Background: Endodermal cyst (EC) is a rare congenital cyst of endodermal origin, but the pathogenesis of this entity remains uncertain. Supratentorial EC is particularly uncommon, but some cases have been reported. Here, we report a case of supratentorial EC that developed at the frontal base which indicates posttraumatic development rather than a congenital origin. Case Description: A 65-year-old man who had a history of orbital bone fracture without rhinorrhea sustained in a traffic accident presented with gradually enlarging frontal-base cystic lesions. Multiple cystic lesions were removed via left frontal craniotomy. The cysts showed no communication with the frontal sinus. Histological examination identified EC. Postoperative course was uneventful and no recurrences have been identified as of 2 years later. Conclusions: According to reported cases, unlike ECs in other intracranial locations, frontal base ECs tend to present at advanced ages. The present case also presented with EC enlargement at an advanced age and two lesions located at the bone hiatus in the frontal base that were presumably caused by trauma. It is possible that sinus communication was repaired as the bone fracture was remodeled, and the remnant sinus epithelial tissues developed into ECs over time. This situation makes it reasonable to presume a posttraumatic rather than a congenital origin. In conclusion, as for frontal base ECs, contrary to the traditional theory, the developmental mechanisms may not necessarily be congenital.

Aortic mural thrombus (AMT) in the ascending aorta is a rare source of embolism. Recently, the usefulness of contrast computed tomography (CT) has been reported, and we sought to examine the differences between cardiac CT and CT angiography (CTA). A 58-year-old patient of acute embolic infarction was treated by endovascular thrombectomy. Postoperative cardiac CT revealed the AMT as an embolic source. The lesion was not detected by the CTA performed 2 days before. This is the first case report of AMT to highlight the apparent utility of cardiac CT. Although trans-esophageal echocardiogram (TEE) is still the first choice for routine embolic exploration, cardiac CT may play a role as an alternative tool aimed to detect small size AMT.

Anterior spinal artery (ASA) aneurysms are rare, and the majority are associated with vascular lesions such as arteriovenous malformations, moyamoya disease, and aortic stenosis. Herein, we report a case of a ruptured anterior spinal artery aneurysm caused by bilateral vertebral artery (VA) occlusion, which was treated by coil embolization. An 83-year-old man was found collapsed at home, and was brought in by emergency. His consciousness level was I-1 on the Japan Coma Scale, and there were no symptoms such as paralysis in the extremities. Computed tomography showed Fisher 3 subarachnoid hemorrhage, while magnetic resonance angiography showed an aneurysm in the right VA. Digital subtraction angiography showed bilateral VA occlusion, and an aneurysm was found on the dilated ASA as a collateral circulation. Coil embolization was performed after confirmation of no hemodynamic problems. No postoperative adverse events were observed. Coil embolization may be an effective treatment for ruptured aneurysms of the ASA.

The classification of spinal extradural arteriovenous fistulas (AVFs) was reported based on a case series treated by microsurgery in 2009 and endovascular interventions in 2011. The present report describes a patient with extradural AVFs at the cervical spine manifesting gradual progressive radiculomyelopathy of bilateral upper extremities. Magnetic resonance imaging (MRI) revealed a mass sign from C1 to C4 at the right ventral side and the spinal cord was deviated to the left and indicated as a flow void sign. Diagnostic angiography revealed an extradural AVFs located at the C1–C4 level that was supplied by bilateral radicular artery from the vertebral artery (VA) and right ascending cervical artery (ACA). The shunting points were recognized multiply at C2/3 and C3/4 levels on the right. The transvenous embolization to the enlarged extradural venous plexus around the shunting points via right hypoglossal canal and the transarterial embolization against multi-feeders of the branch of left radicular artery, right ACA achieved complete occlusion of the lesions. His symptom was gradually recovered, and angiography performed 2 weeks after embolization showed no recurrence. When the arteriovenous shunts in the upper cervical spine were high flow shunts, transvenous approach via the hypoglossal canal might be one option for the treatment of spinal extradural AVFs.

Invasive sphenoid sinus aspergillosis is a rare infection and usually affecting immunocompromised patients. We describe an invasive sphenoid sinus aspergillosis patient with immunocompetent who present progressive ocular dysfunctions. A 66-year-old woman with no history of immune dysfunction was referred to our hospital with orbital complications. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mass lesion extending from the left orbital apex to the sphenoid sinus. Inflammatory diseases were not suspected by laboratory findings, but a transnasal endoscopic biopsy revealed sphenoid sinus aspergillosis. After treatment of antifungal medication, this patient showed improvement and no sign of recurrence during the follow-up period. Diagnosis of invasive sphenoid sinus aspergillosis in an immunocompetent, healthy individual, was challenging. However, if patients have sinus wall deformities and orbital complications, early surgery is necessary to improve their prognosis.

Primary meningeal melanocytoma is a rare benign tumor in the central nervous system (CNS), comprising less than 0.1% of all intracranial tumors. A 44-year-old man presented with occipital headache, nausea, and vomiting. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well-defined intradural extramedullary mass lesion at the craniovertebral junction (CVJ). Gross total removal was achieved, and the patient improved symptomatically. The pathologic findings were consistent with meningeal melanocytoma. No tumor recurrence was seen on follow-up MRI two years after surgery. Cases of primary meningeal melanocytoma located at the CVJ are rare. The preoperative differential diagnosis of meningeal melanocytoma from meningioma is sometimes difficult because of their similar appearance on CT and MRI. Complete surgical removal is curative for most cases. We present a case of gross total removal of a meningeal melanocytoma located in the CVJ with references to the literature.

Situs inversus is a rare congenital anomaly which has the mirror image of systemic organs and vessels from their normal position. We report a case of endovascular thrombectomy for acute middle cerebral artery (MCA) M1 occlusion in a patient with complete situs inversus. A right-handed man in his 70s presented to our hospital with loss of consciousness, right-sided hemiplegia, and total aphasia. Endovascular thrombectomy was undertaken for left M1 occlusion. Guide catheter advanced through right aortic arch and injection showed innominate artery was on the left side. Left common carotid artery arose from left innominate artery. Using aspiration catheter and stent retriever, successful recanalization was achieved after three passes. For treatment of acute stroke with large vessel occlusion in patients with situs inversus, understanding anatomy and clinical features of situs inversus is important.

Double-lumen carotid plaque is a rare pathological condition, and only few reports about this condition have been recorded in the literature. However, no study has used endovascular therapy (EVT) for the treatment of double-lumen carotid plaque. Herein, we present a unique case of double-lumen carotid plaque associated with severe stenosis that was successfully treated with staged angioplasty (SAP). Moreover, a literature review of its pathology and other treatment options has been conducted. SAP is a two-stage carotid artery stenting (CAS) that can prevent hyperperfusion syndrome after revascularization. In this study, a 62-year-old man developed walking disturbance and left hemiparesis. Magnetic resonance imaging (MRI) revealed ischemic lesions in the watershed area of the right hemisphere and an irregular plaque in the right cervical internal carotid artery (ICA). Ultrasonography showed 84% stenosis in the area and a double lumen distal to the stenosis in the right ICA. Digital subtraction angiography (DSA) revealed a double-lumen plaque with 70% stenosis based on the North American Symptomatic Carotid Endarterectomy Trial criteria. SAP was performed after medication therapy and rehabilitation, and the surgery was uneventful. A double-lumen carotid plaque associated with severe stenosis is a rare condition with a high risk of emboli and stroke. In an unstable lesion, carotid endarterectomy is the first option. However, since the patient in this case was at high risk for general anesthesia, SAP was performed. Hence, if an appropriate device is used, EVT can be a safe treatment strategy for unstable and atypical plaques as in this case.

We present an 88-year-old man with cerebral hyperperfusion (CH) after acute reperfusion therapy. He developed acute cerebral ischemia as a result of occluded middle cerebral artery that was subsequently recanalized with endovascular thrombectomy. I-123 N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (SPECT) after reperfusion therapy showed increased cerebral blood flow (CBF) in brain areas that exhibited no abnormal findings on magnetic resonance imaging (MRI). Follow-up MRI did not demonstrate structural brain damage associated with CH. However, later I-123 iomazenil SPECT imaging showed a reduction in benzodiazepine receptor binding potential (BRBP) in these areas, a finding that correlates with cortical neural damage. CH is being increasingly observed after endovascular treatment for acute stroke. However, little is known about CH when not associated with cerebral hemorrhage or infarction. The role of CH after reperfusion therapy in causing brain damage remains unclear. BRBP on I-123 iomazenil SPECT images is useful to evaluate brain neural density: a reduction in cortical BRBP indicates cortical neural damage or loss. Our findings suggest that post-reperfusion hyperperfusion induces cortical neural damage even in the absence of associated brain infarction or hemorrhage on MRI. Early postoperative SPECT is recommended to detect CH after acute reperfusion therapy. CH should be considered when the recovery from stroke is unexpectedly poor for a patient.

Anterior cranial fossa (ACF) dural arteriovenous fistulas (ACF DAVFs) are an infrequent subtype of cranial DAVFs that are usually fed by the anterior ethmoidal artery (AEA) and ophthalmic artery (OA) branches. Due to the lack of dural venous sinus in the ACF, they directly drain into cortical veins, resulting in high bleeding tendency. For this reason, ACF DAVFs have to be treated regardless of whether they are symptomatic or not. A 74-year-old man, with hypertension history, came to our attention because of ear pain, dizziness, and impaired hearing for 2 months. No other pathological conditions have been found in his medical history. The patient underwent brain magnetic resonance imaging (MRI) scan and subsequently second level diagnostic investigations with digital subtraction angiography (DSA), showing a foramen caecum (FC) patency and a persistent, enlarged, arterialized FC vein (FCV) involved in an incidental ACF DAVF (Cognard IV), fed mainly by sphenopalatine arteries (SPAs) branches bilaterally. The patient underwent open surgery performed by small high frontal craniotomy with DAVFs complete closure and without any complications. To the best of our knowledge, this is the first case ever described of FCV persistence with involvement in an intracranial vascular malformation. It has been managed by surgical intervention that can be considered, despite the large skin incision, a minimally invasive approach with an excellent cosmetic result and minimal risk of complications.

Spinal intradural hemorrhage is a rare event; the most common causes of spinal bleeding are traumas, medical therapy with anticoagulants and thrombolytics, vascular malformations, and congenital defects of coagulation. Rarely, spinal cord tumors may cause hemorrhage. Herein, we report the case of a patient with acute and quickly worsening lumbar pain: the neurological examination revealed a flaccid paraplegia caused by an intradural lesion extending on the right side of the spinal cord from T1 to L2 vertebral level. Pathological examination revealed an hemorrhagic schwannoma. Acute spinal subdural hemorrhage caused by spinal schwannomas is a very rare occurrence (29 cases only have been previously reported). Review of the literature with clinico-diagnostic features is presented, surgical treatment is explained, and pathological findings with possible etiopathogenesis of hemorrhage are described.

In recent years, extracorporeal cardiopulmonary resuscitation (ECPR) has been reported to be an effective alternative to conventional CPR for treating patients with reversible causes of cardiac arrest. Nevertheless, the definite indication for ECPR and also surgical interventions during ECPR treatment have not been established, especially in patients with out-of-hospital cardiac arrest (OHCA) caused by subarachnoid hemorrhage (SAH). We treated a comatose 50-year-old woman with refractory cardiac arrest due to aneurysmal SAH-induced takotsubo cardiomyopathy (TCM). The initial cardiac rhythm was ventricular fibrillation. This is the first case report on coil embolization being successfully performed on a patient undergoing ECPR and therapeutic hypothermia (TH) while the patient was still in cardiac arrest, which resulted in complete social rehabilitation. Moreover, the success of this treatment suggests that ECPR and endovascular therapy should be considered for highly selected patients when cardiopulmonary and neurological functions are potentially reversible even in the setting of SAH.

Primary leptomeningeal malignant lymphoma (PLML) is a rare variant of primary central nerve system malignant lymphoma (PCNSL) which is restricted to leptomeninges. The lesions of PLML can often be detected as abnormal enhancement on the surface of central nervous system or the ventricular wall on magnetic resonance imaging (MRIs). Cerebrospinal fluid (CSF) evaluation together with such MRI findings provides the definitive diagnosis of PLML. Here, we present a 45-year-old female case of PLML in which hydrocephalus with disproportionately large fourth ventricle was observed at presentation with gait instability. Head MRI revealed no abnormal enhancement and CSF cytology was negative, leaving the cause of hydrocephalus undetermined. Endoscopic third ventriculostomy (ETV) was effectively performed for hydrocephalus and her symptoms disappeared. Nearly 2 years later, she was brought to emergent room due to unconsciousness with the recurrence of hydrocephalus. MRI showed expanded fourth ventricle and abnormal enhancement on the ventricular wall. The endoscopic surgery for improving CSF flow was successful and inflammatory change was endoscopically observed on the ventricular wall involving aqueduct. Pathological diagnosis of the specimen from the ventricular wall proved B-cell lymphoma. Because neither brain parenchymal masses nor systemic tumors were identified, she was diagnosed with PLML and treated by high-dose methotrexate. She was in a stable state 2 years after the diagnosis of PLML. We report and discuss the characteristics of this case.

Behçet’s disease (BD) is a rare chronic inflammatory disease associated with systemic vasculitis. Involvement of the nervous system in BD is called neuro-BD (NBD). Epilepsy related to NBD is uncommon but responds well to anti-epileptic drugs. We present a case of NBD with drug-resistant mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS). The patient presented with headache, dizziness, disorientation, and generalized seizures. Magnetic resonance imaging (MRI) identified pontine lesions. Chronic inflammation was suspected, and steroid pulse therapy improved his symptoms. He relapsed 1 year after onset and was diagnosed with NBD. MRI revealed bilateral mesial temporal lesions, with the right being edematous and the left atrophic. NBD was controlled by steroid and immunosuppressive medication. Three years after the onset of NBD, the patient suffered MTLE, and MRI suggested left hippocampal atrophy. His seizures became drug-resistant and surgical therapy was considered 12 years after NBD onset. Pre-surgical MRI clearly showed left HS. After evaluations, the patient had left anterior temporal lobectomy (ATL) 13 years after NBD onset under stable NBD. The patient was seizure-free for > 2 years after surgery. Surgery will be an effective treatment for drug-resistant MTLE with HS even in patients with NBD, of course the effects of surgical intervention should be considered.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, and develops infrequently in the central nervous system. To our knowledge, this is the first case of EHE of the spinal cord. An 85-year-old man presented with about 6-month progressive myelopathy. Magnetic resonance imaging (MRI) demonstrated an oval-shaped intradural extramedullary mass at T10 level with extensive intramedullary edema. A reddish tumor was removed via a total laminectomy of T9−T10. Histologically, the tumor cells with nuclear atypia and active mitoses were immunopositive for vascular tumor markers, and formed a lobular architecture associated with capillary-sized vessels lined with edematous endothelial cells. Although very rare, EHE should be considered in the differential diagnosis of a spinal intradural extramedullary tumor.

Adhesive arachnoiditis (AA) is a chronic inflammation inside the dura and remains one of the most challenging diseases. We describe a case of treatment-resistant extensive AA that offers insight into surgical treatment selection. The patient had a 2-year history of progressive spastic gait and was diagnosed with syringomyelia caused by extensive AA. Although syringe-subarachnoid and subarachnoid-subarachnoid shunting resulted in recurrence within a short period, syringo-peritoneal shunting improved the symptoms and there was no recurrence. This case suggests that syringo-peritoneal cerebrospinal fluid (CSF) shunt drainage, which has previously been considered a further step, may be a first-surgery option for extensive AA.

There are some intraspinal cystic lesions presenting with myelopathy. We report a case of myelopathy caused by a possible thoracic interdural ganglion cyst. A 70-year-old man with subacute bilateral lower extremity numbness, muscle weakness, and gait disturbance presented to our hospital. Magnetic resonance (MR) images showed a cystic lesion which compresses the left dorsolateral intraspinal space of T2-3. During surgery, a ganglion cyst was found without adhering to the periphery of the epidural space. The capsule and contents were removed. He showed postoperative improvement in activities of daily living. A postoperative pathological diagnosis of ganglion cyst was made. The development mechanism of thoracic interdural ganglion cysts is unknown. To our knowledge, this is the first report of this disease. Surgery improved symptoms of a patient with myelopathy caused by thoracic interdural ganglion cysts. This must be considered as one of the cystic lesions presenting with myelopathy.

Implantation of left ventricular assist device (LVAD) is widely performed in patients with end-stage chronic heart failure. Infection and stroke are major complications after LVAD implantation. However, the incidence of intracranial mycotic aneurysm after LVAD implantation is rare, and with no standard of care. In this study, we describe a case of an intracranial mycotic aneurysm after LVAD implantation that was successfully treated with trans-arterial embolization (TAE) with N-butyl 2-cyanoacrylate (NBCA) via the brachial artery. A 49-year-old man with a history of implantation of LVAD for ischemic cardiomyopathy was admitted to our institution. He had infectious endocarditis and was administered systemic antibiotics. At 3 weeks after admission, intracranial mycotic aneurysm of the left posterior parietal artery was detected during a diagnostic examination for asymptomatic intracranial hemorrhage. Anticoagulant therapy was administered to prevent thromboembolic complications of LVAD implantation. Under local anesthesia, TAE with NBCA was performed via the brachial artery because of the tortuous anatomy of the origin of the innominate artery and implant of the aortic arch. The aneurysm was completely obliterated. The patient was discharged without neurological deficits. TAE using NBCA could be an effective modality for the treatment of intracranial mycotic aneurysm after LVAD implantation.

Objective: Posterior cerebral artery (PCA) aneurysms are extremely rare and can be difficult to treat. We report successful trapping and thrombectomy of a giant thrombosed P2 segment aneurysm via a transpetrosal approach. Case Presentation: A 62-year-old woman was admitted to our hospital with a progressive left hemiparesis. Magnetic resonance imaging (MRI) showed a 30 mm mass lesion in the right ambient cistern. On vertebral angiography, the right P2 trunk was deviated medially and inferiorly, and the right posterior temporal artery (PTA) was not visualized. We diagnosed a giant thrombosed aneurysm of the right PTA. Surgery was performed via a right posterior transpetrosal approach. The proximal P2 was identified above the oculomotor nerve in the ambient cistern, and a giant PTA aneurysm was found. After coagulating the distal PCA, a temporary clip was applied to the proximal P2, the aneurysm wall was incised, thrombus was removed, and a permanent titanium clip was applied to complete trapping. Postoperative MRI showed disappearance of the aneurysm. The patient’s left hemiparesis was resolved 2 months after the operation, and she was discharged home. Conclusion: Although trans-sylvian and subtemporal approaches are often performed for P2 aneurysms, they have difficulty identifying the distal PCA and may require excessive brain retraction. The transpetrosal approach can also be effective for giant thrombosed P2 aneurysms.

Dystonia is a movement disorder that has various treatment options. For primary dystonia, stereotactic procedures such as deep brain stimulation (DBS) have demonstrated favorable outcomes. For secondary dystonia, however, the treatment outcomes remain inconclusive, and the heterogeneous etiological background is considered to contribute to the poor outcomes of the disease. Here, we report a rare pediatric case of post-stroke focal dystonia treated with conventional radiofrequency ventro-oral (Vo) thalamotomy. The patient was an 11-year-old girl with secondary focal dystonia in her right hand. The dystonia was considered to result from a stroke lesion in the putamen due to vasculitis following varicella-zoster virus infection. We hypothesized that the infarction of the putamen resulted in hyperactivity in the thalamus, and, thus, performed a radiofrequency Vo thalamotomy. Markedly decreased muscle tone in her right hand was noted immediately after surgery. However, the improvement was temporary, as her symptoms returned to baseline level by the 6-month follow-up. Although the observed improvement was temporary in this case, our findings may elucidate the possible mechanisms of secondary focal dystonia. Further studies are needed to establish an effective surgical treatment for secondary focal dystonia.

Superficial temporal artery (STA)–middle cerebral artery (MCA) bypass is the standard surgical treatment for moyamoya disease (MMD). Local cerebral hyperperfusion (CHP) is one of the potential complications, which could enhance intrinsic inflammation and oxidative stress in MMD patients and accompany concomitant watershed shift (WS) phenomenon, defined as the paradoxical decrease in the cerebral blood flow (CBF) near the site of CHP. However, CHP and simultaneous remote reversible lesion at the splenium have never been reported. A 22-year-old man with ischemic-onset MMD underwent left STA–MCA bypass. Although asymptomatic, local CHP and a paradoxical CBF decrease at the splenium were evident on N-isopropyl-p-[¹²³I] iodoamphetamine single-photon emission computed tomography 1 day after surgery. The patient was maintained under strict blood pressure control, but he subsequently developed transient delirium 4 days after surgery. MRI revealed a high-signal-intensity lesion with a low apparent diffusion coefficient at the splenium. After continued intensive management, the splenial lesion disappeared 14 days after surgery. The patient was discharged without neurological deficits. Catheter angiography 2 months later confirmed marked regression of posterior-to-anterior collaterals via the posterior pericallosal artery, suggesting dynamic watershed shift between blood flow supplies from the posterior and anterior circulation. Mild encephalitis/encephalopathy with a reversible splenial lesion could explain the pathophysiology of the postoperative splenial lesion in this case, which is associated with generation of oxidative stress, enhanced inflammation, and metabolic abnormalities. Rapid postoperative hemodynamic changes, including local CHP and concomitant WS phenomenon, might participate in the formation of the splenial lesion.

Subacute subdural hematoma (SASDH) is a neurotraumatic entity. There are few reports of chronological changes of cerebral blood flow (CBF) on arterial spin labeling (ASL) and subcortical low intensity (SCLI) on fluid-attenuated inversion recovery (FLAIR) images of magnetic resonance imaging (MRI) observations from the injury onset, deterioration, to post-surgery. We reported a SASDH patient presenting postoperative cerebral hyperperfusion (CHP) syndrome with chronological changes of those findings. An 85-year-old woman fell and presented right ASDH. She was treated conservatively due to no neurological deficits. On day 3, ASL image revealed increased CBF against brain compression. On day 7, the CBF was normalized on ASL image, but SCLI was confirmed. On day 14, SCLI was strengthened. Then she developed left hemiparesis due to brain compression by SASDH. Considering age and comorbidities, we performed endoscopic hematoma removal under local anesthesia, and her neurological deficits improved after the surgery. On postoperative day 1, she newly presented left upper limb paresis. MRI revealed increased CBF and enhanced SCLI. We diagnosed CHP syndrome, and antihypertensive treatment improved the symptoms gradually. However, SCLI had been consistently observed, and CBF easily changed depending on the blood pressure, suggesting dysfunction of the CBF autoregulation. We showed the endoscopically treated SASDH patient with CBF’s chronological changes on ASL images and SCLI on FLAIR images. Long-time brain compression would lead to dysfunction of the CBF autoregulation, and we should be careful about CHP syndrome after the endoscopic surgery for SASDH.

Infra-optic course of the anterior cerebral artery (IOA) is rare and approximately 55 cases of this anomaly have been described. We present a case of a ruptured anterior communicating artery (ACoA) aneurysm arising at the junction between the left IOA and the bilateral A2 segments, at which the right A1 segment was absent. One of the recurrent arteries of Heubner branched off directly from the aneurysmal dome, and was obstructed at aneurysmal neck clipping via an anterior interhemispheric (AIH) approach. In this report, accompanied anatomical variations and surgical approaches for ACoA aneurysms with IOA are reviewed. An IOA is frequently associated with other vascular anomalies, and the origin of functionally important recurrent arteries of Heubner is also variable. Preoperative accurate evaluation of vessel structures and the maximal exposure at surgery are very important. Pterional approach from the ipsilesional side is reportedly to be safe, but interhemispheric approach is also suggested to be effective as to full exposure to recognize the perianeurysmal anatomical structures including potential vessel anomalies.

A 57-year-old woman with a wide-necked anterior communicating artery (Acom) aneurysm underwent stent-assisted coiling (SAC) due to aneurysm enlargement. Dual antiplatelet therapy was initiated 7 days before the operation, and systemic heparinization was performed while maintaining an activated clotting time (ACT) of approximately 300 s during the procedure. SAC was performed using a laser-cut closed-cell stent and bare platinum coils. At the end of the procedure, the Acom and right anterior cerebral artery (ACA) were occluded by in-stent thrombosis. Following local intra-arterial administration of 480000 U of urokinase, the Acom and right ACA were recanalized, accompanied by extravasation around the Acom aneurysm. A computed tomography (CT) scan revealed a right frontal hematoma, which did not enlarge after the administration of protamine sulfate. The hematoma disappeared spontaneously, and the patient recovered without any neurological deficits. Local administration of urokinase is an effective treatment for in-stent thrombosis. However, because the devices for SAC may cause mechanical injuries to the aneurysms, urokinase should be used cautiously for cerebral aneurysms, even if unruptured.

In case of symptomatic varix associated with cerebral arteriovenous malformations (AVM), nidus is usually treated with transarterial embolization (TAE). However, TAE is not always possible due to inaccessible nidus. A man in his 40s presented with numbness and clumsiness in the right hand. Magnetic resonance imaging (MRI) and cerebral angiography revealed a giant varix associated with an AVM nidus in the left parietal lobe. The varix severely compressed the postcentral gyrus with edema. The main feeder was occluded, and tiny collateral vessels fed the nidus. After admission, his symptoms deteriorated rapidly due to the enlarging varix. To extirpate the varix, selective transvenous embolization (TVE) of a small compartment, the varix neck, between the varix and the main cortical drainer with coils was performed. After treatment, the thrombosed varix gradually shrank, and his symptoms improved. The transvenous coil-plugging technique is a potential strategy for symptomatic varix with a varix neck.

Large cell neuroendocrine carcinoma (LCNEC) is a rare malignant tumor that typically arises in the lungs. It is especially rare in the sinonasal cavity, and treatment has not been established. In this study, we present the case of a 56-year-old woman with a large sinonasal LCNEC that extended into her brain. We performed endonasal endoscopic and transcranial combined surgery followed by chemoradiation therapy. The combined surgery enabled us to approach and remove the extensive tumor from two different directions at one time less invasively. We have achieved good tumor control for 18 months so far.

Neurenteric cysts (NCs) are rare benign congenital neoplasms in the central nervous system that originate from endodermal elements. NCs are more commonly located in the spine than in the brain. Although almost all intracranial NCs are found in the posterior fossa, some have reported supratentorial NCs. The complete excision of the cyst wall is suggested as a curative treatment; however, endoscopic treatment is less discussed. We present a supratentorial intraparenchymal NC in the frontal lobe treated by neuroendoscopic fenestration and review the literature regarding supratentorial NCs. A 43-year-old woman presenting with right hemiparesis and gait disturbance who was found to have a huge cystic lesion with calcification in her left frontal lobe underwent endoscopic fenestration to the ipsilateral lateral ventricle and biopsy. The histopathological diagnosis was consistent with NC. Postoperatively, her right hemiparesis and gait disturbance disappeared. Postoperative MRI showed shrinkage of the cyst. She was discharged without neurological deficits and no recurrence was seen 1 year after surgery. To the best of our knowledge, there have been no reports of a supratentorial intraparenchymal NC treated by neuroendoscopic fenestration. Minimally invasive treatments, such as neuroendoscopic cyst fenestration, can be considered depending on the location of the cyst.

“Diffuse midline glioma (DMG), H3K27M-mutant” was newly classified in the revised World Health Organization (WHO) 2016 classification of central nervous system tumors. Spinal cord DMG, H3K27M-mutant is relatively rare, with poor prognosis, and there are no effective treatment protocols. In this study, we report two cases of spinal cord DMG, H3K27M-mutant treated with bevacizumab. The two patients were women in their 40s who initially presented with sensory impairment. MRI showed spinal intramedullary tumors, and each patient underwent laminectomy/laminoplasty and biopsy of the tumors. Histological examination initially suggested low-grade astrocytoma in case 1 and glioblastoma in case 2. Upon further immunohistochemical examination in case 1 and molecular examination in case 2, however, both cases were diagnosed as DMG, H3K27M-mutant. Case 1 was treated with radiation therapy and temozolomide (TMZ) chemotherapy, which induced a transient improvement of symptoms; 3 months after surgery, however, the patient’s symptoms rapidly deteriorated. MRI showed tumor enlargement with edema to the medulla. Triweekly administration of bevacizumab improved her symptoms for the following 12 months. Case 2 was treated with bevacizumab from the beginning because of acute deterioration of breathing. After bevacizumab administration, both cases showed tumor regression on MRI and drastic improvement of symptoms within a few days. Although spinal cord DMG, H3K27M-mutant has an aggressive clinical course and poor prognosis, bevacizumab administration may offer the significant clinical benefit of alleviating edema, which improves patient’s capacity for activities of daily life.

Intracranial artery occlusion due to a foreign body is a complication associated with cardiac surgery that is treated by various techniques. However, little is known about appropriate strategies for symptomatic intracranial artery stenosis due to an unknown embolic source. We reported a case of middle cerebral artery (MCA) stenosis after mitral valve repair (MVR) for infective endocarditis (IE). An 80-year-old man presented with right hemiplegia. MR angiography findings were normal, and diffusion-weighted imaging revealed subtle ischemic change in the left MCA territory. The patient was diagnosed with cardioembolic stroke owing to IE and performed MVR. Four days later, he suddenly presented with consciousness disorder and left hemiplegia. Computed tomography (CT) confirmed a very low-density area within the right MCA. MR angiography revealed right MCA stenosis, which corresponded to the low-density area on CT images. Diffusion-weighted imaging revealed new ischemic change in the right MCA territory. Angiography confirmed an irregular stenosis at the right M2 with antegrade blood flow, and the hemiplegia resolved during angiography. Conservative therapy was performed; however, the resting 123 I-IMP-single photon emission CT revealed moderate perfusion defect in the right MCA territory, and transient left hemiplegia appeared every few days. Therefore, 19 days after the initial transient ischemic attack, the patient was performed superficial temporal artery–MCA anastomosis, and the patient responded with a good clinical course without recurrence of the ischemic symptoms. This strategy may be a safe and effective treatment for symptomatic intracranial artery stenosis due to an unknown embolic source.

Clear cell meningioma (CCM) is a WHO classification Grade II meningioma. It is a very rare disease, of which only 41 cases of spinal cord CCM in children have been reported to date. CCMs sometimes do not have the “dural attachment” that is usually found in meningiomas, and our understanding of the origin of CCMs is therefore controversial. We hereby present a case of pediatric CCM of the lumbar spine, in which we examined intraoperatively, the detailed anatomical location of the tumor. The case is a 10-year-old boy, who presented to our hospital with a 2-month history of lower back and bilateral lower extremity pain upon waking, which gradually worsened. Lumbar spine CT and MRI revealed an intradural extramedullary tumor at the L3 vertebral level, and surgery was performed to remove it. The tumor was in close contact with the dura mater, and also in contact with the cauda equina via the arachnoid. The tumor was likely located primarily between the dura mater and arachnoid. The pathological diagnosis was CCM, with an MIB-1 index of less than 1%. His back pain and bilateral lower extremity pain improved after surgery, and he was discharged from our hospital. Postoperative radiation therapy was not performed. Based on this case, we suggest that intraoperative examination of the anatomical location of these tumors and accumulation of relevant experience are important to elucidate the embryological mechanisms of this rare disease.

Intracranial dermoid cysts are rare congenital lesions that result from abnormal sequestration of ectodermal cells during neural tube formation. These tumors are especially rare in lateral areas such as in the temporal lobe. In this study, we report a case of dermoid cyst located in the right temporal lobe. A 50-year-old man was referred for further treatment of a tumor. CT revealed a low-density mass lesion in the right temporal lobe, with calcification. MRI showed the lesion with high signal intensity on diffusion-weighted imaging, high-low mixed signal intensity on T1-weighted imaging, and iso-high signal mixed intensity on T2-weighted imaging; the capsule was enhanced with gadolinium. Differential diagnosis included dermoid cyst, epidermoid cyst, teratoma, and neurenteric cyst. We decided to perform surgery for the improvement of his symptom, histopathological diagnosis, and radical cure. A right temporal craniotomy was performed, and the tumor was found adherent to the surrounding brain tissue. The tumor was completely removed under subpial dissection. Hair was confirmed in the tumor content. On histopathology, the cyst wall was lined with stratified squamous epithelium, sebaceous glands, small vessel aggregates, and inflammatory infiltrate. Keratinized material and hair were found in the lumen. The patient was discharged 7 days after surgery with no new neurologic deficits. This case was unusual in terms of the effect of gadolinium enhancement on MRI, and the presence of adipose tissue and calcification were useful for diagnosis. It is vital to consider prevention of chemical meningitis due to intrathecal dissemination of the tumor content intraoperatively.

Corpus callosum swelling has been reported to occur after ventriculoperitoneal shunting for long-standing hydrocephalus. This report presents a case of corpus callosum swelling after intraventricular tumor resection. A 34-year-old woman presented with a headache that worsened over 1 month. Magnetic resonance (MR) images revealed a mass lesion in the left lateral ventricle and obstructive hydrocephalus. She underwent subtotal resection with a transcallosal approach. After tumor resection, she had long-lasting status epilepticus followed by consciousness disturbance. T2-weighted MR images obtained 8 hr after the operation showed a hyperintense area in the corpus callosum. The patient then presented with bilateral dilated pupils 14 hr after the operation due to acute hydrocephalus and tension pneumocephalus. An emergent re-craniotomy was performed and a ventricular drain was placed. The patient recovered consciousness 3 days after the operation. However, she experienced progressive corpus callosum swelling 25 days after the operation, which improved since then. Approximately 4 months after the operation, she returned to her usual workplace with no neurocognitive functional decline. Two years later, she was doing well with no radiological abnormal findings except corpus callosum thinning. Thus, corpus callosum swelling can develop not only after shunting for chronic hydrocephalus but also after intraventricular tumor resection. It occurred relatively acutely and there was no decline in intelligence after long-term follow-up. This case suggests that corpus callosum swelling after intraventricular tumor resection is a rare but noteworthy complication that can improve without intervention.

This report describes a 49-year-old male patient who presented with a pituitary adenoma extending to the suprasellar region. Subarachnoid hemorrhage (SAH) occurred after conventional transnasal transsphenoidal surgery for a non-functioning pituitary adenoma despite no suprasellar arachnoid membrane breakdown. Through extended transsphenoidal route, the suprasellar hematoma was removed and bleeding from a small vessel thought to be the branch of left superior hypophyseal artery was successfully controlled. Indeed, several case reports regarding this rare complication have been published, but the mechanism of SAH has never been identified and the prognosis was poor in most cases. This report illustrates the origin and mechanism of the bleeding clearly using the intraoperative video. This case suggests that immediate therapeutic intervention is necessary and extended transnasal transsphenoidal repeat surgery is useful for an appropriate hemostasis and removal of hematoma located in the suprasellar region.

Trigeminal neuralgia (TN) is characterized by lightning pain paroxysms in the somatosensory distribution of the trigeminal nerve accompanied by hypersensitivity to non-nociceptive stimuli. Epidermoid cysts sometimes cause TN. To plan the surgery, constructive interference in steady state (CISS) image is useful for understanding the tumor’s location, extent, and relationship against the cranial nerves, and epidermoid cysts are shown as hypointense compared to cerebrospinal fluid (CSF). However, we herein describe a case with TN due to epidermoid cysts, whose intraoperative findings are different from the preoperative and postoperative CISS image. A 49-year-old woman has suffered from TN. CISS images revealed the prolonged trigeminal nerve and the hypointense tumor compared to the CSF at the right cerebellopontine angle. CISS image suggested that the tumor would surround the trigeminal nerve, reach into the Meckel cavity, and offend and compress the trigeminal nerve’s root entry zone (REZ). However, contrary to our expectation, the trigeminal nerve was not surrounded by the tumor. Neuroendoscope revealed that the tumor compressed the REZ, but the tumor was not present in the Meckel cavity. We performed partial tumor removal around the trigeminal nerve, and her symptoms improved. However, the postoperative CISS image was similar to the preoperative one, and so we could not evaluate the remaining tumor. The pathological diagnosis was epidermoid cysts. Intraoperative findings are sometimes different from the pre- and postoperative CISS images, making it difficult to follow up the remaining part of the epidermoid cyst.

In cases of a dural arteriovenous fistula (AVF) with a pial arterial supply, postoperative hemorrhagic complications occur frequently. Six cases in which patients were diagnosed with a coexisting dural AVF and pial arteriovenous malformation (AVM) sharing a common drainer are presented. These cases were initially thought to be dural AVFs with pial arterial supplies, but careful examination of preoperative images showed that a pial AVM coexisted near the dural AVF, and that both shared a common drainer. The coexistence of a pial AVM is difficult to notice during surgery; for this reason, determining the presence of a pial AVM on preoperative imaging is essential to safely treat a dural AVF with a pial arterial supply. The details of each case, specifically, the diagnostic evidence for this condition (coexisting dural AVF and pial AVM sharing a common drainer), as well as imaging findings that should be noted, are presented.

Mismatch repair (MMR) gene deficiency is rarely observed in gliomas, a constitutional defect is associated with tumorigenesis in Lynch syndrome, and an acquired defect is associated with hypermutation after temozolomide treatment. However, the meaning of MMR gene deficiency in gliomas is unclear. Two cases of MMR-deficient glioblastomas are reported, and mutational status of oncogenes was compared between primary and recurrent tumor samples in a glioblastoma patient with Lynch syndrome. Additionally, the characteristics of MMR-deficient glioblastomas were analyzed using public glioma datasets to determine the meaning of MMR deficiency in gliomas. Case 1 was a glioblastoma patient with Lynch syndrome, and treatment with pembrolizumab for the recurrent tumor was temporarily effective for a short period. Comparison of mutational changes between primary and recurrent tumor samples showed many additional mutated genes associated with multiple signaling pathways in the recurrent tumor. Tumor recurrence and chemoresistance could be associated with intratumoral heterogeneity and accelerated tumor progression due to defects of multiple signaling pathways. Case 2 was a glioblastoma patient with acquired MMR gene deficiency, and she died of rapid progression of bone marrow metastases. This rare clinical course was considered to be associated with gene expression changes and heterogeneity that resulted from MMR gene deficiency. Two cases of MMR gene-deficient glioblastomas were presented, and their genetic characteristics suggested that their clinical courses could be associated with MMR gene deficiency.

Coagulation factor XIII (F13) deficiency has been known to be a rare disease with estimated one per two million and one of the possible reasons of postoperative hemorrhage; however, it still remains unpenetrated to physicians. We report a case of acute ventriculoperitoneal (VP) shunt dysfunction due to delayed intraventricular hemorrhage, which could be because of F13 deficiency. The patient was a 48-year-old man with a history of post-meningitis hydrocephalus followed by VP shunt placement. He was found unconscious and transferred to our hospital. A brain CT scan demonstrated shunt malfunction, and he underwent emergency shunt revision. The postoperative course was uneventful except for unexpected neck bruises and continuous minor bleeding from the surgical wound. Three days after surgery, he suddenly became comatose and a CT scan revealed the recurrence of hydrocephalus with newly identified small volume of intraventricular hemorrhage. Emergency shunt revision was performed again. The shunt valve was filled with a hematoma and bloody cerebrospinal fluid was drained from the ventricle. Postoperative blood sample examination demonstrated no abnormal findings but a decreased level of F13 activity, which was thought to be a possible cause of postoperative hemorrhage and the shunt valve hematoma. F13 deficiency causes delayed intracranial hemorrhage 24–48 h after neurological surgery. It can only be diagnosed by checking F13 activity with suspicion. If diagnosed accurately beforehand, unexpected postoperative bleeding can be preventable with proper treatment, such as F13 concentrate and cryoprecipitate. The actual number of the patient with F13 deficiency may be more than estimated ever.

Although it is increasingly recognized that coronavirus disease 2019 (COVID-19) can cause multi-organ disease, including acute ischemic stroke, the incidence of coagulation disorder is reported to be lower in Asian countries. We report a case of a 47-year-old Asian man with mild COVID-19 respiratory symptoms who had acute basilar artery occlusion. Despite successful recanalization with mechanical thrombectomy, the patient developed extensive cerebral infarction in the posterior circulation, necessitating decompressive craniotomy. Our findings suggest that severe large vessel occlusion (LVO) can occur even in young Asian patients with mild COVID-19 respiratory symptoms and that its outcome can be extremely severe despite successful recanalization. The management of COVID-19-related LVO can be very challenging, as both the prevention of possible nosocomial infection and early recanalization are required simultaneously.

Non-specific low back pain in athletes can be caused by discogenic back pain, Modic type 1 change, and facet joint arthritis. In this report, we describe a full-endoscopic surgical strategy that we have used to treat a patient with both discogenic pain and Modic type 1 change. The patient was a 32-year-old professional baseball player who played an infield position and had a 2-year history of low back pain. Three years earlier, he had undergone micro-endoscopic discectomy for left herniated nucleus pulposus at L5/S1. His leg symptoms resolved postoperatively, and he returned to playing baseball the following season. However, his low back pain gradually increased. Two years after the initial surgery, he was experiencing low back pain in daily life and found it very difficult to play baseball. Short T1 inversion recovery (STIR) magnetic resonance imaging (MRI) revealed Modic type 1 change and high-signal intensity zones in degenerated discs at L4/5 and L5/S1. Injection of xylocaine 1% reduced the pain temporarily, confirming that the pain generator was at L4/5 and L5/S1. The pathological diagnosis was discogenic pain with Modic type 1 change. We performed full-endoscopic disc cleaning (FEDC) surgery for the Modic type 1 change and thermal annuloplasty (TA) for the discogenic pain at these levels. The patient’s low back pain decreased steadily thereafter. Six months after surgery, he returned to baseball, playing for a full season without pain. We have successfully treated a professional baseball player with discogenic pain and Modic type 1 change by full-endoscopic surgery.

Carotid endarterectomy (CEA) is an established surgical procedure for carotid stenosis. We present the case of a 74-year-old man who underwent CEA for symptomatic internal carotid artery (ICA) stenosis. During the operation, we found that the hyoid bone (HB) and the superior horn of the thyroid cartilage covered the carotid sheath, preventing adequate visualization. Since this was anticipated based on preoperative examinations, the superior horn of thyroid cartilage was removed with the help of an otorhinolaryngologist. The HB was preserved because it could be retracted, and thereafter we performed CEA. Although CEA is a common procedure, the HB and thyroid cartilage are rarely involved in the operation. We present a case report including literature review of acute cerebrovascular syndrome (ACVS) due to HB and thyroid cartilage obstruction.

Intradural extramedullary (IDEM) ependymoma except for tumors originated from the filum terminale or conus medullaris is rare. The present study showed a case of IDEM ependymoma. A 16-year-old boy was referred to our hospital with a complaint of right hypochondriac pain and motor weakness in his right leg. MRI revealed a solitary intradural tumor at Th5–8 level with syringomyelia at Th2–4 level. Microscopic total tumor resection was performed with right hemi-laminectomy of Th4–9. Histological diagnosis was ependymoma (WHO grade 2). Although his leg weakness was worsened transiently, he showed improvement in leg weakness being able to go up and down the stairs 1 month after the surgery. There was no tumor recurrence until now, 7 years after the surgery, without any adjunctive therapies. A total of 44 cases of IDEM ependymoma had been reported in the past literatures. They are thought to arise from ependymal cells which remained during the process of neural tube closure. Like intramedullary ependymomas, most of the IDEM ependymomas have clear border to surrounding tissue and often removed completely. However, a small number of recurrences and malignant transformations had been reported after complete resections despite benign histological features tumors. In the case of totally resected low grade IDEM ependymoma, it is thought to be reasonable to perform long-term periodical radiographic follow-up without postoperative adjunctive therapy.

Neuroendocrine tumors (NETs) are neoplasms that originate from cells of the endocrine and nervous systems, and are commonly found in the gastrointestinal and respiratory tracts. Primary intracranial NETs are extremely rare and have been the focus of only a few studies thus far. Herein, we report the case of a primary intracranial NET of the skull base complicated with tension pneumocephalus after radiotherapy. An 84-year-old woman visited a local hospital for a head injury, and CT revealed a skull base tumor. MRI showed that the tumor was located mainly on the clivus and extended into the paranasal sinuses and nasal cavity. We biopsied the tumor via the nasal cavity, and the pathological diagnosis was NET, WHO grade 2. We subsequently administered focal intensity-modulated radiation therapy, but the patient developed tension pneumocephalus 1 year after radiotherapy. We therefore performed endoscopic transnasal cerebrospinal fluid leak closure with a nasoseptal flap. The postoperative course was successful, and the patient returned home but died of an unknown cause 2 years after discharge. The optimal postoperative management of primary intracranial NETs remains controversial. Tension pneumocephalus related to radiotherapy is a rare complication. Assessing skull bone erosion before radiotherapy and performing regular radiological follow-up examinations are essential to prevent this rare complication.

Brainstem venous congestion due to dural arteriovenous fistula (dAVF) can mimic brainstem glioma and infarction. We report a case of a 56-year-old woman with a transverse-sigmoid sinus (TS) dAVF. On MRI, she presented with brainstem edema that was difficult to distinguish from brainstem glioma and infarction. She was referred to our hospital for mild dysarthria with right hemiparesis and a suspected left pontine glioma. On MRI, contrast enhancement of the lesion was demarcated by the pontine raphe, and the ipsilateral vein of Rosenthal was dilated. Cerebral angiography revealed TS dAVF with an isolated sinus. Transarterial followed by transvenous coil embolization was performed to reduce shunt flow, resulting in symptom improvement and normal findings on MRI and cerebral angiography. Brainstem venous congestion due to TS dAVF is as rare as adult brainstem glioma. Differentiating the above-mentioned three diseases on the basis of diagnostic imaging findings and clinical course is necessary for appropriate and timely treatment.

Stroke associated with pregnancy or puerperium is rare, but represents an important problem for women of childbearing age. We herein report a case of subarachnoid hemorrhage (SAH) due to suspected lateral posterior choroidal artery (LPChA) dissection during delivery. A 34-year-old woman developed deterioration of consciousness during delivery at a maternity clinic. Her Glasgow Coma Scale score was E3V3M6. She was sent to our hospital after 12 hr, where CT revealed SAH with intraventricular hematoma. Radiographic examinations showed contrast pooling on the left LPChA. Repeated angiography showed enlargement of the contrast pooling, which indicated pseudoaneurysm. It also showed a relatively clear but stenosed LPChA communicated with the lesion which could not be recognized in the angiography on day 0. This stenosed LPChA indicated arterial dissection. Therefore, endovascular parent artery occlusion was performed on day 11. Determining the exact extent of dissection was difficult because the LPChA was extremely narrow. Occlusion of the posterior cerebral artery was needed to achieve complete hemostasis, which, however, resulted in infarction of the medial temporal and occipital lobes. At the time of final follow-up 3 years later, the patient was alert and completely independent, but showed persistent incomplete homonymous hemianopsia. We reported a rare case of SAH from suspected LPChA dissection during delivery. Repeated angiography provided information about the source of hemorrhage and definite diagnosis, which opened the way to treatment. It is also important to recognize the difficulty in identifying the exact extent of dissection when treating dissections of small arteries.

Nonocclusive mesenteric ischemia (NOMI) is a rare but life-threatening post-stroke complication. This is the first case series report of NOMI after stroke, describing its characteristics and the difficulty of diagnosis. We retrospectively reviewed 367 stroke patients from April 2018 to May 2019 in our hospital. We identified six patients (1.6%) with NOMI after stroke and described their clinical presentation, including characteristics, vital signs, laboratory parameters, treatment, and outcomes. The mean interval between stroke onset and diagnosis of NOMI was 4.6 days (range, 3–10 days). Five patients had disturbance of consciousness caused by stroke, and therefore the major complaints and symptoms associated with NOMI were nonspecific, possibly resulting in delayed diagnosis and treatment. All patients had a high respiratory rate (≥22 breaths/min). C-reactive protein and lactate levels were high (mean, 21.6 mg/L and 2.4 mmol/L, respectively). All patients underwent emergent abdominal operations. Four patients were discharged with modified Rankin Scale scores ≥4, and two patients died. NOMI can be a fatal post-stroke complication and is challenging to diagnose. Further investigations should be conducted to determine the most efficient way to diagnose NOMI after stroke.

Neurosyphilis is an infection of the central nervous system by Treponema pallidum. Gummatous neurosyphilis, especially spinal syphilitic gumma, is an exceedingly rare manifestation and may be misdiagnosed as other tumors due to its rarity. A 42-year-old man with a medical history of treatment for syphilis presented with rapidly progressive leg paralysis, leg sensory disturbance, and bladder and rectal disturbance. Spinal MRI demonstrated an intradural extramedullary lesion strongly compressing the spinal cord at the T6/7 level, which was accompanied with dural tail sign and perilesional meningeal thickening at the T6–T8 levels. Small intradural extramedullary lesions were also detected at the T1 and T8 levels. Serological and cerebrospinal fluid examinations for syphilis were both positive. In the treatment of spinal syphilitic gumma, the decompression of the spinal cord by lesionectomy followed by postoperative antibiotic treatment is considered to be an optimal procedure in patients with rapid progression of neurological deterioration. In the present case, the symptomatic main lesion that was compressing the thoracic cord was excised by surgery and analyzed by histopathological examination, and another small asymptomatic lesion was resolved by postoperative antibiotic treatment. Spinal syphilitic gumma was diagnosed using both histopathological findings of the surgically resected lesion and another residual lesion that was resolved by postoperative antibiotic treatment.

Although the synchronous occurrence of testicular seminoma and systemic sarcoidosis has been reported, that of intracranial germinoma and systemic sarcoidosis is unknown. A 26-year-old man presented with symptoms of panhypopituitarism and consciousness disturbance. Imaging demonstrated a large nodule in the upper right lung field and swelling of multiple bilateral pulmonary and mediastinal lymph nodes in addition to the bifocal pineal and suprasellar tumors with obstructive hydrocephalus. The pathological diagnosis of the intracranial bifocal tumors was pure germinoma, whereas that of the mediastinal lymph nodes was epithelioid granuloma. Three courses of chemotherapy using carboplatin and etoposide were administered, followed by whole ventricle irradiation. The intracranial tumors completely disappeared, but the lung nodule and mediastinal lymph nodes progressed. Whole-body fluorine-18-fluorodeoxyglucose positron emission tomography demonstrated accumulation in the mediastinal lymphadenopathy, lung masses, and multiple lymph nodes of the whole body. Transbronchial lung biopsy revealed epithelioid granuloma with multinucleated giant cells. In conjunction with the high blood concentration of angiotensin-converting enzyme and soluble interleukin-2 receptor, these findings established a diagnosis of sarcoidosis. This is the first report of synchronous occurrence of intracranial germinoma and sarcoidosis. Such coexistence is extremely rare, but we should mind that sarcoidosis can occur with intracranial germinoma.

A 57-year-old woman who presented with a sudden severe headache and subsequently diagnosed with a subarachnoid hemorrhage was referred to our hospital. CTA revealed a large irregularly shaped dissecting aneurysm of the right vertebral artery (VA) and a small round distal aneurysm of the right posterior inferior cerebellar artery (PICA), which originated just proximal to the VA aneurysm. We diagnosed the VA aneurysm as a ruptured aneurysm and performed endovascular treatment the day the patient was hospitalized. Because the left VA was aplastic, stent-assisted coiling was performed to preserve the blood flow of the right VA, following the administration of antiplatelets and heparin. The proximal part of the laser-cut closed-cell stent jailed the orifice of the right PICA. The postoperative course was uneventful, and she was discharged from the hospital 3 weeks later without any neurological deficits. The follow-up VA angiogram revealed complete disappearance of the distal PICA aneurysm 17 months after stent jailing. Flow reduction by stent jailing of the arterial orifice is considered to be the main mechanism of aneurysmal shrinkage. Jailing of the orifice of the cerebral artery using even a low-metal-coverage stent may cause arterial flow reduction, which may shrink a distal aneurysm.

Double pituitary adenomas (DPAs), especially metachronous DPAs, are extremely rare and there has been no report about DPAs with altered transcriptional factors. We describe the case of a 25-year-old man who presented with acromegaly 7 years after surgery for a non-functioning pituitary adenoma (NFPA). Before the initial surgery, endocrine evaluation confirmed NFPA or silent somatotroph pituitary adenoma (SPA) because of normal serum levels of insulin-like growth factor-1 (IGF-1) and insufficient suppression of growth hormone (GH) levels in the oral glucose tolerance test (OGTT). Immunohistochemistry of resected tissue obtained from gross total resection (GTR) with transsphenoidal surgery (TSS) was negative for follicle-stimulating hormone, luteinizing hormone, GH, and Pit-1 but positive for GATA3, which confirmed the gonadotroph pituitary adenoma (GPA) diagnosis. Seven years later, follow-up brain MRI revealed a 13.3 × 5.6 × 4.7 mm tumor within the sellar turcica. The endocrine evaluation confirmed acromegaly because of high serum levels of IGF-1 and insufficient suppression of GH levels upon OGTT. GTR with TSS was again performed, and immunohistochemistry was negative for GATA3 but positive for GH and Pit-1. Surprisingly, he showed altered transcription factor expressions between initial and recurrent surgery. Based on the overall clinical course and hormonal secretion findings, we speculated metachronous development of a DPA, i.e., SPA followed by GPA, wherein a few remaining cells of the SPA might have regrown after the initial surgery. We conducted a literature review of cases that documented altered hormone secretion at recurrence and emphasized the necessity of identifying a small adenoma when there is a discrepancy between pathological findings and hormone secretion tests.

A 50-year-old man and a 69-year-old woman with consciousness disturbance were diagnosed to have suffered from subarachnoid hemorrhage (SAH) involving the posterior fossa. In both cases, the initial 3D CT angiogram failed to reveal the SAH source in the vertebrobasilar system. Delayed 3D rotational angiography revealed aneurysms on unfamiliar aberrant arteries. One was a dissecting aneurysm located between the proximal part of the posterior inferior cerebellar artery and the distal part of the intracranial vertebral artery. It was trapped and resected; the patient subsequently presented with lateral medullary symptoms. The other aneurysm was between the distal posterior and the distal anterior inferior cerebellar artery. It was successfully embolized; there were no complications. We think that the aberrant aneurysm-harboring vessels encountered in these two patients were primitive arteries on or adjacent to the vertebrobasilar paramedian longitudinal axis and that they persisted past the embryologic stage. Such aneurysms arising from unfamiliar persistent arteries beside the brainstem are extremely rare but must be considered when the SAH source is not detected in the trunk of the vertebrobasilar system.