Ulceration location is associated with clinical course in carotid stenosis.

OBJECTIVE: Plaque ulceration in carotid artery stenosis is a risk factor for cerebral ischemic events; however, the characteristics that determine plaque vulnerability are not fully understood. We thus assessed the association between plaque ulceration sites and cerebrovascular ischemic attack. METHODS: We retrospectively collected the clinical data of 72 consecutive patients diagnosed with carotid artery stenosis with plaque ulcers. After excluding patients with pseudo-occlusion, a history of previous carotid endarterectomy or carotid artery stenting before the ulcer was first discovered, follow-up data of less than 1 month, or carotid endarterectomy or carotid artery stenting performed within 1 month after the ulcer was first discovered, 60 patients were ultimately included. Patients were divided into proximal and distal groups based on the ulcer location relative to the most stenotic point. The primary endpoints were ipsilateral cerebrovascular ischemic events ("ischemic events"), such as amaurosis fugax, transient ischemic attack, or ischemic stroke due to carotid artery stenosis with plaque ulceration. The association between ulcer location and ischemic events was also assessed. RESULTS: In the patients with plaque ulcer, more patients had proximal than distal plaque ulcers (39 vs 21; P = .028). The median follow-up duration was 3.8 years (interquartile range, 1.5-6.2 years). Nineteen patients (32%) experienced ischemic event. Ischemic events occurred more frequently in the distal than in the proximal group (18% vs 59%; P = .005). Kaplan-Meier curves demonstrated a significantly shorter event-free time in the distal group (log-rank P = .021). In univariate analysis, distal ulcer location was associated with ischemic events (odds ratio [OR], 2.94; 95% confidence interval [CI], 1.13-7.65; P = .03). Multivariate analysis using two different models also showed that distal ulcer location was independently associated with ischemic events (Model 1: OR, 3.85; 95% CI, 1.26-11.78; P = .03; Model 2: OR, 4.31; 95% CI, 1.49-12.49; P = .009). CONCLUSIONS: Patients with carotid artery stenosis and plaque ulcers located distal to the most stenotic point are more likely to experience cerebrovascular ischemic attacks. Therefore, carotid plaques with ulcers located distal to the most stenotic point may be a potential indication for surgical treatment.

Irreversible postoperative cognitive impairment after unruptured intracranial aneurysm treatment in the elderly.

PURPOSE: Postoperative cognitive dysfunction and recovery remain unclear in older patients undergoing interventional therapies for unruptured intracranial aneurysms (UIAs). This study aimed to compare changes in postoperative cognitive function between younger and older patients and to detect factors associated with non-recovery from postoperative cognitive dysfunction. METHODS: This study reviewed 59 consecutive patients with UIAs who underwent interventional therapies, including microsurgical clipping or endovascular treatment, from 2021 to 2022. All patients were divided into the older (aged ≥ 70 years) and younger (aged < 70 years) groups. Mini-Mental State Examination (MMSE) and Frontal Assessment Battery (FAB) were performed within 2 months before interventions, at 1 week postoperatively (POW1), and 3-6 months postoperatively (POM3-6). RESULTS: MMSE and FAB scores decreased more frequently in the older group than in the younger group at POW1 (older vs. younger: MMSE: 48% vs. 21%, p < 0.05; FAB: 56% vs. 18%, p < 0.01). In the older group, the FAB Z-score decreased in POW1 and recovered by POM3-6 (p < 0.01), while the MMSE Z-score continued to decrease (p = 0.04). Age and the preoperative MSME Z-score were significantly associated with non-recovery from decreased MMSE score at POM3-6 (recovery vs. non-recovery, age: 62 years old vs. 72 years old, p = 0.03, preoperative MMSE Z-score: 0.16 vs. - 0.90, p < 0.01). CONCLUSIONS: This retrospective study found that older patients were more likely to have a postoperative cognitive decline after UIA treatment and implicated that global cognitive function tended to decline more than executive function in the long term. In addition, this study demonstrated that lower preoperative cognitive function was associated with inadequate postoperative cognitive recovery. The findings potentially contribute to the establishment of indications for treating UIAs in older patients.

Pathological changes in the lenticulostriate artery indicate the mechanisms leading to intracranial hemorrhage in Moyamoya disease: a case report.

This case report details the pathological findings of a vessel wall identified as the bleeding point for intracranial hemorrhage associated with Moyamoya disease. A 29-year-old woman experienced intracranial hemorrhage unrelated to hyperperfusion following superficial temporal artery-middle cerebral artery bypass surgery. A pseudoaneurysm on the lenticulostriate artery (LSA) was identified as the causative vessel and subsequently excised. Examination of the excised pseudoaneurysm revealed a fragment of the LSA, with a disrupted internal elastic lamina and media degeneration. These pathological findings in a perforating artery, akin to the circle of Willis, provide insights into the underlying mechanisms of hemorrhage in Moyamoya disease.

Impact of tailored multimodal treatment for unruptured brain arteriovenous malformation: comparison with a randomized trial of unruptured brain arteriovenous malformations.

PURPOSE: The first randomized controlled study on unruptured brain arteriovenous malformations (bAVM), the ARUBA trial, demonstrate the superiority of medical management; however, it failed to completely rule out the efficacy of therapeutic interventions due to several limitations. This study aimed to examine the outcomes of multimodal interventional treatment for bAVM in terms of safety and efficacy. METHODS: We reviewed 226 consecutive patients with unruptured bAVM admitted to our institute between 2002 and 2022. Treatment methods were divided into medical management and therapeutic intervention, including microsurgery, stereotactic surgery, and endovascular intervention. First, the choice of therapeutic modalities was assessed in the pre-ARUBA (before February 2014) and post-ARUBA (after March 2014) eras. Second, the incidence of symptomatic stroke or death and functional prognosis with a modified Rankin scale (mRS) score of ≥2 at 5 years was compared between the medical management and therapeutic intervention. RESULTS: In the pre- and post-ARUBA groups, 73% and 84% of patients underwent therapeutic interventions, respectively (p = 0.053). The rate of symptomatic stroke or death was lower in patients who underwent interventional therapies than in those who underwent medical management (9.7% vs. 22%, p = 0.022); however, the opposite was observed in the ARUBA trial (31% vs. 10%). The annual incidence of stroke or death was also lower in the interventional therapy group (4.3%/y vs. 1.8%/year, hazard ratio = 0.45, 95% confidence interval: 0.18-1.08, p = 0.032). The rate of mRS score of ≥2 after a 5-year follow-up was 18% and 6% in the medical treatment and intervention groups (p = 0.14). CONCLUSIONS: The therapeutic intervention rate did not decrease, even after the publication of the ARUBA trial. The rate of stroke or death was lower in the intervention group, indicating that a tailored choice of multimodality is safe and effective for managing unruptured bAVM.

[Changes in Gamma Knife Radiosurgery(GKRS)in Patients with Metastatic Brain Tumors before and after COVID-19].

The spread of coronavirus disease 2019(COVID-19)is a concern as it may delay the detection of malignant tumors due to delayed medical checkups. We examined changes in the treatment of metastatic brain tumors before and after COVID- 19. A retrospective review of 211 patients with metastatic brain tumors who underwent initial gamma knife radiosurgery between July 2019 and December 2021 was conducted. Data collected include patient age, gender, the Karnofsky performance status(KPS), primary tumor control, number, total volume, and outcome during the COVID-19 emergency declaration period and outside of it. The patient number was 164 outside of the emergency period and 47 during the period. Symptomatic cases(KPS<90)and poor control of the primary site increased during the COVID-19 period. The treatment number and volume of brain metastasis did not change. Metastatic control after 4 months of treatment also showed no difference. The number of symptomatic patients increased during the emergency declaration period, suggesting that COVID- 19 may have reduced the rate of asymptomatic patients being seen. However, these were not enough to affect the prognosis at 4 months. Overall, the COVID-19 pandemic had a small impact on the provision of stereotactic radiotherapy for metastatic brain tumors.

Quantitative measurement of peritumoral concentrations of glutamate, N-acetyl aspartate, and lactate on magnetic resonance spectroscopy predicts glioblastoma-related refractory epilepsy.

BACKGROUND: Increased extracellular glutamate is known to cause epileptic seizures in patients with glioblastoma (GBM). However, predicting whether the seizure will be refractory is difficult. The present study investigated whether evaluation of the levels of various metabolites, including glutamate, can predict the occurrence of refractory seizure in GBM by quantitative measurement of metabolite concentrations on magnetic resonance spectroscopy (MRS). METHODS: Forty patients were treated according to the same treatment protocol for primary GBM at Ehime University Hospital between April 2017 and July 2021. Of these patients, 23 underwent MRS to determine concentrations of metabolites, including glutamate, N-acetylaspartate, creatine, and lactate, in the tumor periphery by applying LC-Model. The concentration of each metabolite was expressed as a ratio to creatine concentration. Patients were divided into three groups: Type A, patients with no seizures; Type B, patients with seizures that disappeared after treatment; and Type C, patients with seizures that remained unrelieved or appeared after treatment (refractory seizures). Relationships between concentrations of metabolites and seizure types were investigated. RESULTS: In 23 GBMs, seizures were confirmed in 11 patients, including Type B in four and Type C in seven. Patients with epilepsy (Type B or C) showed significantly higher glutamate and N-acetylaspartate values than did non-epilepsy patients (Type A) (p < 0.05). No significant differences in glutamate or N-acetylaspartate levels were seen between Types B and C. Conversely, Type C showed significantly higher concentrations of lactate than did Type B (p = 0.001). Cutoff values of lactate-to-creatine, glutamate-to-creatine, and N-acetylaspartate-to-creatine ratios for refractory seizure were > 1.25, > 1.09, and > 0.88, respectively. CONCLUSIONS: Extracellular concentrations of glutamate, N-acetylaspartate, and lactate in the tumor periphery were significantly elevated in patients with GBM with refractory seizures. Measurement of these metabolites on MRS may predict refractory epilepsy in such patients and could be an indicator for continuing the use of antiepileptic drugs.

Met-PET uptake index for total tumor resection: identification of 11C-methionine uptake index as a goal for total tumor resection including infiltrating tumor cells in glioblastoma.

Glioblastoma multiforme (GBM) is largely due to glioma stem cells (GSCs) that escape from total resection of gadolinium (Gd)-enhanced tumor on MRI. The aim of this study is to identify the imaging requirements for maximum resection of GBM with infiltrating GSCs. We investigated the relationship of tumor imaging volume between MRI and 11C-methionine (Met)-PET and also the relationship between Met uptake index and tumor activity. In ten patients, tumor-to-contralateral normal brain tissue ratio (TNR) was calculated to evaluate metabolic activity of Met uptake areas which were divided into five subareas by the degrees of TNR. In each GBM, tumor tissue was obtained from subareas showing the positive Met uptake. Immunohistochemistry was performed to examine the tumor proliferative activity and existence of GSCs. In all patients, the volume of Met uptake area at TNR ≦ 1.4 was larger than that of the Gd-enhanced area. The Met uptake area at TNR 1.4 beyond the Gd-enhanced tumor was much wider in high invasiveness-type GBMs than in those of low invasiveness type, and survival was much shorter in the former than the latter types. Immunohistochemistry revealed the existence of GSCs in the area showing Met uptake at TNR 1.4 and no Gd enhancement. Areas at TNR > 1.4 included active tumor cells with relatively high Ki-67 labeling index. In addition, it was demonstrated that GSCs could exist beyond the border of Gd-enhanced tumor. Therefore, to obtain maximum resection of GBMs, including infiltrating GSCs, aggressive surgical excision that includes the Met-positive area at TNR 1.4 should be considered.

Tricks and traps of ICG endoscopy for effectively applying endoscopic transsphenoidal surgery to pituitary adenoma.

Differentiating tumor from normal pituitary gland is very important for achieving complete resection without complications in endoscopic endonasal transsphenoidal surgery (ETSS) for pituitary adenoma. To facilitate such surgery, we investigated the utility of indocyanine green (ICG) fluorescence endoscopy as a tool in ETSS. Twenty-four patients with pituitary adenoma were enrolled in the study and underwent ETSS using ICG endoscopy. After administering 12.5 mg of ICG twice an operation with an interval > 30 min, times from ICG administration to appearance of fluorescence on vital structures besides the tumor were measured. ICG endoscopy identified vital structures by the phasic appearance of fluorescent signals emitted at specific consecutive elapsed times. Elapsed times for internal carotid arteries did not differ according to tumor size. Conversely, as tumor size increased, elapsed times for normal pituitary gland were prolonged but those for the tumor were reduced. ICG endoscopy revealed a clear boundary between tumors and normal pituitary gland and enabled confirmation of no more tumor. ICG endoscopy could provide a useful tool for differentiating tumor from normal pituitary gland by evaluating elapsed times to fluorescence in each structure. This method enabled identification of the boundary between tumor and normal pituitary gland under conditions of a low-fluorescence background, resulting in complete tumor resection with ETSS. ICG endoscopy will contribute to improve the resection rate while preserving endocrinological functions in ETSS for pituitary adenoma.

[A Case of Right Frontal Dermoid Cyst with Temporal Imaging Changes in the Desease Course].

Dermoid cysts are rare benign intracranial neoplasms derived from embryonal remnant tissues. Here, we describe a case of dermoid cyst located in the right frontal lobe, which showed repeated changes on CT. An 11-year-old girl was referred to our hospital to treat a brain neoplasm. Brain CT and MRI revealed a cystic tumor in the right frontal lobe. Incidentally, brain CT had been performed 6, 2, and 1 year before the presentation, which demonstrated repeated changes in the tumor over the clinical course. Gross total resection of the tumor was achieved through right frontal craniotomy. Histological findings revealed keratin flakes, mature bones, cholesterol crystals, and granulation with macrophages. The cyst wall was composed of squamous epithelium with adnexal structures, such as hair follicles and sebaceous glands. Therefore, we diagnosed the tumor as a dermoid cyst. The postoperative course was uneventful, and she was discharged on postoperative day 10 without neurological deficits. Dermoid cysts are difficult to be diagnosed on routine neuroimaging. An accurate diagnosis requires details of the clinical course and analysis of both imaging and pathological studies.

Significance of human chorionic gonadotropin as a predictor of resistance to standard chemo-radiotherapy for pure germinoma.

Intracranial pure germinomas in children generally respond well to standard chemo-radiotherapy. However, some patients are refractory to standard therapy and require additional treatment. To investigate the characteristics of this subgroup, we retrospectively analyzed the clinical features and treatment outcomes of a cohort of 21 patients with intracranial pure germinomas who were diagnosed between April 2002 and December 2016 at Ehime University Hospital in Japan. Pure germinoma diagnosis was verified by histological examination of the tumor after surgery, and all patients received standard chemo-radiotherapy. A suite of clinical features, including neuroimaging, human chorionic gonadotropin-ß subunit (HCG-ß), and α-fetoprotein (AFP) in the cerebrospinal fluid (CSF), as well as immunohistochemical expression of HCG-ß, AFP, and Ki-67 in the tumor tissue were analyzed. Nineteen of the 21 patients had a complete response to standard chemo-radiotherapy without early recurrence of the tumors. Of these 19 patients, 17 did not have elevated CSF HCG-ß levels or express HCG-ß in the tumor tissue. However, the two patients who were refractory to standard therapy had elevated CSF HCG-ß levels and expressed HCG-ß in the tumor cells. These data suggest that patients with pure germinoma presenting with both an elevation of HCG-ß in the CSF and HCG-ß expression in the tumor tissue may be refractory to frontline treatment. These markers may predict aggressive germinoma and may ultimately facilitate the development of more effective treatment options.

Identification of characteristic features of pineal germinoma that enhance accuracy of preoperative differentiation in pineal region tumors: its significance on optimum surgical treatment.

The aim of the study is to identify characteristic features of pineal germinoma that enhance preoperative accuracy in differentiating germinoma from other pineal region tumors. Twenty-one consecutive patients with pineal region tumors were enrolled. In all patients, tumor resection was performed to verify the histology. Clinical records including upward gaze palsy of Parinaud's syndrome and neuroimaging were analyzed. In addition, we evaluated the relationship between magnetic resonance imaging (MRI) findings and tumor progression patterns in pineal germinoma. Among 21 patients, 15 patients were diagnosed with germ cell tumor, 4 with pineal parenchymal cell tumor, and 2 with meningioma. Upward gaze palsy was seen in 11 patients; nine had pure germinomas and two had mixed germ cell tumors. These tumors occupied the pineal region with extension to the area of the mesodiencephalic junction (MDJ) and the bi-epithalamic area between the bilateral pulvinar and the third ventricle. Tumor involvement of the former area could cause upward gaze palsy by insulting the rostral interstitial nucleus of the medial longitudinal fasciculus located in the MDJ area. Tumor invasion into the latter area is commonly seen as a cardioid-shaped tumor as the tumor image on the axial MRI view. Upward gaze palsy and a cardioid-shaped tumor image on the axial MRI views were demonstrated to be specific features of pineal pure germinoma. It is suggested that combination of both features may become useful tools to preoperatively differentiate germinoma from other pineal tumors, resulting in achievement of the optimum treatment of pineal region tumors.

[Postoperative Neurological Deficit due to Transient Hypoperfusion in the Pretreated Contralateral Hemisphere after Bypass Surgery in an Adult Patient with Moyamoya Disease:A Case Report].

Postoperative neurological deficits frequently occur in adult moyamoya disease. In this case report, we describe the time course and disease state of a patient with adult moyamoya disease, who experienced a postoperative neurological deficit due to transient hypoperfusion in the pretreated contralateral hemisphere. A 68-year-old female presented with a sudden onset of left hemianopia due to ischemic moyamoya disease. She had severely low cerebral blood flow(CBF)in the right hemisphere and deterioration of cerebrovascular reactivity in the left hemisphere. First, right combined bypass surgery was performed; subsequently, left combined bypass surgery was performed. Two days after left hemisphere surgery, left hemiparesis gradually appeared. Hypoperfusion of the right hemisphere and hyperperfusion of the left hemisphere were revealed by SPECT and CT perfusion imaging. Blood pressure was controlled to normal levels, and an antiepileptic drug, antiplatelet drug, and edaravone were administered. The patient gradually recovered 2 weeks later, and was able to resume her normal daily life. During the clinical course, laterality of CBF was improved following improvement of clinical symptoms. Abnormal postoperative disproportion of the CBF may occur after revascularization surgery for adult moyamoya disease. Prompt assessment of CBF and proper treatment are needed.

Efficacy of Early Superficial Temporal Artery-Middle Cerebral Artery Double Anastomoses for Atherosclerotic Occlusion in Patients with Progressing Stroke.

BACKGROUND: We investigated the efficacy of early superficial temporal artery-middle cerebral artery (STA-MCA) double anastomoses for patients with progressing stroke due to atherosclerotic occlusion. MATERIALS AND METHODS: Nine consecutive patients who underwent early STA-MCA double anastomoses were enrolled. All patients presented with progressing stroke despite maximal medical treatment. Cerebral blood flow in 7 patients was analyzed by single-photon emission tomography. Clinical outcomes were investigated postoperatively, and we evaluated the utility of early STA-MCA double anastomoses. RESULTS: Nine patients in the present study included those with middle cerebral artery occlusion (n = 6) and internal carotid artery occlusion (n = 3). The mean age was 58.4 years. Subjects comprised 1 female (11.1%) and 8 males (88.9%). The cause was low perfusion ischemia due to atherosclerotic occlusion with a small infarct. The mean regional cerebral blood flow (rCBF) ratio in the middle cerebral artery territory compared to the normal side was 69.6 ± 5.3%. The duration from onset to surgery was 1-8 days (median, 3.11 days). All patients underwent early STA-MCA double anastomoses, and no reperfusion-induced hemorrhage occurred. All of them slowly achieved obvious remission compared to symptoms on admission and achieved a good functional outcome. CONCLUSIONS: Early STA-MCA double anastomoses were safe and effective, and early revascularization resulted in rapid neurological improvement. We recommend this procedure for patients with progressive ischemia due to main trunk artery occlusion, when the rCBF flow ratio with the normal side was 70 ± 10%, even at the subacute stage.

[Ruptured C2 Aneurysm of the Internal Carotid Artery with Ipsilateral Unruptured Giant Internal Carotid Artery Aneurysm:A Case Report].

A giant intracranial internal carotid artery(ICA)aneurysm is difficult to treat. We encountered a case with an unruptured giant intracranial ICA aneurysm with a ruptured C2 aneurysm of the ipsilateral ICA. In this case report, we describe how the treatment and review the literature. A 59-year-old man presented with sudden-onset headache and vomiting. He was diagnosed at a local neurosurgical unit as having a subarachnoid hemorrhage and admitted to our hospital. Cerebral angiogram revealed an unruptured giant ICA C3-4 aneurysm with a ruptured aneurysm of the ipsilateral ICA C2. The ruptured aneurysm had a wide neck and was located in the medial wall of the ICA C2;it was difficult to perform neck clipping or coil embolization. We performed a high-flow bypass using a radial artery graft followed by trapping of the left ICA. Postoperative imaging showed no new ischemic damage, and the patency of bypass flow was good. The left ophthalmic artery was perfused from the external carotid artery. Although the patient experienced incomplete left oculomotor nerve palsy and left lower quadrantanopsia, his neurological function had recovered within a year, and he was able to resume his previous work. High-flow bypass with ICA trapping is effective for ICA giant aneurysm. Attention to postoperative ophthalmic function and oculomotor symptoms is important.

[A Case of Ruptured Distal Anterior Choroidal Artery Aneurysm Associated with a Twig-Like Middle Cerebral Artery, Treated with Single-Stage Aneurysm Clipping and STA-MCA Double Anastomoses in the Acute Phase].

We report the case of a patient who has progressed well over 5 years following single-stage aneurysm clipping and superficial temporal artery-middle cerebral artery(STA-MCA)double anastomoses in the acute phase, for a ruptured distal anterior choroidal artery(AChA)aneurysm accompanied by a twig-like MCA. The patient was a 49-year-old female who developed a sudden severe headache and disturbance of consciousness due to subarachnoid hemorrhage and intraventricular hemorrhage(IVH). Cerebral angiography showed a right twig-like MCA associated with an abnormal vascular network and a ruptured aneurysm in the distal AChA. A day after emergency ventricular drainage for acute hydrocephalus, right frontotemporal craniotomy enabled distal AChA aneurysm clipping, together with removal of the IVH via transchoroidal fissure approach, in addition to STA-MCA double anastomoses to prevent recurrence of hemorrhage. The IVH resolved after surgery and no new infarct area was observed. Cerebral angiography revealed the disappearance of the aneurysm, good patency of the double bypass, and reduction of the abnormal vascular network. The patient gradually recovered without any neurological deficits, except for mild memory disturbance. Five years after the surgery, the patient has experienced no recurrence. The single-stage operation of aneurysm clipping and STA-MCA double anastomoses was made possible by devising an approach for a ruptured cerebral aneurysm, even in the acute stage. The successful improvement of cerebral circulation and prevention of cerebral hemorrhage from an early stage could serve as a reference for the treatment of similar hemorrhagic cases.

[A Case of Ruptured Peripheral Cerebral Aneurysm at Abnormal Vessels Associated with Middle Cerebral Artery Stenosis:Similarity to Moyamoya Disease].

We report a case of ruptured peripheral cerebral aneurysm at abnormal vessels associated with severe stenosis at the middle cerebral artery (MCA). A 66-year-old woman was admitted at our hospital with headache on foot. Computed tomography (CT) showed intracerebral hemorrhage in the left fronto-basal area. Three-dimensional-CT and conventional angiogram revealed abnormal vessels, which were similar to those seen in moyamoya disease, with a small enhancement close to the hematoma. On day 11, subsequent cerebral angiogram demonstrated an aneurysm at the peripheral portion of an abnormal vessel arising from the left A2. On day 17, soon after the diagnosis of the ruptured aneurysm was made (while still at the subacute stage), we operated on the aneurysm. Superficial temporal artery (STA)-MCA anastomosis was also performed to preserve cerebral blood flow and reduce hemodynamic stress. Several days after the operation, she had transient aphasia due to hyperperfusion of the MCA territory, but eventually recovered with no neurological deficit at discharge. Follow-up study revealed revascularization from the branches of the external carotid artery as well as the STA. On admission, we initially thought that this patient had abnormal vessels associated with arteriosclerotic MCA stenosis. However, the postoperative clinical course as well as the histopathological specimens of both the abnormal artery with the aneurysm and the STA revealed similar findings to those of moyamoya disease. Although this case did not satisfy the criteria for moyamoya disease, it is conceivable that a single arterial occlusive lesion associated with moyamoya-like vessels might develop in the same mechanism with that of moyamoya disease.

[Hemorrhagic Adult Unilateral Moyamoya Disease with Multiple Unruptured Intracranial Aneurysms: A Case Report].

Adult unilateral moyamoya disease with intracranial aneurysm is frequently reported in the literature, but there is much variation in its treatment. In this case report, we describe the time course and treatment regimen of a patient with moyamoya disease and review the literature regarding moyamoya disease with intracranial aneurysm. A 64-year-old man had untreated intracranial aneurysm and unilateral moyamoya disease for 10 years. He presented with sudden-onset right hemiparesis and aphasia due to a subcortical hemorrhage. He was admitted to the local neurosurgical unit, and upon resolution of symptoms, he was admitted to our hospital. A cerebral angiogram revealed the champagne bottleneck sign of the left carotid artery and obliteration of the top of the left intracranial carotid artery with a moyamoya phenomenon. Two unruptured intracranial aneurysms were identified in the anterior communicating artery(Acom A) and the right intracranial carotid artery(C3). We performed superficial temporal artery-middle cerebral artery anastomosis followed by aneurysmal neck clipping of the Acom A aneurysm. Postoperative imaging showed no new ischemic damage and improved cerebral blood flow. Although the patient experienced temporal worsening of aphasia, his function recovered a few months later and he was able to resume his normal daily life activities. The combination of direct bypass surgery and aneurysmal neck clipping might be a therapeutic option for hemorrhagic unilateral moyamoya disease with unruptured intracranial aneurysm.

[A Case of ECA-MCA Double Anastomoses for Hemorrhagic Type of Twig-Like MCA].

Herein, we describe the case of a superficial temporal artery(STA)- and occipital artery (OA)-middle cerebral artery (MCA) double anastomoses that we performed to treat a hemorrhagic twig-like MCA. A 55-year-old man presented to our hospital for investigation of an incidentally identified left MCA occlusion. Left cerebral angiography revealed a twig-like MCA. The (123)I-IMP-single photon emission computed tomography( SPECT) demonstrated no reduction in the cerebral blood flow (CBF), so the patient was initially observed with no treatment. Three months later, he was readmitted with a disturbance of consciousness. The cranial computed tomography revealed a subcortical hemorrhage in the left frontotemporal region and a subdural hematoma. The hematoma was removed via emergency craniotomy. The abnormal vessels were identified and resected, but the parietal branch of the STA was damaged during the skin incision. The histological examination did not reveal the marked fibrous thickening of the intima or wavy internal elastic lamina typically seen with Moyamoya disease. Six months after the initial surgery, a STA-MCA bypass surgery was planned to prevent a future hemorrhage by reducing the hemodynamic overload of the twig-like MCA and improving the cerebral ischemia in the MCA territory that was detected on the preoperative SPECT. However, the parietal branch of the left STA had been injured during the first operation, so we performed a double anastomoses to the MCA using the frontal branch of the STA and the OA. The patient's postoperative course was uneventful and he was discharged 2 weeks after surgery. The follow-up study performed 1 year postoperatively demonstrated no evidence of cerebral infarction and revealed an improvement in the resting CBF and vascular reactivity in the left cerebral hemisphere. No subsequent cerebrovascular events have occurred in this patient during the 7 years since the double anastomoses surgery. Direct bypass for flow conversion from the internal carotid artery to the external carotid artery can be an indispensable treatment for patients with a hemorrhagic twig-like MCA.

[Long-term Outcome over Five Years after Surgical Revascularization in Adult Moyamoya Disease].

BACKGROUND AND PURPOSE: The long-term outcome of patients with adult moyamoya disease following revascularization surgery remains unclear. Here, we investigated these outcomes more than five years after revascularization surgery. PATIENTS AND METHODS: This study included 48 cerebral hemispheres from 26 patients who underwent revascularization surgery for adult moyamoya disease. The mean follow-up duration was 14.3(5.5-25.6)years. The risk factors of late-onset hemorrhage after revascularization surgery were compared between the bleeding and non-bleeding groups. We also estimated cumulative bleeding-free survival rates using Kaplan-Meier curves. RESULTS: Four patients experienced late-onset bleeding during the follow-up period. Bleeding occurred after an average of 11(8.5-16.4)years following revascularization surgery. The annual hemorrhagic rate was 1.14%. In the bleeding group, higher Suzuki stage(p=0.02), posterior cerebral artery(PCA)involvement(p<0.01), cerebral aneurysm(p=0.04), microbleeds(p=0.03), and post-operative periventricular anastomosis, especially in the thalamus and the choroidal artery(p<0.01 and p=0.01 respectively), were significantly different from those in the non-bleeding group. Kaplan-Meier analysis showed that the probability of late-onset bleeding was higher with postoperative periventricular anastomosis(p<0.01 for thalamic type, p=0.01 for choroidal type), higher Suzuki stage(p<0.01), PCA involvement(p<0.01), and cerebral aneurysm(p=0.02). CONCLUSIONS: In spite of good perfusion and reduction in moyamoya vessels after revascularization surgery, the risk of bleeding persisted. Periventricular anastomosis, especially in the thalamus and choroidal artery, after surgery, higher Suzuki stage, presence of obstructive lesions in the PCA, and cerebral aneurysm may be associated with late-onset bleeding after surgery. More studies are needed to identify the risk factors for late-onset bleeding after revascularization surgery for moyamoya disease.

Utility of three-dimensional computed tomography for anatomical assistance in endoscopic endonasal transsphenoidal surgery.

Endoscopic endonasal transsphenoidal surgery (ETSS) has been widely applied to pituitary adenomas. However, anatomical orientation is difficult when structures of the sphenoidal sinus are complicated. This study investigated the usefulness of three-dimensional computed tomography (3D-CT) modeling in planning surgical procedures for ETSS and providing anatomical guidance during surgery. CT data from 99 consecutive patients with pituitary adenoma treated between January 2008 and March 2014 were used to reconstruct 3D-CT models. Based on these images, the architecture of sphenoidal sinus, particularly structures surrounding the sellar floor, was visualized for preoperative simulation of surgical procedures. These 3D-CT images were also compared to surgical views during ETSS to evaluate applicability of the images. These models clearly demonstrated the morphology of the nasal cavity and structures of the sphenoidal sinus, including bony prominences of the internal carotid arteries (ICAs) and optic canals by successively eliminating sphenoidal structures. The 3D-CT images permitted determination of the maximum marginal line of the opening of the sellar floor by presenting vital structures such as ICAs and optic canals. With this 3D-CT model, the surgeon could access the sella more easily, open the floor widely enough for each individual patient, and resect the tumor maximally without complications. Preoperative 3D-CT models distinctly visualized the optic canals, bilateral ICAs, and complicated structures of sphenoidal septa. The 3D-CT images were useful for preoperative planning and as a road map during endoscopic surgery for pituitary adenoma, facilitating maximum tumor resection without complications.