Exertional vertebrobasilar insufficiency (VBI) secondary to the non-atherosclerotic cause is uncommon. We herein report the case of a patient who developed exertional VBI long after extracranial right vertebral artery (VA) dissection. At the time of dissection, the right VA was completely occluded near its origin, but the distal flow was compensated by the collateral flow from the right deep cervical artery (DCA). After conservative management, the patient was discharged without neurologic deficit. Six years later, he developed recurrent VBI in association with the exertion of his right shoulder. A vascular evaluation revealed that the right proximal VA was still occluded, and there was no evidence of right subclavian artery lesions. The intracranial right VA flow was markedly reduced during the period, while branches of the right DCA were given off to the muscles of the right shoulder and neck. Then, occipital artery (OA)-posterior inferior cerebellar artery (PICA) anastomosis was performed. Intraoperative indocyanine green videoangiography (ICG) confirmed that the flow of the right PICA was predominantly supplied from the compensatory flow from the contralateral VA, and the antegrade flow in the right VA was clearly delayed in comparison to that of the left VA while there were prominent branches providing the blood flow to the medulla oblongata. After the anastomosis, these medullary branches provided the blood flow to the medulla oblongata more quickly and extensively than before. Postoperatively, VBI no longer occurred even after exertion. Surgical revascularization can be a viable option in the treatment of refractory VBI of the non-atherosclerotic cause.

We report a rare case of an aneurysm originating from the penetrating artery of the distal middle cerebral artery (MCA). A 76-year-old man without a notable past history presented with sudden-onset severe headache, left hemiparesis, and a decreased level of consciousness. Computed tomography (CT) revealed subarachnoid hemorrhage (SAH) with intracerebral hemorrhage (ICH) in the right temporal lobe extending into the ventricle. Contrast-enhanced CT (CE-CT) demonstrated a focus of contrast enhancement (CE) adjacent to the hematoma in the right frontal lobe. An aneurysm fed by a penetrating artery branching off from the right distal MCA was found on angiography. The patient underwent emergency resection of the aneurysm and hematoma evacuation. Histological analysis revealed that arterial dissection may be an associated factor in the pathogenesis of this peripheral aneurysm formation. A focus of CE within or adjacent to the hematoma may be useful for diagnosing this peripheral aneurysm. ICH can result in a life-threatening situation. Therefore, microsurgery may be the first treatment choice for aneurysms in this location.

Herein, we describe a case of traumatic direct carotid cavernous fistula (DCCF) treated with target coil embolization using the combined transarterial and transvenous balloon-assisted technique. The patient was a 59-year-old woman who had been involved in a vehicular accident. She was admitted to the hospital due to chemosis and exophthalmos. Cerebral angiography revealed a shunt from the internal carotid artery (ICA) to the cavernous sinus (CS), which indicated DCCF. Thus, target coil embolization using the combined transarterial and transvenous balloon-assisted technique was performed. Angiography was performed 1 week after surgery to confirm the disappearance of DCCF. No recurrence was observed during the 1-year follow-up after treatment. Thus, target coil embolization using the combined transarterial and transvenous balloon-assisted technique is safe and effective for the treatment of traumatic DCCF.

Here we describe a case of recurrent ischemic strokes due to fragile innominate artery plaque successfully treated using endovascular stent grafting. An 80-year-old man presented with a history of recurrent strokes that were refractory to medical treatment. Computed tomography and magnetic resonance images of the thorax revealed a gross intramural plaque in the innominate artery. He was successfully treated using endovascular stent grafting. An AFX stent graft device was used to prevent further embolic strokes. The AFX stent graft has a unique endoskeleton design with a thin-walled expanded polytetrafluoroethylene fabric—known as active sealing structure—attached to the implant. Postoperatively, the patient has experienced no recurrent strokes in over 2 years of follow-up. The stent grafting procedure could be an optimal treatment option for treating fragile innominate artery plaques.

Immunoglobulin G4-related sclerosing disease (IgG4RD) is an emerging immune-mediated fibro-inflammatory disorder which can involve any organ. We describe the first IgG4-RD spondylitis treated with total en-bloc spondylectomy (TES). A 55-year-old man presented with back pain. Magnetic resonance imaging (MRI) of the thoracic spine revealed a pathologic compression fracture on T11 vertebral body and both pedicles suggestive of primary bone tumor or bone metastasis. We conducted TES of T11, because we could not exclude the possibility of primary bone tumor including giant cell tumor. Immunohistochemical examination of the pathology specimens from pleura around the pedicle demonstrated diffuse infiltration of IgG4-bearing plasma cells. Six weeks later from the surgery, a delayed serologic test was done and his serum IgG4 concentration was 45 mg/dL. The final diagnosis was probable IgG4RD on the basis of serological, imaging, histopathological findings. After 6 weeks of oral prednisolone treatment, patient's back pain improved dramatically. IgG4RD is very rare systemic disease and its paraspinal soft tissue like pleura involvement with vertebra body invasion was absent until now. Our experience indicated that surrounding soft tissue biopsy would be helpful when a percutaneous vertebra bone biopsy mismatched with the image studies, even though vertebra body was main pathological lesion considering the possibility of IgG4RD.

The authors report a rare autopsy case. A 59-year-old woman underwent flow diverter (FD) therapy using a pipeline embolization device (PED) for a large paraclinoid internal carotid artery aneurysm. Follow-up magnetic resonance (MR) examinations were performed 6 months after the treatment. Although the T2-weighted images showed progressive thrombosis of the aneurysm, the silent MR angiography (MRA) clearly showed the residual blood flow within the aneurysm. The patient committed suicide 2 months after the follow-up MR examinations. An autopsy specimen showed a small section of the defective membranes with the PED that matched the entry point of residual blood flow seen clearly in the silent MRA. Macroscopic photograph and hematoxylin and eosin stained sections showed defective endothelialization. In contrast, complete endothelialization was observed in membranes covering the PED. The autopsy findings after FD therapy showed defective endothelialization that perfectly matched and corroborated the silent MRA findings.

Pneumocephalus is generally secondary to direct damage to the skull base. Spontaneous intracerebral pneumatocele without head injury was extremely rare, but previously reported as a serious complication of shunt procedures. We describe a 40-year-old man with intracerebral pneumocephalus who previously underwent craniotomy for large frontal convexity meningioma and lumbo-peritoneal shunting. He presented with gait disturbance 14 months after tumor resection. Computed tomography and magnetic resonance imaging showed intracerebral pneumocephalus in the right temporal lobe, which continued into the mastoid air cells through a bone defect of the right petrous bone. We performed urgent right temporal craniotomy to reduce the mass effect and to repair the fistula. Intraoperatively, bone defects were identified at the roof petrous bone, into which the encephalocele had penetrated. The herniated cerebral parenchyma was removed, and the pneumocephalus opened. The dura was closed with sutures and covered with fascia. To elucidate the underlying mechanism for the development of intracranial pneumocephalus, the previous images obtained before or immediately after resection of meningioma were reviewed. We founded that multiple preexisting bone defects and encephaloceles, one of which was considered to be the cause of the intracerebral pneumocephalus. This case demonstrates that intracerebral pneumocephalus can be caused by preexisting bone defect and encephalocele, and this finding may be useful for prediction of pneumocephalus after shunt procedures.

Cardiovascular malformations during embryogenesis can lead the inferior laryngeal nerve to branching directly from the cervical vagus nerve and entering the larynx. This rare anatomical variation is known as a non-recurrent inferior laryngeal nerve (NRILN), and increases the risk of accidental injury resulting in postoperative vocal cord paralysis during neck surgery. We report a case of an 83-year-old man who presented with left symptomatic internal carotid artery stenosis with a right-sided aortic arch and aberrant left subclavian artery (ALSCA). We performed carotid endarterectomy (CEA) using intraoperative neuromonitoring to avoid NRILN injury. To the best of our knowledge, this is the first report of searching for a left NRILN by electrophysiology during CEA. Neurovascular surgeons need to understand the variations of the NRILN associated with congenital cardiovascular anomalies and effective use of intraoperative nerve monitoring (IONM). We discuss the embryological origin, IONM, and surgical pitfalls of this case.

Subpial lipomas, which are also known as nondysraphic intramedullary spinal cord lipomas, are not associated with spinal dysraphism resulting from the failed primary neurulation. Retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that originates from the late arrest of secondary neurulation. We treated a 6-year-old boy presented with myoclonus of the lower limbs, who had subpial lipoma at the lumbar cord, just rostral to the low-lying conus, which was tethered by a cord-like structure (C-LS) continuous from the conus and extending to the dural cul-de-sac. Following cord untethering from C-LS and minimal debulking of the lipoma, the myoclonus was improved. Histological examination of C-LS revealed a large central canal-like structure in the neuroglial core and the diagnosis of RMC was made. Subpial lipomas can be incidentally coexistent with spinal dysraphism resulting from the failed secondary neurulation, such as RMC.

We describe a case of pulsatile anterior communicating artery aneurysm (A-com AN) with a peculiar angiographic appearance. An 81-year-old man was referred to the department of neurosurgery for a large A-com AN artery aneurysm, which was detected incidentally. The patient hoped not to be treated but to be followed up. After 4 months, magnetic resonance imaging (MRI) revealed the presence of a cerebral edema and hematoma around the aneurysm, and partial thrombus in the upper wall of the aneurysm was suspected. Based on these findings, the patient underwent an immediate coil embolization a day after admission. However, the portion of the neck remnant increased in size after the first procedure. Therefore, 8 months after the initial procedure, he was treated with stent coil embolization. Contrary to the first procedure, angiographic evaluation revealed an active pulsating aneurysm. Moreover, MRI revealed the presence of a partial thrombus in the upper neck segment of the aneurysm, with an intensity that changed over time. The patient underwent cautious treatment and was discharged without any symptoms. This is the first case study to reveal an A-com AN with active pulsation and the relationship between the pulsatile portion of the aneurysm and thrombosed portion by MRI.

Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm without metastatic potential. Here, we report a very rare sporadic case of an intracranial supratentorial extradural DTF measuring 82 mm in a 1-year-old girl, that recurred twice following surgery over the course of 16 months, requiring two other surgeries. In three surgeries, we resected a huge tumor with the dura which was thought to be tumor origin and removed this tumor infiltrated the frontal skull base by drilling widely. Furthermore, we treated the tumor invading the bone flap using liquid nitrogen for 20 minutes, and subsequently used it to perform a cranioplasty. This tumor has not recurred for past 8 months. DTF invading the skull base is prone to recurrence, and liquid nitrogen treatment is considered to be effective in pediatric patients, who need cranioplasty with tumor-infiltrating autologous bone flaps.

Intracranial cavernous malformations (CMs) are benign vascular malformations that arise mostly within the brain parenchyma, but occasionally from the dura mater. Here, we report an extremely rare case of a 29-year-old pregnant woman presenting with hemorrhage in a dural-based CM at the temporal convexity. She presented with headache at 38 weeks of pregnancy. Imaging showed a hemorrhagic mass lesion in the left temporal lobe. Consciousness was clear, with no apparent neurological symptoms or headache. Her baby was delivered by emergency Caesarean section. Magnetic resonance (MR) imaging revealed an enhanced lesion around the hematoma and flow void appearances. Cerebral angiography confirmed the left middle meningeal artery feeding the lesion with apparent contrast medium pooling. Surgical removal of the lesion as a single block was performed. Histological examinations were consistent with CM. The lesion was located outside the brain and attached to the dura mater of the convexity, so the final diagnosis was dural-based CM. The patient was discharged home with her baby without any neurological deficits, and no recurrence has been seen for 2 years. Dural-based CM at the temporal convexity was detected, presenting as headache induced by hemorrhage during pregnancy. The relationship between bleeding of the lesion and pregnancy remains unclear, but female hormones and vascular growth factors during pregnancy can induce morphological changes and angiogenesis in CMs.

We report a rare case of a split cord malformation (SCM) combined with an open myelomeningocele (MMC) on the right hemicord. The patient was a male neonate, who exhibited an MMC in the lumbosacral region at birth. Both of his lower limbs moved with slight spasticity, but no atrophic changes or clubfoot deformities were seen. Three-dimensional computed tomography (CT) demonstrated a bony septum, and the patient was diagnosed with a type I SCM. Magnetic resonance imaging (MRI) showed an MMC on the right hemicord (a hemimyelomeningocele). The repair of the open MMC and the removal of the septum were performed immediately to prevent infection and neurological deterioration. Intraoperatively, the right hemicord was thinner than the left hemicord. No additional neurological deficits or complications appeared during treatment. Our findings suggest that when a minor hemicord is affected by both an SCM and an open MMC, good functional outcomes of the lower limbs can be achieved.

Pineal glial cysts associated with bilateral hearing impairment are very rare. Here, we present the case of a 13-year-old boy with a pineal cyst, which caused severe bilateral hearing impairment persisting from 6 years of age. When the patient was 6 years old, the bilateral hearing acuity was about 40 dB on audiometry. Upon admission to our otolaryngology department, his audiogram revealed a bilateral worsening of the hearing acuity (80 dB). Magnetic resonance imaging (MRI) revealed an abnormal pineal cyst with tectal compression from the left with hardly normal bilateral brainstem auditory evoked potentials (BAEPs). We obtained informed consent for exploratory surgery and employed the right occipital transtentorial approach for pineal cyst removal. Based on histological examination, we diagnosed a glial cyst of the pineal gland. At 12 months postoperatively, the patient’s hearing improved, showing a bilateral hearing acuity of 40 dB on audiometry. Since the auditory pathway has both crossed and uncrossed fibers at the upper pons and midbrain level, compression at the lateral lemniscus or inferior colliculus level can cause bilateral hearing impairment. In the present case, there was a possible slow pineal cyst growth that eventually compressed the upper pons to the midbrain, lateral lemniscuses, or inferior colliculi from the left side, this eventually led to bilateral hearing impairment. These findings indicate that surgery can improve hearing acuity in patients with a pineal cyst associated with progressive hearing impairment.

Ischemic stroke associated with intracranial aneurysm is rare but potentially happens because of emboli originating from aneurysm sac or aneurysmal thrombosis extension to the parent artery. We describe two patients who present subarachnoid hemorrhage (SAH) soon after ischemic stroke. Case 1. A 51-year-old woman with a history of multiple endovascular therapy for ruptured basilar top aneurysm presented with double vision. Magnetic resonance imaging (MRI) revealed infarcts in the right thalamus and left occipital cortex. Four days after ischemic stroke, she suffered from sudden onset headache, computed tomography (CT) showed diffuse SAH with intraventricular hemorrhage. Case 2. A 62-year-old man presented with right facial palsy and sensory disorder. MRI revealed an infarct in the left pons. Four days after ischemic stroke, he became comatose and CT showed diffuse SAH. Both cases develop ischemic stroke adjacent to the aneurysms and subsequently cause devasting aneurysm rupture, suggesting ischemic stroke as a warning sign of aneurysm rupture. In such cases, early treatment of the aneurysm should be considered.

We describe a rare case of an anterolaterally projecting clinoid segment aneurysm of the internal carotid artery (ICA) causing oculomotor palsy. A 76-year-old woman was referred to our facility because of right oculomotor palsy that had been found just before surgery to remove bilateral cataracts. Neuroimaging revealed that the patient had an aneurysm at the clinoid segment that projected anterolaterally, eroding the anterior clinoid process. The aneurysm was thought to be compressing the oculomotor nerve, which runs at the upper part of the lateral wall of the cavernous sinus, thereby causing oculomotor palsy. Endovascular coiling of the aneurysm was successfully performed, and the oculomotor palsy was alleviated postoperatively. Anatomically, there exists the carotid collar between the arterial wall of the clinoid segment and the anterior clinoid process, containing the clinoid venous plexus in it. Hence, the anterolateral wall of the clinoid segment, although protected by a stiff bony structure, has an anatomical base that allows it to protrude centrifugally. Once protrusion occurs, the bone may be eroded by remodeling caused by the aneurysm’s pulsed beating.

Basilar artery occlusion (BAO) accounts for only 1% of all strokes, and cerebral infarction resulting from tumor emboli has been infrequently demonstrated; therefore, few reports described BAO due to tumor embolus and its treatment experience. We report here an 83-year-old man with an acute BAO caused by embolized lung tumor invading right pulmonary vein that was revealed as metastasis of prostate adenocarcinoma. The patient underwent rapid recanalization through acute thrombectomy with a direct aspiration first pass technique (ADAPT) with Penumbra catheter. Successful recanalization was achieved in reperfusion grade of thrombolysis in cerebral infarction (TICI) 2b, and the embolus revealed a highly elastic hard tumorous mass of which texture was too tough to be caught by stent retriever. Immunohistopathologic examination of the embolus revealed adenocaricinoma of the prostate. In spite of that the recanalization was obtained, the patient died of the brain stem infarction after 7 days from the onset. We experienced a rare case of acute BAO caused by embolized prostate cancer metastasizing lung and invading pulmonary vein. When we face to patients with lung tumor invading pulmonary vein, tumor embolus should have been strongly considered and aspiration thrombectomy may be safer and more effective for the condition because of the difficulty of predicting an embolus’s texture before treatment.

Langerhans cell histiocytosis (LCH) is a disease characterized by the proliferation of Langerhans cells. Most cases of LCH occur in children, although it can be seen in adults as well. We encountered an adult case of LCH. A 44-year-old woman who was diagnosed as diabetes insipidus underwent a magnetic resonance imaging (MRI) of the head which revealed sellar and suprasellar gadolinium-enhanced mass. Prolactin level was high and cabergoline was prescribed. The size of this mass had reduced, so we supposed the tumor was prolactinoma. However, after 4 years of observation, it had increased once again. The biopsy of pituitary stalk lesion was performed via transcranial approach. The histological diagnosis was initially gangliocytoma. The patient complained of back pain after surgery. Three months after the biopsy, a computed tomography (CT) scan revealed multiple osteolytic lesions throughout the entire body. One of the osteolytic lesions of the skull was removed to determine the diagnosis. The pathological examination of the skull led to a diagnosis of LCH. We concluded retrospectively that the lesion of the pituitary stalk was LCH mimicking gangliocytoma though classical pathological findings were not obtained. In conclusion, LCH should be considered as a differential diagnosis in adult cases of diabetes insipidus with hypothalamic–pituitary lesion.

Aneurysms of the large basilar artery (BA) occasionally cause cranial nerve palsy and motor disorder through mass effect. Since 1967, five cases of cerebral aneurysm leading to parkinsonism have been reported. Herein, we describe a rare case of progressive parkinsonism caused by the recurrence of a large aneurysm of the basilar tip after stent-assisted coil embolization. A 66-year-old man visited our hospital with an asymptomatic large aneurysm (maximum diameter, 21 mm) of the BA tip. Magnetic resonance imaging (MRI) revealed no perianeurysmal edema. Coil embolization with a Y-configuration stent with cross-placement was performed. Although thrombus formation occurred and the perforator infarction was complicated, complete occlusion was achieved. Three months later, the patient developed progressive and severe parkinsonism. MRI revealed mild enlargement of the aneurysm and perianeurysmal mesencephalic edema with minor neck recurrence. A trial administration of levodopa and additional stent-assisted coil embolization were performed. Levodopa dramatically improved parkinsonism; thus, the patient’s symptoms were controlled by a continuous levodopa regimen. In a large BA-tip aneurysm patient, moderate regrowth and minor neck recurrence occurred after initial treatment, and chronic compression of the midbrain caused secondary parkinsonism. In such cases, it is important to consider levodopa administration and therapeutic strategies to prevent recurrence or regrowth.

Transient global amnesia (TGA) can be caused by medications, ischemia, metabolic abnormalities, and seizures. We describe two cases of TGA following coil embolization for a basilar-tip aneurysm. A 73-year-old woman developed transient acute anterograde amnesia after coil embolization for a basilar-tip aneurysm. Diffusion-weighted imaging (DWI) revealed an ischemic lesion in the anterior nucleus of the thalamus. A 67-year-old woman developed transient acute amnesia after a stent-assisted coil embolization of a basilar-tip aneurysm. A DWI showed ischemic lesions in the anterior nucleus of the thalamus. Any ischemic changes to areas of the anterior nucleus that are fed by the thalamoperforating and premammillary arteries should be considered in a differential diagnosis for TGA in patients who have undergone coil embolization for a posterior circulation cerebral aneurysm.

Erdheim–Chester disease (ECD) is a rare systemic disease characterized by non-Langerhans histiocytosis. Pituitary involvement, often manifesting as diabetes insipidus, is the most common central nervous system (CNS) lesion. However, significant mass formation compressing the optic apparatus is rarely reported. We present a case of ECD-related suprasellar mass treated with an endoscopic transnasal approach, with emphasis on the surgical strategy and the intraoperative findings. The mass was fibrous, significantly hard, and strongly adhered to the optic nerves, causing visual impairment. A subtotal resection was performed with preserving the adhesion between the mass and the optic nerves, and her visual symptoms improved remarkably after surgery. We highlight the surgical procedure of ECD-related suprasellar mass, from an endoscopic point of view. Due to strong adhesion of the mass to the surrounding optic apparatus and perforators, complete resection may be harmful; judicious mass reduction with preserving such adhesion would contribute to better visual outcomes.

Seizure clusters (SCs) are acute repetitive seizures with acute episodes of deterioration during seizure control. SCs can be defined as a series of grouped seizures with short interictal periods. Vagus nerve stimulation (VNS) is a treatment option for drug-resistant epilepsy. We present a case where VNS suppressed epileptic SCs, which had persisted for several months. A 13-year-old boy with congenital cerebral palsy and mental retardation had drug-resistant epilepsy with daily jerking movements and spasms in both sides of his body. The seizures were often clustered, and he experienced two sustained SC episodes that persisted for a few months even with prolonged use of continuous intravenous midazolam (IV-MDZ). The patient underwent VNS device placement at the second sustained SC and rapid induction of VNS. Because the tapering of IV-MDZ did not exacerbate the SC, midazolam was discontinued 4 weeks after VNS initiation. Non-refractory SCs also disappeared 10 months after VNS. The seizure severity was improved, and the frequency of seizures reduced from daily to once every few months. The epileptic activity on electroencephalography (EEG) significantly decreased. This case highlights VNS as an additional treatment option for SC. VNS may be a therapeutic option if SC resists the drugs and sustains. Additional studies are necessary to confirm our findings and to investigate how device implantation and stimulation parameters affect the efficacy of VNS.

von Hippel–Lindau (VHL) disease is characterized by neoplastic and cystic lesions, such as central nervous system (CNS) hemangioblastoma and clear cell renal cell carcinoma (RCC), arising in multiple organs. Here, we report a case of an RCC that metastasized to a spinal hemangioblastoma in a patient diagnosed with VHL disease. This is a unique case study because visceral neoplasms rarely metastasize to the CNS. The patient had undergone posterior fossa surgery for the removal of hemangioblastomas in the right cerebellar hemisphere as a child. He was diagnosed with RCC at the age of 20 years, and he underwent partial nephrectomy at the age of 35 years. The patient underwent surgical removal of a spinal tumor from Th8, which was also diagnosed as a hemangioblastoma at the age of 40. However, the residual spinal tumor rapidly regrew within 1.5 years. A second surgery was performed due to progressive leg motor weakness. The resected tumor from the second surgery had two distinct components between the tumor center and the margin. Immunohistochemistry of CD10, PAX 8, and inhibin A demonstrated the predominant region of the tumor was RCC. Pathological findings confirmed tumor-to-tumor metastasis of the RCC migrating into residual spinal hemangioblastoma. It can be challenging to distinguish hemangioblastoma from RCC in neuroimaging. We suggest that tumor-to-tumor metastasis should be considered as a differential diagnosis if benign tumors grow rapidly, even if the pathological diagnosis does not initially confirm malignancy. The biological mechanisms of RCC migrating into residual hemangioblastoma are discussed.

Most cases of cavernous sinus dural arteriovenous fistula (CS-dAVF) are treated via the inferior petrous sinus (IPS) through the transfemoral vein approach, but there are cases where treatment through the superficial middle cerebral vein (SMCV) is required. A hybrid operating room (OR) is useful because it allows for smooth direct surgery and endovascular treatment in a clean surgical field. We herein report a case of simultaneous treatment for CS-dAVF by coil embolization via a contralateral SMCV and middle cerebral artery (MCA) aneurysm by clipping in a hybrid OR. A 68-year-old woman had been suffering from left chemosis and ptosis for 2 months before visiting our hospital. Digital subtraction angiography (DSA) revealed Borden type II and Cognard type II a+b CS dAVF with parenchymal hemorrhaging and an unruptured left M1/M2 junction aneurysm. Since passing through the CS via the femoral vein was unsuccessful, we decided to access the right CS via the left CS through the intercavernous sinus (ICS) via the left SMCV by the pterional approach in a hybrid OR equipped with a multi-axis working system angiography machine. Endovascular treatment via direct cannulation into the contralateral SMCV following craniotomy in a hybrid OR is an optional strategy for treating complicated CS-dAVF.

Microvascular decompression (MVD) is the gold standard in the treatment of hemifacial spasm (HFS), and endovascular surgery has been described as a treatment only for aneurysm-induced HFS in several previous cases. We describe symptomatic HFS caused by a normal vertebral artery (VA) trunk adjacent to the ipsilateral dissecting VA aneurysm completely cured after stent-assisted coil embolization. A 52-year-old man presented with a 2-month history of gradually worsening left HFS. Magnetic resonance imaging (MRI) and cerebral angiography revealed a dissecting VA aneurysm on the left side. Based on the findings from preoperative MRI, not the aneurysmal dome itself, but the VA trunk just distal to the aneurysmal dome was considered likely to be compressing the root exit zone (REZ) of the facial nerve. Stent-assisted coil embolization was conducted for the VA aneurysm, and the stent was deployed to cover the wide neck of the aneurysm and offending zone of the VA trunk simultaneously. HFS started to show improvement just after the procedure and complete disappearance within 1 year. HFS was completely resolved by stenting of the offending artery. Stents may show efficacy for “intra-arterial decompression” by reducing pulsatility against the REZ of the facial nerve due to the thickness and rigidity of the stent metal and delayed endothelialization.

Giant cell glioblastoma (GCG) is a rare subtype of glioblastoma multiforme (GBM), and it often occurs in younger patients; however, its onset in children is extremely noticeable. A 7-year-old girl presented with a headache and restlessness. A giant tumor that was 7 cm in diameter was found by magnetic resonance imaging (MRI) in the left frontal lobe with intracranial dissemination. Because the tumor had extended to the lateral ventricles and occluded the foramen of Monro causing hydrocephalus, she underwent ventricular drainage and neuro-endoscopic biopsy from the left posterior horn of the lateral ventricle. The initial pathological diagnosis was an atypical teratoid/rhabdoid tumor (AT/RT). When the dissemination subsided after the first chemotherapy with vincristine, doxorubicin, and cyclophosphamide, she underwent the first tumor resection via a left frontal transcortical approach. After surgery, the second chemotherapy with ifosfamide, cisplatin, and etoposide was not effective for the residual tumor and intracranial dissemination. The second surgery via a transcallosal approach achieved nearly total resection leading to an improvement of the hydrocephalus. The definitive pathological diagnosis was GCG. Despite chemo-radiation therapy, the dissemination in the basal cistern reappeared and the hydrocephalus worsened. She was obliged to receive a ventriculo-peritoneal (VP) shunt and palliative care at home; however, her poor condition prevented her discharge. Ten months after admission, she died of tumor progression. The peritoneal dissemination was demonstrated by cytology of ascites. In conclusion, although unusual, pediatric GCG may be disseminated at diagnosis, in which case both tumor and hydrocephalus control need to be considered.

Telangiectatic osteosarcoma (TOS) is a subtype of osteosarcoma. TOS in the elderly and TOS in the skull are very rare. Here, we report a case of TOS in the frontal bone of an elderly patient. The patient was a 79-year-old woman who was identified as having a right frontal bone lesion. The patient was initially diagnosed with an intradiploic epidermoid cyst (IEC). A 60mm cystic lesion with bone destruction appeared 5 years later, which enlarged over 2 months. The fluid in the cyst seemed to be blood that had lost its clotting ability. Tumor and the surrounding tissue were resected. The postoperative course was favorable. Postoperative magnetic resonance imaging (MRI) showed no evidence of residual lesions. There was no metastases. Histopathologically, the cyst wall was composed of fibrous connective tissue and did not contain epithelial components. There were no skin appendages and keratinized tissues in the lesion. Based on these findings, the diagnosis changed to aneurysmal bone cyst (ABC). Subsequent immunohistochemical examinations confirmed that the MIB-1 index was 50% in some sections and there were atypical cells showing osteogenic properties in other sections. Based on these results, the patient was finally diagnosed with TOS. The differential diagnoses for cystic lesion presenting bone destruction include TOS, ABC, and IEC. In case of cystic lesion with bone destruction, early diagnosis based on histopathological study is important and complete resection with surrounding tissues is required, given the possibility of TOS.

Flow diverter stent has been a promising device for intracranial aneurysm treatment. For treating aneurysms located in the anterior circulation, critical branches may be covered by flow diverter stent. The occlusion incidence of these branches has been reported, and even if branch vessel occlusions occur, associated neurological deficits are extremely rare. We present a 55-year-old woman who had a large saccular aneurysm at the right internal carotid artery (ICA). A developed fetal-type posterior communicating artery (PCOM) originated from the sac. We administered flow diverter stent deployment with coil insertion following surgical anastomosis of the superficial temporal artery to the posterior cerebral artery (STA-PCA) with ligation of the origin of the PCOM. On the seventh morning following the intervention, ischemic complication developed due to anterior choroidal artery occlusion jailed by the flow diverter stent. The occlusion of anterior choroidal artery covered by flow diverter stent is extremely rare. However, if the branch arises from the aneurysm sac, occlusion can transpire and induce serious complication. The most probable cause of occlusion in this case was that the orifice was jailed apart from the stent strut because the branch originated from the sac rather than the neck. Furthermore, the progression rate of intra-aneurysm thrombus formation is also an important factor affecting the side branch occlusion.

Cavernous internal carotid artery (ICA) aneurysm complicated by simultaneous and spontaneous formation of thromboses in the aneurysm and the parent artery is a rare clinical condition. Although the majority of patients have good outcomes, some patients experience severe ischemic stroke. Here, we report a case of symptomatic large cavernous ICA aneurysm complicated by rapid growth of an intra-aneurysmal thrombosis with simultaneous parent artery thrombosis. A 68-year-old female presented with sudden-onset diplopia, right ptosis, right conjunctival hyperemia, and paresthesia of the right face. Magnetic resonance imaging (MRI) and digital subtract angiography (DSA) revealed the presence of a large partially thrombosed aneurysm in the cavernous portion of the right ICA. We planned endovascular embolization using a flow-diverting (FD) stent. Dual-antiplatelet therapy (DAPT) with aspirin and clopidogrel was started 2 weeks prior to treatment. Although the neurological state was stable, DSA conducted on the day of the endovascular treatment showed rapid growth of an intra-aneurysmal thrombosis and de novo thrombosis in the parent artery. Direct aspiration was performed via a distal support catheter with proximal blood flow arrest using a balloon-guide catheter, and the FD stent was successfully deployed. The patient’s symptoms improved postoperatively and DSA obtained 12 months after the procedure confirmed complete occlusion of the aneurysm. Although the exact mechanism of simultaneous thrombosis formation of the aneurysm and its parent artery remains unclear, it is important to recognize that rapid growth of the thrombosis increases the risk of ischemic stroke.

Lumboperitoneal (LP) shunting is a standard treatment for idiopathic normal pressure hydrocephalus (iNPH), with equivalent efficacy to ventriculoperitoneal (VP) shunting, and it is associated with a favorable outcome in approximately 75% of patients with iNPH. Despite the advantages, LP shunting can result in problems associated with the lumbar catheter, the obstruction of which has not been well described. This report presents two cases of LP shunt malfunction caused by lumbar catheter misplacement into the spinal subdural epiarachnoid space (SSES), and by subsequent obstruction. A 67-year-old man and a 69-year-old woman with iNPH underwent LP shunt placement without intraoperative fluoroscopy. Shortly after the surgery, they experienced a temporary improvement of their symptoms which was, however, followed by recurrence within a few months. This was suggestive of shunt malfunction. Although shunt pumping tests were normal, shuntography and subsequent computed tomography (CT) revealed lumbar catheter misplacement into the SSES. Shunt revisions, in which only the lumbar catheters were exchanged, were performed with intraoperative fluoroscopy and shuntography. Their symptoms have improved again following the revisions. In the present cases, lumbar catheter misplacement into the SSES caused LP shunt malfunction, and shuntography and CT were useful to detect the abnormality. Moreover, unrecognized lumbar catheter misplacement into the SSES might potentially have occurred in some patients considered as “non-responders” to LP shunting; hence, shuntography may be useful in those patients.

Discal cysts are rare intraspinal extradural cysts that communicate with the corresponding intervertebral discs, and the diagnosis is difficult to distinguish from other causes of low back pain and radiculopathy. Optimal management for this type of cyst has not been determined because of its rarity. Here, we report successful treatment of a discal cyst and lumbar disc herniation using full endoscopic surgery in a professional baseball player with a chief complaint of weakness in his left lower leg. He had been treated conservatively but symptoms did not improve. Discography helped us to differentially diagnose discal cyst from other cystic lesions. Conventional surgical treatment would have resulted in considerable loss of baseball playing time for the patient. We opted to perform minimally invasive transforaminal full endoscopic surgery under local anesthesia to treat the discal cyst and lumbar disc herniation simultaneously without resection of bone and ligament handling. We removed the discal cyst and disc herniation, which released tension on the left nerve root at the L5 level, and then performed thermal annuloplasty to avoid recurrence. Postoperative course was good and he returned to play baseball at his original competitive level 3 months later. To our knowledge, there have been no previous reports of successful full endoscopic surgery for discal cyst and lumbar disc herniation performed simultaneously in a professional baseball player. It can be difficult to decide on the proper treatment for discal cysts, but full endoscopic surgery for symptomatic discal cyst might be one good option especially for elite athletes.

When a patient presents with dyspnea, most physicians immediately associate it with cardiopulmonary diseases but not with the neurologic ones. Dyspnea due to cervical spondylosis rarely occurs, making it under-recognized. We report a case of a 57-year-old man who complained of dyspnea a month after his traffic accident. Chest X-ray showed a left diaphragm elevation, and cervical computed tomography (CT) revealed foraminal stenoses at C3/4, C4/5, and C5/6 on both sides, especially C3/4 on the left side. Anterior cervical discectomy and fusion at C3/4 and C4/5 were performed via a standard anterior cervical approach. Foraminal stenoses due to osteophyte were found to be more severe in the left side; therefore, thorough foraminotomies were performed. Titanium-coated polyether–ether–ketone (PEEK) cages filled with an artificial bone graft were inserted into both intervertebral spaces. His dyspnea improved immediately after the operation. Postoperative spirometry showed a gradually improving respiratory function. Therefore, cervical spondylosis should be considered to cause dyspnea, although it is an atypical symptom. Considering previous reports, outcomes achieved with surgical treatment were better than that with conservative therapy for cervical spondylotic radiculopathy-related dyspnea.

Growing skull fracture (GSF) is a rare complication of pediatric head injury. Early diagnosis and immediate surgical intervention are required for the prevention of irreversible brain damage. Surgical management involves water-tight closure of the dural defect and commonly uses autologous materials because of tissue compatibility; however, a large skin flap and craniotomy are necessary to harvest the autologous materials and repair the dural defect. We describe a successful case of endoscopic-assisted duraplasty using collagen matrix in a female infant suffering from early phase GSF. A 4-month-old female infant presented with a GSF. We surgically treated her because the fracture width progressively expanded 6 days post-injury. A zigzag skin incision was made, and the extent of the skull fracture and dural laceration was observed using an endoscope. Utilizing the collagen matrix, duraplasty was performed to completely seal the dural defect. Subsequently, cranioplasty was performed and the opposite sides of the fracture margins were drawn and bonded by nylon suture. Postoperatively, the patient did not develop any complication or experience recurrence. This is the first report of duraplasty using collagen matrix in GSF, and the collagen matrix can be used as a dural substitute. This novel technique was safe and a less invasive surgical approach for treating patients with GSF.

Although hemorrhagic complications may arise with thrombolytic therapy using recombinant tissue plasminogen activator (rt-PA), deterioration following administration of rt-PA for hemorrhagic disease is an iatrogenic complication. Caution has recently been raised regarding aortic dissection. A case of cervical epidural hematoma treated with rt-PA is reported herein. The patient was an 87-year-old woman with a history of hemodialysis, brainstem infarction, and stenosis of bilateral internal carotid arteries treated with ticlopidine. She was transferred to our hospital with severe occipital and neck pain. Diffusion-weighted imaging revealed patchy signal hyperintensity in the left cerebellar hemisphere. Right hemiparesis appeared 2 h later, but repeat magnetic resonance imaging (MRI) revealed no new lesions. Administration of rt-PA was performed under a diagnosis of hyper-acute cerebral infarction. Irregular hemodialysis was initiated for pulmonary edema. Complete tetraplegia appeared after hemodialysis, 10 h after rt-PA administration. Repeat MRI revealed cervical epidural hematoma, and hematoma removal was performed. After 10 days, hemiparesis recovered to manual muscle testing (MMT) 2 in the left extremities but remained at MMT0 in the right extremities. Cervical epidural hematoma is a rare complication in stroke practice. Although rt-PA should be administered as soon as possible, since “time is brain,” spending a few minutes on spinal MRI is preferable to prevent iatrogenic deterioration. For atypical cases of cerebral infarction, the possibility of cervical epidural hematoma should be considered.

Meningiomas are the most common intracranial primary neoplasm in adults, and show various histological subtypes, indicating heterogeneous clinical and molecular genetic characteristics. Different subtypes of meningioma coexisting independently within the main tumor of another different subtype is a quite rare clinical situation. A 69-year-old woman presented with a several- year history of dizziness as a non-specific complaint. Magnetic resonance imaging (MRI) revealed an extra-axial mass lesion in the left parieto-occipital region including two well-demarcated, round mass components. Total resection was performed via left parieto-occipital craniotomy. Two white masses were identified within the main tumor, with neither showing dural attachments. Pathological findings showed the main mass represented meningothelial meningioma and the demarcated mass lesions were both fibrous meningiomas. No transitional features existed between these subtypes. No differences in genetic characteristics were evident between subtypes of meningioma. We have described, apparently for the first time, a case of two fibrous meningiomas coexisting in an isolated manner in meningothelial meningioma with the similar molecular genetic profile.

A 74-year-old man presented with symptoms of intermittent claudication. A diagnosis of lumbar spinal canal stenosis (LSS) at L3/4 and L4/5 was made based on the clinical and radiological findings. Bilateral lateral recess stenosis was detected at both these levels on magnetic resonance imaging (MRI) and on computed tomography (CT) scans obtained after myelography. Four nerve roots were impinged bilaterally at L4 and L5. The initial plan was to perform conventional laminectomy at L3/4 and L4/5 under general anesthesia. However, the neurologists pointed out that the patient had comorbidities of parkinsonism and severe carotid artery stenosis, meaning that an increase or decrease in blood pressure during general anesthesia could cause a stroke. Therefore, we changed the surgical plan to four-stage full-endoscopic ventral facetectomy at L3/4 and L4/5 bilaterally under local anesthesia. There were no surgery-related complications after any of the four operations. The patient’s symptoms improved after the final operation and the modified MacNab criteria indicated a good clinical outcome. Full-endoscopic lumbar decompression surgery under local anesthesia may be effective in elderly patients who are in poor general health.

The authors report a rare case of spinal osseous epidural arteriovenous fistula (AVF) with intradural reflux. A 71-year-old lady with a past history of a T12 compression fracture and neurofibromatosis type 1 presented with progressive paraparesis. Magnetic resonance (MR) images of the thoracolumbar spine showed edema of the spinal cord and flow voids. Catheter angiography revealed segmental arteries from T11 to L1 feeding an AVF in the epidural space. The AVF drained not only into the epidural venous plexus but also into the perimedullary veins. Of note, there was an intraosseous drainage route that involved the basivertebral vein of T12. Under the diagnosis of spinal osseous epidural AVF with intradural reflux, surgical interruption of the intradural arterialized draining vein was performed. Spinal osseous epidural AVF with intradural reflux is rare with only four cases reported in the past. We believe that spinal osseous epidural AVF should be recognized as a variant of spinal epidural AVF.

Late relapse of herpes simplex encephalitis (HSE) is defined as the recurrence of HSE more than 3 months after the initial exposure. The postoperative diagnosis of HSE following neurosurgery is complicated because the clinical presentation can mimic other common complications of neurosurgery. Cerebrospinal fluid polymerase chain reactions (CSF-PCR) is the gold standard for the diagnosis of HSE. We describe a case of late HSE relapse after epilepsy surgery in a patient who required a brain biopsy due to repeated negative CSF-PCR results. A 38-year-old woman had a history of HSE from the age of 3 years. She had intractable epilepsy from the age of 20 years and underwent right posterior quadrant disconnection (PQD) at the age of 38 years. Postoperatively, she had a right hemispheric intracerebral hemorrhage (ICH) and her consciousness was gradually worsening. Her consciousness improved after removal of the ICH. However, her consciousness gradually deteriorated again. Fluid-attenuated inversion recovery (FLAIR) revealed bilateral hyperintensity in the frontal lobes, including the white matter. CSF-PCR for herpes simplex virus (HSV) was performed twice, but yielded negative results. We performed a brain biopsy to target FLAIR hyperintensity in the right frontal lobe. PCR of the brain specimen was positive for HSV. Her consciousness improved with acyclovir, methylprednisolone, and cyclophosphamide. To our knowledge, this is a case of HSE induced by epilepsy surgery which had the longest duration until relapse after the initial HSE episode. A brain biopsy can be used to confirm the diagnosis of suspected HSE when CSF-PCR results are negative.

Iatrogenic dissection (ID) is a well-known complication of neuroendovascular treatments. ID is predominantly attribute to endothelial injury by the manipulation of wires and/or catheters, and is generally detected in angiography during the procedure. We present a rare case with delayed ID due to deployment of a carotid stent. A 71-year-old man presented with transient motor weakness in the right extremity. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) showed previous multiple cerebral infarctions without a diffusion sign, stenosis with vulnerable plaque in the left common carotid artery (CCA), and an extremely flexed internal carotid artery (ICA). On dual antiplatelet medication, carotid artery stenting (CAS) was completed with favorable dilation of the carotid lumen. Computed tomography angiography 4 days after the procedure revealed high-grade stenosis at the ICA adjacent to the distal edge of the deployed stent. ID with intramural hematoma was diagnosed on MRI. The ID was conservatively treated and remarkably diminished 4 months after the procedure. The patient was asymptomatic during the entire clinical course. This delayed ID was considered to be due to an endothelial injury caused by the distal edge and the constant radial force of the open-cell stent against the flexed vessel and exacerbated by dual antiplatelet therapy. Even in a patient with favorable arterial dilation in CAS procedure, the possibility of a delayed ID should always be considered.

Tremor associated with encephalitis is usually transient and rarely becomes chronic and refractory. Treatment for such tremor using deep brain stimulation (DBS) has not yet been reported. We report an uncommon case of chronic tremor after encephalitis of unknown etiology and its outcome treated with thalamic DBS. A 47-year-old man presented with a 6-month history of medically refractory tremor after non-infectious and probable autoimmune encephalitis. The patient showed an atypical mixture of resting, postural, kinetic, and intention tremor. The tremor significantly disabled the patient’s activities of daily life (ADL). The patient underwent bilateral thalamic DBS surgery. DBS leads were placed to cross the border between the ventralis oralis posterior (Vop) nucleus and ventralis intermedius (Vim) nucleus of the thalamus. Stimulation of both the Vop and Vim using the bipolar contacts controlled the mixed occurrence of tremor. The ADL and performance scores on The Essential Tremor Rating Assessment Scale (TETRAS) improved from 47 to 0 and from 44 to 9, respectively. The therapeutic effects have lasted for 24 months. Administration of combined Vop and Vim DBS may control uncommon tremor of atypical etiology and phenomenology.

We report a rare case of primary central nervous system (CNS) lymphoma as methotrexate-associated lymphoproliferative disorders (MTX-LPD). A 75-year-old woman who had been treated for rheumatoid arthritis (RA) with MTX for 3 years was admitted to our hospital complaining of unsteady gait, nausea, and vomiting. T2-weighted image of magnetic resonance imaging (MRI) showed multiple high intensity mass-like lesions including right lateral, frontal and temporal lobes, and right cerebellar hemisphere. We performed surgical biopsy, and the pathological and immunohistochemical examinations identified T-cell lymphoma. The tumor regressed and the symptoms were resolved soon after MTX withdrawal. Primary CNS lymphoma due to MTX-LPD is a rare disease and only eight cases including ours are reported.

We experienced a rare case of transdural herniation of cauda equina caused by increased pressure with spinal subdural extra-arachnoid hygroma (SSEH) following lumbar microsurgical decompression. A 68-year-old woman presented with complaints of right leg pain and intermittent claudication. By the diagnosis of L2/3 lumbar spinal stenosis, microsurgical decompression was performed. The surgery was successful with no issues arising such as damage to the dura mater. Lumbar magnetic resonance imaging (MRI) performed 8 days after the surgery confirmed asymptomatic SSEH on the ventral side of the cauda equina. However, posterior cervical pain and lower back pain developed 32 days after the surgery. Lumbar MRI demonstrated that SSEH had markedly increased and advanced from the lumbar spine to the cranium, compressing the spinal cord posteriorly. In addition, herniation of the cauda equina was confirmed in the dura. An emergency surgery was performed. The herniated cauda equina was fully positioned in the dural sac, and the arachnoid membrane with accumulation of spinal fluid on the ventral side was fenestrated. Immediately after the surgery, the patient’s symptoms disappeared. Sufficient caution is required regarding the possibility of SSEH associated with spinal failed back surgery syndrome as it can become excessively enlarged, leading to a poor prognosis.

A typical teratoid/rhabdoid tumors (AT/RT) are highly malignant embryonal tumors in children that are associated with inactivation of the integrase interactor 1 (INI1) gene. Several adult cases of AT/RT have been reported, which were characterized by the sellar occurrence and predominantly occurred in females with INI1 mutation variants. However, clinical and genetic features are poorly understood in this unusual entity. We experienced a case of a 45-year-old female with sellar AT/RT presenting diplopia, who underwent subtotal removal of the tumor by the endoscopic endonasal transsphenoidal approach. Pathological diagnosis was AT/RT with INI1 inactivation on immunohistochemistry. Subsequently, multiple lung metastases were confirmed on fluorodeoxyglucose positron emission tomography (FDG-PET). Although she received postoperative chemoradiotherapy, she died of cerebrospinal fluid dissemination. Autopsy revealed cerebrospinal dissemination and lung metastasis of AT/RT. Biallelic alterations in the INI1 gene were identified by direct sequencing, harboring on different alleles (compound heterozygous mutations) was observed, which is the potential genetic pattern in adult AT/RT. Literature review indicated that lung metastasis frequently occurs in sellar AT/RTs, which is accompanied by cavernous sinus invasion. These observations suggested that cavernous sinus invasion causes haematogenous metastasis to the lung in sellar AT/RT. We discuss clinical and pathological features in adult sellar AT/RT to improve understanding of this unique entity.

Cerebral hyperperfusion syndrome (CHS) after surgical clipping for cerebral aneurysm is a rare entity. The authors present a 76-year-old woman with a large left internal carotid-posterior communicating artery aneurysm. After successful clipping with temporary occlusion of the internal carotid artery, the patient exhibited motor aphasia. ¹⁵O-gas positron emission tomography (PET) showed extreme elevation of the regional cerebral blood flow (rCBF) along with a mildly decreased regional cerebral metabolic rate for oxygen (rCMRO₂) and a remarkable decrease in the oxygen extraction fraction (OEF) in the territory of the ipsilateral superior trunk of the middle cerebral artery. These data indicated local hyperperfusion. She had fully recovered from the aphasia by postoperative day (POD) 18. PET showed normalization of CBF on POD 27. To our knowledge, this is the first case report to show hyperperfusion syndrome, clearly detected by ¹⁵O-gas PET, after aneurysmal neck clipping.

Dorsal arachnoid web (DAW) is a rare entity, which has been reported only in the thoracic spine. The authors report the first case of DAW developing in the cervical spine. A 78-year-old man with several-year progressive gait disturbance and bilateral lower-extremity numbness was referred to our hospital on the suspicion of a non-enhancing cystic cervical spinal tumor. Magnetic resonance imaging (MRI) showed a focal indentation along the dorsal surface of the spinal cord at C7 associated with widened cerebrospinal fluid (CSF) space and increased T2-weighted signals in the cord at C5–C7. DAW was suspected, but because of the atypical location for DAW, computed tomography (CT) myelogram was performed and demonstrated an incomplete blockage of the CSF flow at C7 with neither visible abnormal membranes nor a cyst formation. Intraoperative ultrasonography and operative findings revealed that two fluttering membranes disturbed the CSF flow. The pathology of the excised membranes was arachnoid tissues. DAW should be suspected based on the characteristic MRI findings even if the location is in the lower cervical spine. This case seems to support the theory that DAW may be an incomplete or disrupted formation of an arachnoid cyst.

Dermatofibrosarcoma protuberans (DFSP) originates from the dermal layer of the skin; the optimum treatment is an extended marginal resection. We describe a case of DFSP of the scalp with a skull invasive defect that was thoroughly examined pathologically to determine the optimum length of surgical margins. The tumor cells infiltrated up to 26 mm into the dermal tissues, whereas no infiltrating tumor cells were present in the skull, indicating the combination of marginal resection of the dermal tissues and lower of the skull can be a clinically relevant strategy for treatment of DFSP cases with skull invasion.

Intravenous indocyanine green (ICG) videoangiography is reportedly useful for vascular neurosurgery, and for treating hemangioblastoma due to its high vascularity. Videoangiography obtained after intra-arterial ICG injection has emerged as a more useful option than that after intravenous injection. This report offers the first description of a case of hemangioblastoma successfully treated using intra-arterial ICG videoangiography, and describes the efficacy of this technique. A 20-year-old man presented with progressive cerebellar ataxia and dysphagia. Magnetic resonance imaging (MRI) revealed an enhanced solid tumor in the medulla oblongata. Digital subtraction angiography (DSA) showed a highly vascularized tumor. Surgery was performed to remove the tumor in a hybrid operating room. A catheter was introduced into the vertebral artery (VA) for intra-arterial ICG videoangiography. Superficial feeders and drainers were identified and flow dynamic changes in the tumor were assessed by intra-arterial ICG videoangiography. The tumor was removed after confirming lack of flow in the drainer. Intra-arterial ICG videoangiography was more useful than intravenous ICG videoangiography in hemangioblastoma surgery for identifying feeders and drainers and assessing flow dynamics in the tumor. Use of Flow 800 made these findings simpler and easier to evaluate.

Brain stem gliomas (BSG) in adults are rare and less aggressive than those in children. However, the molecular profile of adult BSG cases has not been well characterized. We report a case of adult BSG with isocitrate dehydrogenase (IDH) mutation. A 43-year-old male was admitted to our hospital with diplopia and right-sided hypesthesia. An open biopsy led to the tumor being diagnosed as a diffuse astrocytoma. Immunohistochemically, the tumor was positive for IDH1 R132H, but negative for H3K27M. The patient received 54 Gy of local radiotherapy and adjuvant temozolomide, which resulted in the size of the lesion decreasing significantly. At 56 months after the initial diagnosis, the patient was referred to our hospital with a severe headache and ataxia. Magnetic resonance imaging (MRI) revealed a contrast-enhanced lesion in the brain stem, which extended into the left cerebellar hemisphere and brainstem. Partial tumor removal was performed, and a pathological examination revealed the features of glioblastoma. Immunohistochemically, the tumor was positive for IDH1 R132H and p53 and negative for ATRX. To the best of our knowledge, there are few reports about adult case of brain stem astrocytoma to be confirmed via histological and molecular examinations of the primary and recurrent tumor. We exhibit detailed pathological and molecular findings which resembles to IDH mutant supratentorial diffuse astrocytic tumors.

A de novo aneurysm of a cerebral artery, defined as a newly growing aneurysm after aneurysmal clipping, but not close to a previously clipped one, is relatively rare. Five studies have reported that the annual incidence of de novo aneurysm formation ranged from 0.3% to 1.8%. A 56-year-old man presented with headache. Magnetic resonance angiography (MRA) and computed tomography (CT) showed an aneurysm with arachnoid hemorrhage located at the left middle cerebral artery (MCA) associated with an azygos anterior cerebral artery (ACA). Eight years later, the patient complained of dizziness, and MRA demonstrated no visualization of the MCA on the left due to metal artifact, but a new lesion, an azygos ACA aneurysm, 9 mm in diameter, was seen. Clipping was performed using multiple clips through the interhemispheric space. Late follow-up examination with MRA or three-dimensional CT to detect de novo aneurysms should be considered in a patient with this vascular anomaly after subarachnoid hemorrhage.

Hemorrhagic venous infarction secondary to deep brain stimulation (DBS) surgery occurs rarely and can cause delayed intracranial hemorrhage. Venous cerebral infarction after DBS surgery is commonly caused by coagulation of the superficial cerebral veins, which usually produces transient symptoms but leaves no permanent sequelae. We report a case of hemorrhagic venous infarction resulting in severe sequelae, likely due to coagulation of the lateral venous lacuna during DBS surgery.