compatible with chondroid chordoma. The third surgery was performed. Postoperatively, her symptoms improved, and cyber knife therapy was administered for the residual part of the tumor. CONCLUSIONS: Although intrasellar chondroid chordomas are extremely rare, they should be considered in the differential diagnosis of tumors located in the sella turcica.

Layilin enhances the invasive ability of malignant glioma cells via SNAI1 signaling.

BACKGROUND: Malignant gliomas are characterized by high invasive ability. In this study, we investigated roles of layilin, a C-type lectin-homologous protein, in the invasive ability of malignant glioma cells. METHODS: Expression of layilin was investigated by western blotting in the malignant glioma cell lines of U251-MG, A172, and T98G and in astrocytes. The effects of layilin-knockdown on the expression and protein levels of snail family transcriptional repressor 1 (SNAI1), a transcriptional factor involved in the acquisition and enhancement of invasive ability in malignant gliomas, and on the expression of its target genes, matrix metalloproteinase 2 (MMP2), MMP9, and collagen type I alpha 1 chain (COL1A1), were investigated by qPCR and/or western blotting. Furthermore, the effects of layilin-knockdown on the expression and protein levels of metastasis associated 1 family member 3 (MTA3), a transcriptional repressor of SNAI1, were also investigated by qPCR and western blotting. Finally, the effects of layilin-knockdown on the invasive ability of the cells were investigated by a wound healing assay. RESULTS: All the tested malignant glioma cells highly expressed layilin, compared to astrocytes, one of representative glial cell types. Layilin-knockdown reduced SNAI1 both at the mRNA and protein levels in A172 cells, and consequently mRNA levels of MMP2, MMP9, and COL1A1 were also reduced. Furthermore, layilin-knockdown increased nuclear protein levels of MTA3 in A172 cells. Notably, layilin-knockdown suppressed the invasive ability of the cells. CONCLUSION: Layilin up-regulates the expression of SNAI1 via down-regulation of MTA3. This process enhances the invasive ability of malignant glioma cells.

[A Case of Primary Extradural Meningioma Located at the High Convexity:A Case Report and Review of the Literature].

OBJECTIVES: Primary meningiomas arising outside the intracranial component are rather rare and have been termed primary extradural meningiomas(PEMs). We present a case of an intraosseous-type PEM occurring at a high convexity location and discuss the clinical characteristics of PEMs. CASE: An 80-year-old woman presented with a soft and painless subcutaneous mass of approximately 10 cm in diameter in the right parietal region, which had appeared 1 year previously. Mild cognitive dysfunction and left hemiparesis were observed upon admission. A skull radiograph and a bone window computed tomography scan revealed an osteolytic lesion at the above-mentioned site. Magnetic resonance imaging indicated that the mass was inhomogeneously enhanced and seemed to extend through the skull defect both intra- and extra-cranially. The preoperative diagnosis was a metastatic skull bone tumor due to the patient's history of breast cancer. During surgery, the tumor was found to be solid and had expanded through both the inner and outer tables of the skull, destroyed the inner table at one location, and perforated into the subdural space via the thinned, but not infiltrated, dura mater. The tumor was removed along with a wide margin of surrounding healthy bone and a cranioplasty was performed using a titanium plate. The histopathological diagnosis was atypical meningioma(World Health Organization grade II). Postoperatively, no adjuvant therapies(radiation and/or chemotherapy)were administered and the patient was reported to be well at 8 months post-surgery with no evidence of tumor recurrence. CONCLUSIONS: Although PEMs are rather rare, clinicians should consider the differential diagnosis of osteolytic skull vault tumors.

[De Novo Cavernous Angioma Secondary to a Developmental Venous Anomaly:A Case Report and Review of the Literature].

OBJECTIVE: Previously, cavernous angiomas(CAs)have been thought to be only congenital in origin. Recently, however, a few cases of de novo CAs have been reported in the literature. We present a case of a de novo CA and discuss the etiology of the newly appeared CA. CASE REPORT: A 29-year-old man was presented to a local clinic because of hypersomnia. MRI demonstrated a heterogeneous mass peripherally located, which was in contact with a developmental venous anomaly(DVA)at the left thalamus. Six years before the presentation, he visited the same clinic because of faintness, and MRI results indicated no abnormality except for the DVA. Three weeks later, he suddenly experienced difficulty in speech, and the MRI revealed an increase in the size of the mass. Subsequently, he was admitted at our institution, and neurological examination revealed aphasia and right hemiparesis. A left carotid angiogram on venous phase showed a narrowing of the DVA, which was seen as it entered the internal cerebral vein. The diagnosis of a de novo CA was made. The mass was completely resected through the transcallosal transventricular approach to avoid injuring the DVA. The DVA could not be found during surgery. The pathological diagnosis was in line with the findings of CA. Postoperatively, the patient continued having difficulty in speech and was transferred to another institution for speech rehabilitation. CONCLUSIONS: Although the association of CA and DVA has been described with increasing frequencies recently, the etiology of de novo CA in the case of this association has been a matter of debate. In the present case, it was speculated that a narrowing of the DVA resulted in increased venous pressure and caused the development of de novo CA.

Hypoxia-induced production of peptidylarginine deiminases and citrullinated proteins in malignant glioma cells.

BACKGROUND: Recently, it has been reported that hypoxia highly enhances expression of peptidylarginine deiminase (PAD) 4 and production of citrullinated proteins in some tumor cells. However, little is known about malignant gliomas on this issue. Therefore, we here investigated whether expression of PADs was induced by hypoxia and whether PADs citrullinated intracellular proteins if induced using U-251 MG cells of a human malignant glioma cell line. METHODS: Expression of PADs in U-251 MG cells, cultured under hypoxia or normoxia for 24 h, was investigated by quantitative polymerase chain reaction (qPCR). Citrullination of proteins in the cells and the cell lysates incubated for 48 h with or without Ca2+ was detected by western blotting. Citrullinated proteins were identified by mass spectrometry. RESULTS: The mRNA levels of PAD1, 2, 3, and 4 were up-regulated by hypoxia in a hypoxia-inducible factor-1-dependent manner in U-251 MG cells. In spite of the increased expression, intracellular proteins were not citrullinated. However, the induced PADs citrullinated U-251 MG cell-derived proteins when the cells were lysed. Multiple proteins citrullinated by hypoxia-induced PADs were identified. In addition, the extracellular domain of vascular endothelial growth factor receptor 2 was citrullinated by human PAD2 in vitro. CONCLUSION: Our data may contribute to understanding of pathophysiology of malignant gliomas from the aspects of protein citrullination.

Ruptured dermoid cyst of the lateral cavernous sinus wall with temporary symptoms: a case report.

BACKGROUND: Dermoid cysts are non-neoplastic tumors that arise from defects in the separation of the neuroectoderm. Cyst rupture rarely occurs spontaneously and the most common symptom is headache, followed by seizure. Although many cases of ruptured dermoid cysts present with symptoms, reports of cases that are asymptomatic, or where symptoms disappear, are rare. CASE PRESENTATION: We report the case of a 66-year-old Asian man with a history of sudden onset headache who was found to have high amounts of fat material in the subarachnoid space and a fat suppression mass in the left cavernous sinus. He underwent oral steroid therapy. Five days after starting medication his headache symptoms disappeared. Routine neurological imaging was then performed without surgical procedure. Magnetic resonance imaging revealed evidence of the remains of a static lesion 6 months after his first visit. He has remained headache free for 10 months since the initial event. CONCLUSIONS: Although cases of ruptured dermoid cysts presenting with consistent symptoms have been commonly reported, until now there were few reports on asymptomatic cases or cases where symptoms disappeared. We believe that surgical intervention is unnecessary for ruptured dermoid cysts with minimal symptoms.

[Coil Embolization of a Ruptured Anterior Communicating Artery Aneurysm Forming a Pseudoaneurysm: Report of Three Cases].

The formation of an intracranial pseudoaneurysm due to a ruptured saccular aneurysm is a rare condition that exhibits characteristic angiographic findings referred to as "ghost aneurysm" or "snowman's head". Currently, no detailed information about the treatment of this condition is available. Clipping has been reported to be more effective and better for removing massive hematomas than endovascular intervention. Moreover, endovascular coil embolization during the acute phase carries a high risk of repeated aneurysm rupture due to the fragility of the pseudoaneurysmal blood clot. Here, we describe three cases of pseudoaneurysm formation following the rupture of an anterior communicating artery aneurysm, and suggest the possibility that ruptured saccular aneurysms with pseudoaneurysm formation can be treated safely and effectively with endovascular coil embolization.

Percutaneous transluminal angioplasty in a patient with internal carotid artery stenosis following gamma knife radiosurgery for recurrent pituitary adenoma.

BACKGROUND: Intracranial vascular complications following radiosurgery are extremely rare. CASE DESCRIPTION: We report a case of stenosis in the internal carotid artery 5 years after gamma knife radiosurgery for a recurrent pituitary adenoma. Percutaneous transluminal angioplasty was performed successfully with anatomical and functional improvement. CONCLUSION: These results suggested the importance of monitoring for arterial stenosis in the long-term follow-up. Moreover, this is the first case of endovascular treatment as an effective therapy for intracranial arterial stenosis due to radiotherapy.

Acute phase endovascular intervention on a pseudoaneurysm formed due to rupture of an anterior communicating artery aneurysm.

A 79-year-old woman presented with a subarachnoid hemorrhage. Angiography revealed pseudoaneurysm formation due to rupture of a true saccular anterior communicating artery aneurysm. Coil embolization, limited to the true aneurysm, was performed successfully with a favorable clinical outcome. This procedure can be considered as an alternative treatment option for similar aneurysms in cases where surgical clipping is contraindicated.

Simple transposition technique for microvascular decompression using an expanded polytetrafluoroethylene "belt": technical note.

Microvascular decompression (MVD) is a standard surgical procedure for treating vascular compression syndromes. There are two basic ways to perform MVD: interposition using a prosthesis and transposition. With the transposition technique, adhesions and granuloma around the decompression site are avoided, but the required operation is more complex than that for the interposition method. We describe a simple, quick MVD transposition procedure that uses a small "belt" cut from a sheet of 0.3-mm-thick expanded polytetrafluoroethylene membrane. The belt has a hole at the wide end and the other end tapered to a point. The belt is encircled around offending vessels by inserting the pointed end into the hole. The pointed end is then passed through a dural tunnel over the posterior wall of the petrous bone and is tied two or three times. This method avoids the risks involved in handling a surgical needle close to the cranial nerves and vessels.

[A case of solitary bone cyst in the skull increasing in size after trivial head injury].

A 12-year-old boy had been known to have a small swelling in the left high vertex for several years. After a trivial head hit to the site of the swelling, the swelling enlarged gradually. A bone window CT scan showed a lesion having bubble-like lytic change in the left parietal bone. Similar changes, but small, were able to be pointed out in a CT scan taken seven years previously. In the following 13 months CT scans eventually revealed sequential increases to 3.5 cm in diameter. Surgical exploratory resection of the mass was performed. Intraoperatively, partial destruction of the outer skull table and a simple cyst with serous yellowish brown colored fluid were identified. There was no finding adherent to the diploic structure. The bone defect after excision was reconstructed by using a titanium plate. The patient was followed up for 2 years after the surgery. Bone window CT showed bony development of normal appearance. Histological examination showed the cyst wall consisted of fibrous connective tissue but there were neither epithelial nor endothelial cells. The histopathological diagnosis of SBC was most likely. SBC is relatively common in long bones, but rarely in flat bones. Only several cases of the SBC of cranial bone have been reported. Although a craniectomy for total excision followed by cranioplasty by resin was common, in cases of children, cyst removal with titanium plate application would be an alterative. SBC increasing in size after head injury is extremely rare, but clinicians may need to be aware of cystic skull bone tumors increasing in size after head injury.

Treatment of an unruptured fusiform aneurysm of the internal carotid artery associated with Wegener's granulomatosis by endovascular balloon occlusion. Case report.

A 22-year-old woman developed an unruptured fusiform aneurysm of the internal carotid artery 7 months after being diagnosed with Wagener's granulomatosis. Intracranial aneurysmal formation is an extremely rare complication of Wegener's granulomatosis. This rare case of intracranial aneurysm was treated by endovascular balloon occlusion.

A case of intracerebral metastasis in osteosarcoma without active pulmonary metastasis.

Intracerebral metastasis in osteosarcoma is extremely rare. A 14-year-old girl who had previously been operated upon for osteosarcoma of the femur presented with seizures and left hemiparesis. A right parietal lesion with calcification and brain oedema was found. After resection of the mass, pathology revealed an osteosarcoma metastasis.

Intracranial foreign body granuloma caused by dural tenting suture.

Our patient presented with a mass lesion mimicking a meningioma. The mass was resected, but pathological examination confirmed a foreign body granuloma, which was caused by silk fibres used as tenting sutures 8 years previously. Herein, we describe the case and review the neurosurgical literature on intracranial foreign body granulomas.

[A case of ruptured internal carotid artery "kissing aneurysms": case report and review of the literature].

OBJECTIVE: A rare case of ruptured kissing aneurysms on the right internal carotid-posterior communicating artery (ICPCA) and -anterior choroidal artery (ICAchA) is reported. CASE: A 47-year-old female was transferred to our hospital because of subarachnoid hemorrhage (SAH). Cerebral angiography revealed two aneurysms on the right ICPCA and ICAchA. Right frontotemporal craniotomy was performed to obliterate them on the day of admission. Despite the presence of angiographical cleavage, these two aneurysms were attached to each other tightly, and it was extremely difficult to dissect the space between them and premature rupture occurred. A Sugita long straight clip was inserted parallel to internal carotid artery to obliterate the body of ICAchA aneurysm and the neck of ICPCA aneurysm. Another straight clip was applied to the neck of the former aneurysm. Both PCA and AchA could be secured successfully. Postoperatively, although she developed symptomatic vasospasm on the 10th day, she discharged without any neurological deficits 40 days later. CONCLUSIONS: Because of the difficulty in dissection of aneurysms, the operation for kissing aneurysms has been recognized as hazardous and challenging since Jefferson. We emphasize that a clipping technique described above should be kept in mind as a safe value, though meticulous dissection of each aneurysmal neck followed by independent neck clipping is reasonable.

Developmental contribution of c-maf in the kidney: distribution and developmental study of c-maf mRNA in normal mice kidney and histological study of c-maf knockout mice kidney and liver.

Maf is a family of oncogenes which encodes a nuclear bZip transcription factor protein and has been originally identified from the avian oncogenic retrovirus, AS42. Maf genes have been reported to have critical roles in embryological development and cellular differentiation. In this study, in situ hybridization with (35)S-labeled antisense riboprobes was used to investigate the distribution of c-maf mRNA in balb/c mouse kidneys from 12 (E12) through 17 days (E17) of gestation and then 1 and 4 weeks after birth. Immunocytochemistry of 4-week-old mouse kidney using anti-c-maf antisera was also performed. Kidney and liver sections from c-maf knockout mice at 4 weeks were stained with hematoxylin-eosin, and their histological features were examined. Expression of c-maf mRNA was first detected on E16 in the renal proximal tubules, and it was expressed through 4 weeks after birth. In the c-maf knockout mice at 4 weeks the cytoplasmic volume of the proximal tubule and liver cell was smaller. These findings suggest that expression of the c-maf gene may be involved in the embryological development and/or cell differentiation of kidney and liver cells.