Development and validation of machine learning models to predict postoperative infarction in moyamoya disease.

OBJECTIVE: Cerebral infarction is a common complication in patients undergoing revascularization surgery for moyamoya disease (MMD). Although previous statistical evaluations have identified several risk factors for postoperative brain ischemia, the ability to predict its occurrence based on these limited predictors remains inadequately explored. This study aimed to assess the feasibility of machine learning algorithms for predicting cerebral infarction after revascularization surgery in patients with MMD. METHODS: This retrospective study was conducted across two centers and harnessed data from 512 patients with MMD who had undergone revascularization surgery. The patient cohort was partitioned into internal and external datasets. Using perioperative clinical data from the internal cohort, three distinct machine learning algorithms-namely the support vector machine, random forest, and light gradient-boosting machine models-were trained and cross-validated to predict the occurrence of postoperative cerebral infarction. Predictive performance validity was subsequently assessed using an external dataset. Shapley additive explanations (SHAP) analysis was conducted to augment the prediction model's transparency and to quantify the impact of each input variable on shaping both the aggregate and individual patient predictions. RESULTS: In the cohort of 512 patients, 33 (6.4%) experienced postrevascularization cerebral infarction. The cross-validation outcomes revealed that, among the three models, the support vector machine model achieved the largest area under the receiver operating characteristic curve (ROC-AUC) at mean ± SD 0.785 ± 0.052. Notably, during external validation, the light gradient-boosting machine model exhibited the highest accuracy at 0.903 and the largest ROC-AUC at 0.710. The top-performing prediction model utilized five input variables: postoperative serum gamma-glutamyl transpeptidase value, positive posterior cerebral artery (PCA) involvement on preoperative MRA, infarction as the rationale for surgery, presence of an infarction scar on preoperative MRI, and preoperative modified Rankin Scale score. Furthermore, the SHAP analysis identified presence of PCA involvement, infarction as the rationale for surgery, and presence of an infarction scar on preoperative MRI as positive influences on postoperative cerebral infarction. CONCLUSIONS: This study indicates the usefulness of employing machine learning techniques with routine perioperative data to predict the occurrence of cerebral infarction after revascularization procedures in patients with MMD.

The Potential Role of Preoperative Posterior Cerebral Artery Involvement in Predicting Postoperative Transient Neurological Deficits and Ischemic Stroke After Indirect Revascularization in Patients With Moyamoya Disease.

OBJECTIVE: Transient neurological deficits (TNDs) are known to develop after direct bypass for Moyamoya disease and may be risk factors for subsequent stroke. However, the factors involved in the development of TNDs and stroke after indirect revascularization alone, including their association with subsequent stroke, remain unclear. The purpose of this study was to investigate this issue. METHODS: The subjects of the study were 30 patients with Moyamoya disease who underwent a total of 40 indirect revascularization procedures at our institution. Clinical and radiological data were collected retrospectively. To examine factors associated with the development of postoperative TND/stroke/asymptomatic disease, the clinical characteristics of each group were statistically compared. RESULTS: The mean age at surgery was 7 years (range 1-63). TNDs developed after surgery in 9 out of 40 patients (22.5%). Stroke in the acute postoperative period occurred in 3 patients (7.5%), all of whom experienced cerebral infarctions. Demographic data and preoperative clinical information were not different between the groups. However, posterior cerebral artery involvement on preoperative imaging was significantly associated with the development of TNDs and stroke (P = 0.006). Furthermore, postoperative stroke was associated with unfavorable outcomes (P = 0.025). CONCLUSIONS: Posterior cerebral artery involvement is significantly associated with the occurrence of TNDs. In contrast, TNDs after indirect revascularization have little relationship with the subsequent development of stroke. TNDs usually resolve without new strokes, and a better understanding of this particular pathology could help establish an optimal treatment regimen.

Delayed leukoencephalopathy following non-coil embolization flow diverter stent deployment for an intracranial aneurysm.

This report examines delayed leukoencephalopathy as a postoperative complication after the use of flow diverter (FD) devices for endovascular cerebral aneurysm treatment. A case involving a 78-year-old female treated with a pipeline embolization device for a left internal carotid artery aneurysm is presented. Despite adherence to dual anti-platelet therapy, the patient developed intermittent headaches and memory issues 3 months post-operation. MRI revealed T1-enhancing foci and T2 hyperintense signal abnormalities in the left cerebral hemisphere, without new ischemic lesions, indicating potential embolic events or foreign body reactions. Following aphasia, a change from clopidogrel to prasugrel and the initiation of steroid pulse therapy led to the resolution of symptoms and MRI abnormalities over 6 months. This case underscores the reversibility of delayed leukoencephalopathy with appropriate intervention.

Characteristics of donor vessels and cerebral blood flow in the chronic phase after combined revascularization surgery for moyamoya disease.

OBJECTIVE: This study aimed to analyze whether the development of donor vessels after combined revascularization surgery for moyamoya disease (MMD) is related to cerebral blood flow (CBF) changes. METHODS: We retrospectively reviewed the charts of 11 adult (12 hemispheres) and 13 pediatric (19 hemispheres) patients who underwent combined revascularization in our department. The total vessel cross-sectional area (TVA) was the sum of the cross-sectional areas of the superficial temporal, middle meningeal, and deep temporal arteries imaged using time-of-flight magnetic resonance angiography. The ipsilateral relative CBF (RCBF) on the brain surface in the craniotomy area was calculated by single-photon emission computed tomography. ΔTVA and ΔRCBF were defined as the preoperative and postoperative ratios of TVA and RCBF, and their correlations were analyzed in adult and pediatric patients. RESULTS: The TVA and RCBF showed a significant increase after surgery, regardless of the age group. However, there was no significant correlation between ΔTVA and ΔRCBF in either the adult or pediatric groups. While the adult group exhibited significantly higher ΔRCBF values compared to the pediatric group (p < 0.01, r = -0.44), the ΔTVA values were higher in the pediatric group compared to the adult group (p = 0.06). CONCLUSIONS: In the chronic phase after combined revascularization surgery for MMD, the development of measurable TVA of donor vessels does not necessarily correlate with an increase in CBF around the craniotomy area.

Efficacy of intraoperative irrigation with artificial cerebrospinal fluid in chronic subdural hematoma surgery: study protocol for a multicenter randomized controlled trial.

BACKGROUND: The surgical techniques for treatment of chronic subdural hematoma (CSDH), a common neurosurgical condition, have been discussed in a lot of clinical literature. However, the recurrence proportion after CSDH surgery remains high, ranging from 10 to 20%. The standard surgical procedure for CSDH involves a craniostomy to evacuate the hematoma, but irrigating the hematoma cavity during the procedure is debatable. The authors hypothesized that the choice of irrigation fluid might be a key factor affecting the outcomes of surgery. This multicenter randomized controlled trial aims to investigate whether intraoperative irrigation using artificial cerebrospinal fluid (ACF) followed by the placement of a subdural drain would yield superior results compared to the placement of a subdural drain alone for CSDH. METHODS: The study will be conducted across 19 neurosurgical departments in Japan. The 1186 eligible patients will be randomly allocated to two groups: irrigation using ACF or not. In either group, a subdural drain is to be placed for at least 12 h postoperatively. Similar to what was done in previous studies, we set the proportion of patients that meet the criteria for ipsilateral reoperation at 7% in the irrigation group and 12% in the non-irrigation group. The primary endpoint is the proportion of patients who meet the criteria for ipsilateral reoperation within 6 months of surgery (clinical worsening of symptoms and increased hematoma on imaging compared with the postoperative state). The secondary endpoints are the proportion of reoperations within 6 months, the proportion being stratified by preoperative hematoma architecture by computed tomography (CT) scan, neurological symptoms, patient condition, mortality at 6 months, complications associated with surgery, length of hospital stay from surgery to discharge, and time of the surgical procedure. DISCUSSION: We present the study protocol for a multicenter randomized controlled trial to investigate our hypothesis that intraoperative irrigation with ACF reduces the recurrence proportion after the removal of chronic subdural hematomas compared with no irrigation. TRIAL REGISTRATION: ClinicalTrials.gov jRCT1041220124. Registered on January 13, 2023.

Effect of thin-split encephalomyosynangiosis on transient neurological events in revascularization surgery for pediatric patients with moyamoya disease.

OBJECTIVE: Transient neurological events (TNEs) are among the most important events after revascularization surgery in pediatric patients with moyamoya disease (MMD). Although hemodynamic changes and crying are representative factors of TNEs, brain compression by encephalomyosynangiosis (EMS) is another important cause of TNEs. Therefore, the authors assumed that making the EMS as thin as possible reduces the frequency of TNEs. However, thin-split EMS can lead to insufficient development of collateral vessels. This study aimed to evaluate the effects of thin-split EMS in combined revascularization surgery on postoperative outcomes in pediatric patients with MMD. METHODS: The authors retrospectively included 56 consecutive combined revascularization surgeries in the anterior cerebral circulation in pediatric patients with MMD. These surgeries were classified into the former group and thin-split EMS group. The temporal muscle was halved in the former EMS group and split as thinly as possible in the thin-split EMS group. The authors performed between-group comparisons of postoperative stroke events and TNEs during the acute period and the development of collateral flow and stroke events during the chronic period. RESULTS: Former and thin-split EMS procedures were performed in 37 and 19 patients, respectively. TNEs without crying or hemodynamic changes were observed significantly less frequently in the thin-split EMS group than in the former EMS group (0 [0.0%] in the thin-split EMS group vs 9 [24.3%] in the former EMS group, p = 0.021). There were no significant between-group differences in the development of indirect bypass during the chronic period (good: 34 [91.9%] and poor: 3 [8.1%] in the former EMS group vs good: 16 [84.2%] and poor: 3 [15.8%] in the thin-split EMS group; p = 0.397). Additionally, there were no significant between-group differences in the incidence of chronic clinical events, including death or stroke. CONCLUSIONS: Thin-split EMS can reduce TNEs that do not accompany crying or hemodynamic changes. Furthermore, the procedure has sufficient prevention effects against stroke events during the chronic postoperative period in pediatric patients with MMD.

Safety and efficacy of the endoscopic transsphenoidal transclival approach performed using direct cortical stimulation for pontine cavernous malformations.

OBJECTIVE: Surgical treatment of brainstem cavernous malformations (CMs) is challenging. Surgery using the endoscopic transsphenoidal transclival approach (eTSTCA) is reported as a useful alternative for ventral brainstem CMs. However, CMs located in the ventral midline of the brainstem are rare, and only a small number of case reports on these CMs treated with the eTSTCA exist. The efficacy and safety of the eTSTCA have not yet been fully examined. METHODS: A retrospective analysis was performed for 5 consecutive patients who underwent surgery via the eTSTCA for treating ventral pontine CMs. RESULTS: The average maximum CM diameter was 26.0 mm (18-38 mm). All patients underwent MR-diffusion tensor imaging, which confirmed that the corticospinal tract (CST) deviated posteriorly or laterally to the CM. Direct brainstem cortical stimulation was performed to localize the CST before making the cortical incision. After the excision of the CM, the cavity was filled with artificial CSF to make an aqueous surgical field (wet-field technique) for observing the tumor cavity and confirming complete hemostasis and resection. Total removal was achieved in all patients. The preoperative modified Rankin Scale score was 3 in 3 patients and 4 in 2 patients, whereas it was 1 in 2 patients and 0 in 3 patients 3 months after surgery. Postoperative CSF leakage was observed in 1 patient, and transient abducens nerve palsy was observed in 1 patient. No other intra- or postoperative complications were observed. CONCLUSIONS: MR-diffusion tensor imaging and direct brainstem cortical stimulation were useful to ascertain the proximity of the CST to the CM. The endoscope provides a clear view even underwater, and it was safe and effective to observe the entire CM cavity and confirm complete hemostasis without additional retraction of the brainstem parenchyma, including the CST. The eTSTCA provides a direct access point to the lesion and may be a safer alternative treatment for patients whose CST deviates laterally or posteriorly to the CM.

Posterior cerebral artery involvement in unilateral moyamoya disease is exclusively ipsilateral and influenced by RNF213 mutation gene dose: The SUPRA Japan study: PCA involvement in unilateral moyamoya.

OBJECTIVES: The characteristics and clinical implications of posterior cerebral artery (PCA) involvement in unilateral moyamoya disease (U-MMD), such as laterality, frequency of the RNF213 p.R4810K mutation, and clinical outcomes, have not been well studied. POPULATION AND METHODS: We analyzed a cohort of 93 patients with U-MMD who participated in the SUPRA Japan study. Clinical characteristics and radiological examinations were collected from medical records. The presence of the p.R4810K mutation was determined using a TaqMan assay. The clinical outcome was assessed using the modified Rankin Scale (mRS). Univariate and multivariate logistic regression analyses were performed to assess the associations. RESULTS: Among the patients with U-MMD, PCA involvement was observed in 60.0 % (3/5) of patients with homozygous mutation, 11.3 % (7/62) of those with heterozygous mutation, and 3.8 % (1/26) of those with wild type, showing a significant linear trend (p < 0.001 for trend). PCA involvement was observed exclusively on the same side as the affected anterior circulation. Dyslipidemia and cerebral infarction at initial onset were independently associated with mRS ≥1. Hypertension was associated with mRS ≥1 and it was also linked to infarction at initial onset, suggesting a potential confounding effect. Although PCA involvement showed a trend for higher mRS, it was not statistically significant. CONCLUSIONS: Our findings indicate a gene dose effect of the p.R4810K mutation on PCA involvement, with the homozygous state showing the most significant effect. Both genetic and modifiable factors such as dyslipidemia may influence the progression of U-MMD.

Difference of coil distribution made by finishing coils in large size aneurysm model with radiolucent coils.

We conducted a study to understand the characteristics of the finishing coils to select the appropriate coil for the final stage of embolization. Consequently, experimental embolization was performed on a 10 mm spherical silicone aneurysm filled with radiolucent coils, which simulated a volume embolization ratio of 20%. Nine different coils (i-ED complex ∞ SilkySoft, SilkySoft, ExtraSoft, V-Trak HyperSoft helical, Barricade 10 complex finishing, Optima complex 10 soft, Target 360 Ultra, Galaxy G3 mini, and Axium prime 3D ExtraSoft) were analyzed six times at random. After each coil insertion, indices that include area, Feret diameter, circularity, and centroid center of mass were calculated using biplane x-ray images. Furthermore, these data were analyzed using the spring constant k, which represents the stiffness of the coil. In multiple comparisons, a significant difference was observed in the area analysis. The i-ED complex ∞ SilkySoft was more widespread than Target 360 Ultra (p < 0.05). However, no significant differences were observed in the other indices. The spring constant k value of Target 360 Ultra was 2.5 times larger than that of the i-ED complex ∞ SilkySoft, and it negatively correlated with the area index rather than with the other indices. Notably, it was suggested that the smaller the spring constant k, the wider the distribution of the finishing coils. Although there was little difference between the coils, some coils had characteristics suggesting that good embolization could be expected using appropriate finishing coils.

Development of machine learning models for predicting unfavorable functional outcomes from preoperative data in patients with chronic subdural hematomas.

Chronic subdural hematoma (CSDH) often causes neurological deterioration and is treated with hematoma evacuation. This study aimed to assess the feasibility of various machine learning models to preoperatively predict the functional outcome of patients with CSDH. Data were retrospectively collected from patients who underwent CSDH surgery at two institutions: one for internal validation and the other for external validation. The poor functional outcome was defined as a modified Rankin scale score of 3-6 upon hospital discharge. The unfavorable outcome was predicted using four machine learning algorithms on an internal held-out cohort (n = 188): logistic regression, support vector machine (SVM), random forest, and light gradient boosting machine. The prediction performance of these models was also validated in an external cohort (n = 99). The area under the curve of the receiver operating characteristic curve (ROC-AUC) of each machine learning-based model was found to be high in both validations (internal: 0.906-0.925, external: 0.833-0.860). In external validation, the SVM model demonstrated the highest ROC-AUC of 0.860 and accuracy of 0.919. This study revealed the potential of machine learning algorithms in predicting unfavorable outcomes at discharge among patients with CSDH undergoing burr hole surgery.

Higher abundance of Campylobacter in the oral microbiome of Japanese patients with moyamoya disease.

Genetic factors alone cannot explain the pathophysiology of moyamoya disease (MMD), and environmental factors such as an immune response are thought to be involved. Oral and gut microbiomes have attracted attention as environmental factors in the pathophysiology of some vascular and autoimmune diseases. However, the relationship between MMD and these microbiomes is yet to be thoroughly investigated. This prospective case-control study aimed to compare the microbiomes of Japanese patients with MMD with those of healthy individuals to identify the specific bacteria involved in MMD. Saliva and fecal samples were collected from 16 patients with MMD who had not undergone revascularization surgery. Fifteen healthy individuals were matched for age, sex, and body mass index. The microbiomes were determined using 16S rRNA sequencing and analyzed using QIIME2. Differentially abundant microbes were identified using LEfSE and ANCOM-BC. In the oral microbiome, the two analytical methods showed that Campylobacter was more abundant in patients with MMD than in healthy individuals. Differences in the gut microbiome were smaller than those in the oral microbiome. In conclusion, the oral microbiome profiles of patients with MMD significantly differ from those of healthy individuals. Campylobacter spp. could be a substantial environmental factor in the pathophysiology of MMD.

Transvenous embolization via the facial vein for intraorbital dural arteriovenous fistula: illustrative case.

BACKGROUND: Intraorbital arteriovenous fistula (IOAVF) is a rare type of intracranial fistula that presents with ocular signs similar to those of cavernous sinus dural arteriovenous fistula. The treatment of IOAVF is based on the vascular architecture of each case due to its infrequent occurrence. The authors report the case of an IOAVF that was successfully treated with embolization via the facial vein, with good outcomes. OBSERVATIONS: A 78-year-old woman presented with left eyelid swelling, pulsatile ocular protrusion, and left ocular conjunctival hyperemia. Ophthalmological evaluation revealed elevated intraocular pressure; time-of-flight magnetic resonance angiography revealed a dilated left superior ophthalmic vein. Digital subtraction angiography showed an arteriovenous shunt in the left superior orbital fissure, which was treated using transvenous coil embolization. The patient experienced immediate improvement in left ocular protrusion and conjunctival hyperemia. Ophthalmological evaluation 1 month after treatment showed normal intraocular pressure in the left eye. No neurological symptoms were observed, and there was no recurrence of the arteriovenous shunt 3 months postoperatively. LESSONS: The authors report a rare case of IOAVF treated with embolization via the facial vein with a good outcome. A thorough understanding of the vascular architecture using three-dimensional images is useful for determining endovascular access and procedures.

Transarterial embolization and transvenous embolization for transverse-sigmoid sinus dural arteriovenous fistulas with cortical venous reflux: A comparative study.

BACKGROUND: Dural arteriovenous fistulas (DAVFs) with cortical venous reflux (CVR) carry a high risk for neurological sequelae or death. Recently, transverse-sigmoid sinus DAVFs were shown as good indications for transarterial embolization (TAE) with ONYX. Here, we compared the effectiveness and safety of conventional transvenous embolization (TVE) with those of TAE with ONYX for transverse-sigmoid sinus DAVFs with CVR. METHODS: Sixty-one patients with transverse-sigmoid sinus DAVFs were treated from April 2013 to May 2020. Among them, 37 patients with CVR were included. Transarterial embolization and TVE were compared in terms of complete occlusion and residual CVR immediately after treatment, complications with worsening modified Rankin Scale (mRS) ≥ 1, amount of contrast media used during treatment, radiation exposure, and surgical procedure time. RESULTS: Ten patients were treated with 10 TAEs using ONYX and 27 patients with 29 TVEs. Transarterial embolization and TVE showed no differences in the overall complete occlusion rate (80% [8/10 patients] vs. 80% [23/27], respectively), whereas the residual rate of existing CVR was 10% (1/10 patient) vs. 3.4% (1/27) in the TAE and TVE groups, respectively. No complications with worsening mRS ≥1 occurred in either group. Among the parameters of amount of contrast media, radiation dose, and operative time, only radiation dose in the TAE group was lower than that in the TVE group (median: 2239 mGy vs. 3268 mGy, respectively; p = 0.07). CONCLUSION: For transverse-sigmoid sinus DAVFs with CVR, TAE treatment reduced radiation exposure. However, both TAE and TVE achieved high complete occlusion rates and low complication rates.

Real-world data of clazosentan in combination therapy for aneurysmal subarachnoid hemorrhage: a multicenter retrospective cohort study.

Aneurysmal subarachnoid hemorrhage (aSAH) may lead to cerebral vasospasm, significantly associated with morbidity and mortality. In double-blind, placebo-controlled phase 3 studies, clazosentan reduces cerebral vasospasm-related morbidity and all-cause mortality in patients with aSAH. There are no reports about the clinical efficacy of clazosentan combination therapy with some other drugs. Initially, we explored the efficacy of clazosentan combination therapy with cilostazol, statin, and antiepileptic drugs. Subsequently, we assessed the add-on effect of fasudil to clazosentan combination therapy for aSAH patients. This multicenter, retrospective, observational cohort study included Japanese patients with aSAH between June 2022 and March 2023. The primary outcome was the ordinal score on the modified Rankin Scale (mRS; range, 0-6, with elevated scores indicating greater disability) at discharge. Among the 47 cases (women 74.5%; age 64.4 ± 15.0 years) undergoing clazosentan combination therapy, 29 (61.7%) resulted in favorable outcomes. Overall, vasospasm occurred in 16 cases (34.0%), with four cases (8.5%) developing vasospasm-related delayed cerebral ischemia (DCI). Both hypotension and vasospasm-related DCI were related to unfavorable outcome at discharge. Fasudil were added in 18 (38.3%) cases. Despite adding fasudil to clazosentan combination therapy, the incidence of aSAH-related vasospasm did not decrease. Added-on fasudil to combination therapy related to pulmonary edema, vasospasm, and vasospasm-related DCI, and unfavorable outcomes. Clazosentan combination therapy could potentially result in favorable outcomes for aSAH patients to prevent post-aSAH vasospasm-related DCI. The add-on effect of fasudil to combination therapy did not demonstrate a significant impact in reducing aSAH-related vasospasm or improving outcomes at discharge.

Simultaneous Aneurysmal Subarachnoid Hemorrhage and Epistaxis in an Untreated Prolactinoma: A Case Report and Literature Review.

A 61-year-old man presented with massive epistaxis, amaurosis, nausea, and severe headache. A detailed examination revealed a subarachnoid hemorrhage and prolactinoma. Angiography showed a small internal carotid artery pseudoaneurysm and inadequate collateral circulation; thus, uncomplicated coil embolization was performed. Considering the side effects of medication, such as cerebrospinal fluid rhinorrhea, the patient was followed up for asymptomatic prolactinoma without medication after discharge. At 40 months later, aneurysm recurrence was confirmed. Flow diverter device placement was performed, and the outcomes were excellent. In the present report, we described a rare case of a ruptured internal carotid artery aneurysm in an untreated prolactinoma and discussed the literature.

Clinical Efficiency of an Artificial Intelligence-Based 3D-Angiography for Visualization of Cerebral Aneurysm: Comparison with the Conventional Method.

PURPOSE: Artificial intelligence (AI)-based three-dimensional angiography (3D-A) was reported to demonstrate visualization of cerebral vasculature equivalent to that of three-dimensional digital subtraction angiography (3D-DSA). However, the applicability and efficacy of the AI-based 3D­A algorithm have not yet been investigated for 3D-DSA micro imaging. In this study, we evaluated the usefulness of the AI-based 3D­A in 3D-DSA micro imaging. MATERIALS AND METHODS: The 3D-DSA micro datasets of 20 consecutive patients with cerebral aneurysm (CA) were reconstructed with 3D-DSA and 3D­A. Three reviewers compared 3D-DSA and 3D­A in terms of qualitative parameters (degrees of visualization of CA and the anterior choroidal artery [AChA]) and quantitative parameters (aneurysm diameter, neck diameter, parent vessel diameter, and visible length of AChA). RESULTS: Qualitative evaluation of diagnostic potential revealed that visualization of CA and the proximal to middle parts of the AChA with 3D­A was equal to that with conventional 3D-DSA; in contrast, visualization of the distal part of the AChA was lower with 3D­A than with 3D-DSA. Further, regarding quantitative evaluation, the aneurysm diameter, neck diameter, and parent vessel diameter were comparable between 3D­A and 3D-DSA; in contrast, the visible length of the AChA was lower with 3D­A than with 3D-DSA. CONCLUSIONS: The AI-based 3D­A technique is feasible and evaluable visualization of cerebral vasculature with respect to quantitative and qualitative parameters in 3D-DSA micro imaging. However, the 3D­A technique offers lower visualization of such as the distal portion of the AChA than 3D-DSA.

Paroxysmal Sympathetic Hyperactivity in Stroke.

OBJECTIVE: Paroxysmal sympathetic hyperactivity (PSH) is a life-threatening neurological emergency associated with severe brain injury. Stroke-related PSH, particularly post-aneurysmal subarachnoid hemorrhage (aSAH) PSH, has been relatively understudied and is often misdiagnosed as an aSAH-related hyperadrenergic crisis. This study aims to clarify the feature of stroke-related PSH. METHODS: This study discusses the case of a patient with post-aSAH PSH and identifies 19 articles (25 cases) on stroke-related PSH by searching the PubMed database from 1980 to 2021. RESULTS: In the total cohort, 15 (60.0%) patients were male and the average age was 40.1 ± 16.6 years. The primary diagnoses included intracranial hemorrhage (13 cases, 52.0%), cerebral infarction (7 cases, 28.0%), subarachnoid hemorrhage (4 cases, 16.0%), and intraventricular hemorrhage (1 case, 4.0%). The sites of stroke damage were predominantly the cerebral lobe (10 cases, 40.0%), basal ganglia (8 cases, 32.0%), and the pons (4 cases, 16.0%). The median time of PSH onset after admission was 5 (1-180) days. Most cases employed combination therapy with sedation drugs, beta-blockers, gabapentin, and clonidine. On the Glasgow Outcome Scale, outcomes included death (4 cases, 21.1%), vegetative state (2 cases, 10.5%), severe disability (7 cases, 36.8%), and in only one case (5.3%) was a good recovery noted. CONCLUSIONS: The clinical features and treatment of post-aSAH PSH differed from those of aSAH-related hyperadrenergic crises. Early diagnosis and treatment can prevent severe complications. PSH should be acknowledged as a potential complication of aSAH. Differential diagnosis can aid in developing individualized treatment plans and improving patient prognosis.

Ruptured Middle Cerebral Artery Aneurysm in an Infant: Case Report and Literature Review.

Intracranial aneurysms (IA) in infants are reportedly rare at 0.5% to 4.5% of all aneurysms. Furthermore, subarachnoid hemorrhage in infants younger than three months are even rarer as it has been reported in approximately 20 cases only till date. A 3-month-old infant with seizures and impaired consciousness was admitted to our hospital. Three-dimensional computed tomography angiography (3D-CTA) revealed a dissecting aneurysm with a maximum diameter of 13 mm in the right M2. Internal trapping using detachable coil were successfully performed, following which he was discharged without significant neurological deficit after one month of onset. Thus, we have reported a rare case of a large ruptured dissecting IA in a 3-month-old infant, in the right middle cerebral artery (MCA), successfully treated with an endovascular therapy, along with a literature review.

Secondary aneurysmal bone cyst of the frontal bone with fibrous dysplasia showing rapid expansion: a case report.

A 19-year-old woman presented with swelling of the left forehead without pain. She did not have any relevant past or family history. Computed tomography showed destruction of the outer cortex of the frontal bone. A solitary mass lesion with a fluid collection was detected with magnetic resonance imaging. Because the swelling of the left forehead had enlarged rapidly with osteolytic changes, surgical removal of the lesion was performed. The lesion appeared to be enveloped in a fibrous capsule. The soft lesion was removed from the frontal bone. The outer frontal bone was absent, although the inner frontal bone was preserved. Then, the frontal bone was resected with margins from the edge of the erosion. The dura mater under the lesion was intact. A cranioplasty was performed using titanium mesh. On histological examination, the trabecular bones revealed irregular shapes and arrangements, indicating fibrous dysplasia. There was a continuous high-cell-concentration pathological lesion outside the fibrous dysplasia. There were numerous cells, such as mononuclear cells, osteoclast-like multinucleated giant cells, foam cells, and red blood cells. The osteoclast-like multinucleated giant cells and other cells did not show significant nuclear atypia. Immunostaining with H3.3G34W was negative, and the ubiquitin-specific peptidase 6/Tre-2 gene showed no rearrangements. The histopathological diagnosis was secondary aneurysmal bone cyst with fibrous dysplasia. Additional postsurgical therapy was not performed. There has been no evidence of recurrence of the lesion for two years.

Neuronal Loss in the Bilateral Medial Frontal Lobe Revealed by 123I-iomazenil Single-photon Emission Computed Tomography in Patients with Moyamoya Disease: The First Report from Cognitive Dysfunction Survey of Japanese Patients with Moyamoya Disease (COSMO-Japan Study).

Cognitive impairment in adult patients with moyamoya disease (MMD) is sometimes overlooked and can occur in patients with no ischemic or hemorrhagic lesions. Better profiling and reliable diagnostic methods that characterize the group and associate the impairments and pathology of MMD are required in order to deliver appropriate treatments and support. The potential of 123I-iomazenil single-photon emission computed tomography (SPECT) for this issue has been reported in some studies, but the universality of this method remains unclear. A multicenter study of adult patients (aged 18-60 years) with MMD who experienced difficulties in social lives despite normal activities of daily living was implemented to delineate the common characteristics of this group of patients. In this study, iomazenil SPECT, besides patient characteristics, cognitive functions, and conventional imaging, was acquired to examine whether this method is suitable as a universal diagnostic tool. A total of 36 patients from 12 institutes in Japan were included in this study. Domain scores of world health organization quality of life 26 indicated low self-rating in physical health and psychological domains. The percentages of patients who had <85 in each index were 27.8%-33.3% in the WAIS-III and 16.7%-47.2% in the Wechsler Memory Scale-Revised. The group analysis of iomazenil SPECT demonstrated a decreased accumulation in the bilateral medial frontal areas in comparison with the normal control, whereas there were no specific characteristics on conventional imaging in the cohort. Iomazenil SPECT is a possible universal diagnostic method for the extraction of patients with cognitive impairment in MMD.