Hypertensive disorders of pregnancy in moyamoya disease: A single institution experience.

OBJECTIVE: The characteristics of pregnancy and delivery in patients with moyamoya disease (MMD) remain unclear. We retrospectively investigated perinatal outcomes in patients with MMD to evaluate the risks associated to this condition. MATERIALS AND METHODS: Clinical data of women with MMD who delivered at the University of Tokyo Hospital between 2000 and 2021 were collected. Maternal characteristics including genetic data, obstetric complications, method of delivery and anesthesia, neonatal outcomes, neurological events during pregnancy, delivery, and postpartum course, were reviewed. RESULTS: Thirteen pregnancies with MMD were identified. The median maternal age was 30 years. The initial clinical symptoms were identified as transient ischemic attack, infarction, and headache. Eight patients had a history of bypass surgery. The median gestational age at delivery was 37 weeks. DNA samples were collected from five patients, responsible for six pregnancies. Of these six cases, five had the RNF213 c.14429G > A (p.Arg4810Lys) heterozygous variant. Of the 13 pregnancies, seven had hypertensive disorder of pregnancy (HDP). Additionally, three of five pregnancy cases with RNF213 p.Arg4810Lys heterozygous variant presented with HDP. Nine patients underwent cesarean section, and four delivered vaginally with epidural anesthesia. One case of ischemic stroke was confirmed during the postpartum period. Regarding newborns, neither Apgar scores lower than 7 nor neonatal intensive care unit admissions were reported. CONCLUSIONS: This study suggests that the frequency of HDP is higher in patients with MMD compared to those with normal pregnancies. Strict blood pressure control should be performed in patients with MMD during pregnancy and postpartum period.

Association Between the Onset Pattern of Adult Moyamoya Disease and Risk Factors for Stroke.

BACKGROUND AND PURPOSE: Few previous studies have comprehensively explored the relationship between the onset pattern of adult moyamoya disease and risk factors for stroke. We performed a retrospective analysis focusing on risk factors for stroke and related findings on magnetic resonance imaging/angiography with respect to the pattern of disease onset. We also examined whether risk factors for stroke were associated with an increased risk for symptomization in asymptomatic patients. METHODS: A total of 178 adult patients with moyamoya disease (asymptomatic, n=84; ischemic, n=71; hemorrhagic, n=23) at the University of Tokyo Hospital from 2000 to 2018 were included in this study. Data pertaining to patient background and magnetic resonance imaging findings were analyzed retrospectively. In the asymptomatic group, the effects of stroke-associated risk factors on symptom onset were analyzed. RESULTS: Comparisons among the 3 groups revealed no significant difference in the frequency of risk factors for stroke. The proportion of patients with magnetic resonance imaging/angiography findings indicating anterior choroidal artery anastomosis or microbleeds was significantly higher in the hemorrhagic group than in the asymptomatic or ischemic group. Among asymptomatic patients, the hazard ratios for symptomization with hypertension and dyslipidemia were 6.69 ([95% CI, 1.23-36.4] P=0.028) and 8.14 ([95% CI, 1.46-45.2] P=0.017), respectively. CONCLUSIONS: The development of anterior choroidal artery anastomosis and microbleeds on magnetic resonance imaging/angiography was significantly associated with hemorrhagic onset. Hypertension and dyslipidemia may increase the risk of cerebrovascular events in asymptomatic patients, and thus, early intervention to these factors may be important.

Development of clinical competence assessment tool for novice physical and occupational therapists-a mixed Delphi study.

[Purpose] The aim of this study was to clarify essential abilities of novice physical and occupational therapists for independent execution of their duties and to develop a clinical competence assessment tool. [Subjects] Forty-five experienced therapists participated in this study. [Methods] A two-phase mixed-methods design was used. First, semi structured interviews were conducted on 15 experienced therapists to create a comprehensive list of essential abilities that novice therapists need. Second, 30 experienced therapists participated in a two-round Delphi study to select items for the assessment tool being developed. [Results] Fifty-five items were extracted and classified into three categories: basic attitudes, therapeutic skills, and clinical practice-related thoughts. [Conclusion] Present results suggest that not only knowledge of execution of therapy-related duties and therapeutic skills is essential in novice therapist, but also appropriate abilities in social adjustment, self-management, and self-education. The newly developed tool might be useful for postgraduate education in clinical practice.

Reliability of clinical competency evaluation list for novice physical and occupational therapists requiring assistance.

[Purpose] This study examined the reliability of a clinical ability evaluation table developed in a previous study with the aim of clarifying the abilities necessary for therapists to independently implement their duties. [Subjects and Methods] Forty-eight physical therapists with less than 2 years of clinical experience were targeted for evaluation, 48 main supervisors, and 48 sub-supervisors, 144 in total, were studied. [Results] The total score was lower when the evaluation was conducted by the target therapists themselves than when it was conducted by the main or sub-supervisors. Regarding the reliability of the total scores for the entire scale and each category, values representing the intra-rater reliability were higher when the evaluation was conducted by the target therapists or main supervisors, while there were marked differences between high and low values for each item. Regarding the inter-rater reliability, both the total scores for the entire scale and each category, as well as values for each item, were low. [Conclusion] Values representing the intra-rater reliability of the study table were low, indicating the necessity of further improvement.

A bibliometric analysis of the 100 most-cited clinical articles in the research of intracranial artery stenosis and intracranial atherosclerosis.

Background: Intracranial arterial stenosis (ICAS), caused by intracranial atherosclerosis, is one of the major causes of ischemic stroke. This study identified the top 100 most-cited publications on ICAS through a bibliometric analysis. Methods: Two independent authors conducted a search in the Web of Science database for clinical articles on ICAS published between 1993 and 2022. The top 100 most-cited articles were then extracted. For each article, the analysis covered the title, author, country of origin/affiliation, journal, total number of citations, number of citations per year, and type of study. Results: The top 100 most-cited papers in the ICAS were authored by 565 authors from 12 countries and published in 29 journals. In terms of the 5-year trend, the largest number of papers were published between 2003 and 2007 (n = 31). The median number of citations for the 100 papers was 161 (range 109-1,115). The journal with the highest proportion of the 100 most published articles was Stroke, accounting for 41% of articles and 37% of the citations. According to country of origin, the United States of America accounted for the largest number of articles, followed by China, Japan, and South Korea, with these four countries together accounting for 81% of the total number of articles and 88% of the citations. Trends in the past five years included the use of terms such as acute ischemic stroke and mechanical thrombectomy. Conclusion: The findings of this study provide novel insight into this field and will facilitate future research endeavors.

Sustaining consistent cobalt-60 dose rate enhances radiosurgical outcomes in brain arteriovenous malformation management.

BACKGROUND AND PURPOSE: The impact of cobalt-60 dose rate (Co-60 DR) on outcomes of stereotactic radiosurgery (SRS) for brain arteriovenous malformations (AVMs) remains fully elucidated. This study explored the association between Co-60 DR and SRS outcomes in AVM treatment. MATERIALS AND METHODS: 772 AVM patients from 1990 to 2020 were included. High DR was defined as ≥ 2.4 Gy/min. AVM patients were categorized into 3 cohorts based on the nidus volume: small (<5 mL), medium (≥5 mL and < 10 mL), and large (≥10 mL). The primary endpoint was AVM obliteration; secondary endpoints included post-SRS hemorrhage. RESULTS: Cumulative obliteration rates of the large AVM were significantly increased in the high DR group than those in the low DR group (84 % vs. 45 % at 5 years, log-rank test; p = 0.011). Multivariable analysis showed that the obliteration rate was significantly elevated for the high DR group in the large AVM cohort with an adjusted hazard ratio (HR) of 1.78 (95 % confidence interval [CI]: 1.00-3.17, p = 0.049). Post-SRS hemorrhage rates of the entire cohort were significantly decreased in the high DR group than in the low DR group (2.5 % vs. 5.3 % at 5 years, log-rank test; p = 0.035). Multivariable analysis revealed post-SRS hemorrhage was reduced in the high DR group with an adjusted HR of 0.47 (95 % CI: 0.24-0.92, p = 0.026). CONCLUSION: High DR may lead to increased efficiency for large AVMs and decreased post-SRS hemorrhage in all AVM cases. Sustaining a higher Co-60 DR could potentially yield favorable outcomes for SRS for AVMs.

Custom shaping of distal access catheter for navigation of microcatheter into inferolateral and meningohypophyseal trunk feeders.

OBJECTIVE: Navigating a microcatheter to tiny feeding arteries such as the inferolateral trunk (ILT) and meningohypophyseal trunk (MHT) of the internal carotid artery (ICA) is technically challenging because of the anatomical features of steep angulation, small diameter, and significant caliber difference from the ICA. To guide the microcatheter to these ICA side branch feeders, the authors thought that a custom shaping of the intermediate catheter would be helpful to determine the direction of the microcatheter and strongly back it up. The aim of this study was to report the detailed methodology and clinical outcomes of patients who had undergone the embolization of ILT and MHT feeders using this method. METHODS: This was a retrospective study of consecutive patients with intracranial tumors and dural arteriovenous fistulas (DAVFs) who had undergone endovascular embolization. The details of the embolization procedures were examined, especially the technique of guiding a low-profile microcatheter supported by the thermoformed intermediate catheter. The authors assessed the patient background, procedural outcomes, and postoperative complications. RESULTS: Between October 2013 and June 2022, 43 patients with intracranial tumors and 45 with DAVFs underwent embolization at the authors' institutions. Among these cases, embolization of the ICA side branch feeder was attempted in 10 intracranial tumors and 2 DAVFs. Of these 12 patients, the microcatheter was guided deep enough for successful embolization in 11 cases (92%). Two asymptomatic arteriovenous fistulas were complicated during guidewire manipulation deep in the feeder. Yet, both disappeared completely after glue injection into the feeding artery. No spasm or dissection of the parent artery occurred during the procedure. Postoperative radiological evaluation showed no intracranial hematoma or symptomatic cerebral infarction. One patient (9%) developed permanent postoperative cranial neuropathy that gradually improved after embolization. CONCLUSIONS: This study revealed that custom shaping of distal access catheters is an effective technique that enabled the embolization of ILT and MHT feeders with a success rate of more than 90%.

Stroke Events and Risk Factors in Older Patients with Moyamoya Disease.

BACKGROUND: We aimed to comprehensively analyze the epidemiology, natural history, stroke events and their risk factors, and the RNF213 p.Arg4810Lys variant in older patients with moyamoya disease (MMD). METHODS: We enrolled patients with MMD followed-up at our hospital between 2000 and 2023. Those who developed MMD at age ≥60 years or were diagnosed at a younger age and followed-up after age 60 years were included. Baseline characteristics, onset type, radiologic features, and RNF213 p.Arg4810Lys variant status were investigated. RESULTS: Among 56 patients with 100 affected hemispheres, 62 were asymptomatic, 26 experienced ischemic onset, and 12 had hemorrhagic onset. A higher incidence of anterior choroidal artery (AchA) dilatation and lower proportion of favorable modified Rankin scale scores were detected in hemorrhagic onset, whereas greater prevalence of bypass surgery in ischemic onset. Of 76 asymptomatic hemispheres at the age of 60 years, subsequent stroke events occurred in 10 hemispheres, comprising 8 hemorrhages and 2 ischemias. Risk factors for de novo hemorrhage in asymptomatic hemispheres included AchA dilatation and choroidal anastomosis. Comparison of the RNF213 p.Arg4810Lys variant status showed no significant differences in baseline characteristics, onset types, or imaging findings, except for a higher percentage of patients in the GA group with a family history of MMD. CONCLUSIONS: Hemorrhagic events were the most prevalent and prognostically deteriorating factors in older patients with MMD aged ≥60 years. AchA dilatation and choroidal anastomosis were predictors of de novo hemorrhage in asymptomatic nonsurgical hemispheres in older patients with MMD.

Suzuki Stage VI Unilateral Moyamoya Disease Presenting With Massive Intraventricular Hemorrhage.

Moyamoya disease (MMD) is characterized by stenosis of the terminal portion of the internal carotid artery (ICA) and the development of collateral vessels. In late Suzuki stage MMD, ICA almost disappears, and the moyamoya vessels gradually regress. We report a case of late Suzuki stage unilateral MMD presenting with intraventricular hemorrhage. A 76-year-old woman who had previously been diagnosed with right ICA occlusive disease was referred to our hospital due to impaired consciousness. Radiological evaluation revealed massive intraventricular hemorrhage. After endoscopic hematoma removal, digital subtraction angiography (DSA) was performed to examine the vascular anatomy, which revealed numerous basal moyamoya vessels originating from the posterior cerebral artery. Three-dimensional rotational angiography identified a choroidal anastomosis originating from the posterior choroidal artery as the hemorrhage source. The patient had an RNF213 p.Arg4810Lys heterozygous variant in the germline. Based on the DSA findings, MMD was diagnosed, and the patient was transferred to a rehabilitation hospital with good postoperative consciousness. In conclusion, patients diagnosed with ICA occlusive disease may have late Suzuki stage MMD, potentially leading to major hemorrhage; therefore, antithrombotic medications should be administered with caution. In diagnosing ICA occlusive disease, the assessment of periventricular anastomosis should be considered, taking into account the possibility of MMD.

Double-catheter technique for the embolization of recurrent cerebral aneurysms: A single-center experience.

Background: Recurrent cerebral aneurysms have complex shapes and are often technically challenging to treat with a single microcatheter. This study evaluates the clinical characteristics and treatment outcomes of patients who received double-catheter coil embolization for recurrent cerebral aneurysms. Methods: Patients who underwent double-catheter coil embolization at our institution between April 2011 and March 2022 for recurrent aneurysms were included in the study. Baseline characteristics, course to recurrence, details of the procedures, and outcomes after endovascular treatment were retrospectively analyzed based on past medical records. Results: Eight patients with recurrent aneurysms were treated with the double-catheter technique. One patient had a subarachnoid hemorrhage due to a rupture of a recurrent aneurysm and the others had radiological recurrence during follow-up. The initial treatment for the aneurysm was clipping in one case and coiling in seven cases. All the aneurysms were located at bifurcation sites. During retreatment, balloon remodeling technique was used in five cases. Angiographic features immediately after the treatment included complete occlusion in one case, neck remnant in three cases, and dome filling in four cases. There were no procedure-related severe complications, besides preexisting oculomotor nerve palsy due to the mass effect of the aneurysm worsened in one patient. The mean follow-up period after retreatment was 4.3 years. There was one case of recurrence after retreatment in which additional endovascular coiling was necessary. Conclusion: This study demonstrated that the double-catheter technique could be a safe and useful treatment option for patients with recurrent aneurysms at bifurcation sites.

Orbital Arteriovenous Fistula Causing Only Visual Impairment due to Compression of the Optic Nerve by the Dilated Superior Ophthalmic Vein.

Orbital arteriovenous fistula (AVF) is a rare disease, and its standard therapeutic strategy has not been established. A 70-year-old male consulted an ophthalmologist due to a visual field defect in his left eye. Neurological findings showed visual impairment but no symptoms such as exophthalmos, conjunctival congestion, or diplopia. Magnetic resonance imaging showed marked dilation of the left superior ophthalmic vein (SOV). Cerebral angiography revealed an AVF that was limited to the left orbit. The feeder was a branch of the ophthalmic artery that originated from the first portion, and the drainer was the SOV, which was meandering and significantly dilated. Since the only symptom was visual impairment, the etiology was considered to be compression of the optic nerve due to a dilated SOV rather than increased venous pressure. Transvenous embolization via the facial vein was performed, and a visual field examination 1 week after the operation revealed marked improvement. Orbital AVF that develops only with visual impairment is extremely rare. As demonstrated with this case, coil embolization for proper position and reduction of the venous pressure, which relieves compression on the optic nerve, may be useful in improving the visual impairment.

Bypass Surgery for Adult-Onset Hemorrhagic Moyamoya Disease: Analysis Classified by Site of Initial Bleeding.

OBJECTIVE: Prevention of rebleeding events is crucial for patients with hemorrhagic moyamoya disease (MMD), as these increase the risk of mortality. Bypass surgery is effective in preventing subsequent hemorrhage, particularly in patients with posterior hemorrhage, but its efficacy in those with anterior hemorrhage remains unclear. We analyzed the effects of surgical intervention, stroke risk factors, and radiological features on rebleeding events. METHODS: Patients with hemorrhagic-onset MMD who were followed at our institution between 2000 and 2022 were included (41 adult patients, 45 hemispheres). Baseline characteristics and radiological features (anterior or posterior hemorrhagic site, Suzuki grade, posterior cerebral artery involvement, and periventricular anastomosis) were thoroughly reviewed. RESULTS: Of the 45 hemispheres, hemorrhage developed in 9 (20%) hemispheres, with a median duration until rebleeding of 38 (range: 1-44) months. Rebleeding rates were significantly lower in the surgical group than in the nonsurgical group (odds ratio: 0.09; 95% confidence interval [CI]: 0.01-0.79; P = 0.011), and Kaplan-Meier analysis revealed a significantly longer interval between bleeding events in the surgical group (1.3%/y vs. 5.3%/y; P = 0.002), especially in the anterior hemorrhage group (1.3%/y vs. 5.1%/y; P = 0.019). The hazard ratio of surgical intervention for rebleeding with initial anterior hemorrhage was 0.11 (95% CI: 0.01-0.98; P = 0.048). In the nonsurgical group, the presence of hypertension shortened the time to subsequent hemorrhage (P = 0.004). CONCLUSIONS: Surgical intervention may decrease the risk of rebleeding in hemorrhagic onset MMD patients, even in those presenting with anterior hemorrhage. Hypertension was a significant risk factor for rebleeding in nonsurgical patients.

Cerebrovascular Events During Treatment for Systemic Malignant Tumors in Patients with Moyamoya Disease.

OBJECTIVE: With the increasing incidence of malignancies, the importance of cancer-associated stroke is emphasized. Although moyamoya disease is a leading cause of stroke, no reports have documented cancer-associated stroke in patients with this condition. We aimed to investigate cerebrovascular events during malignancy treatments in patients with moyamoya disease. METHODS: A total of 405 patients with moyamoya disease who visited our hospital between January 2000 and March 2022 were retrospectively examined. We evaluated the management of moyamoya disease, presence of the ring finger protein 213 p.Arg4810Lys variant, treatments for malignant tumors, presence of cerebrovascular events during treatment, and follow-up periods and outcomes. RESULTS: Among the 405 patients, 17 patients with moyamoya disease (4.2%) were diagnosed with malignancies. Among patients aged 60 years and over, 7 out of 67 (10.4%) had malignancies. Of the 17 patients, 11 (64.7%) were symptomatic, and 7 (41.2%) had revascularization surgery. 9 patients were treated with oral antiplatelet drugs. There was no significant difference between the groups with and without malignancy regarding the presence of the ring finger protein 213 p.Arg4810Lys variant (80.0% vs. 62.7%, P = 0.33). All patients underwent surgical treatment, and 7 (41.2%) received chemotherapy. One death due to tumor progression was reported. No cerebrovascular event was observed during malignancy treatments and follow-up periods, which had a mean duration of 6 years. CONCLUSIONS: In our cohort, malignancy treatments in patients with moyamoya disease were safely conducted without cerebrovascular events. However, it is advisable to avoid hypotension, dehydration, hyperventilation, and long-term discontinuation of antiplatelet drugs during the treatment of malignant tumors.

Clinical and Genetic Characteristics of Patients with Moyamoya Disease who Experienced Both Ischemic and Hemorrhagic Events.

OBJECTIVE: Cerebrovascular events in moyamoya disease are mainly classified into ischemic or hemorrhagic onset. It is rare for one patient to develop both ischemia and hemorrhage in moyamoya disease; detailed clinical course and genetic characteristics of such patients have not been elucidated. We aimed to clarify the clinical features of patients with both ischemic and hemorrhagic cerebrovascular events. METHODS: We analyzed the background factors, radiological features, and genotype of ring finger protein 213 c.14429 G > A (p.Arg4810Lys) of patients with moyamoya disease who visited our hospital between 1996 and 2020, and experienced both ischemic and hemorrhagic cerebrovascular events. Additionally, we analyzed factors that caused subsequent hemorrhage in adult-onset ischemic moyamoya disease. RESULTS: Of 262 patients, 12 presented with both ischemia and hemorrhage, of which, 4 exhibited pediatric onset and 8 had adult onset. In pediatric-onset subjects, ischemia was the initial event in all cases. Hemorrhagic events occurred at a median of 24.7 years postoperatively in patients who had undergone bypass surgery. In adult-onset subjects, ischemia preceded hemorrhage in 7 patients. In males, the interval to subsequent hemorrhage was significantly shorter for adult-onset ischemic moyamoya disease, and the hazard ratio for hemorrhagic events was 5.45. The ring finger protein 213 p.Arg4810Lys heterozygous variant was present in 9 patients. CONCLUSIONS: A majority of patients with moyamoya disease with both ischemia and hemorrhage experience an ischemic event first. Patients who developed ischemia in childhood may develop subsequent hemorrhage in approximately 20-25 years after bypass surgery. Male sex is a risk factor for a subsequent hemorrhagic event in adult-onset ischemic moyamoya disease.

STA-A3 Bypass Using Radial Artery Graft for Progressive Cerebral Infarction of Bilateral ACA Region after STA-MCA Bypass Surgery for Moyamoya Disease.

Direct revascularization surgery, such as superficial temporal artery (STA)-middle cerebral artery (MCA) bypass, is effective in preventing ischemia and hemorrhage for moyamoya disease. On the other hand, when ischemia of the anterior cerebral artery (ACA) region progresses after ipsilateral STA-MCA bypass, it is difficult to perform revascularization from the viewpoint of the donor artery. A 55-year-old woman with right hemiparesis was diagnosed with cerebral infarction due to moyamoya disease. Left STA-MCA bypass was performed with no postoperative complications, but memory impairment and decreased motivation were observed 2 months after the operation. Magnetic resonance imaging and angiography revealed new infarction in the bilateral ACA area and deterioration in the signal intensity of bilateral ACAs. Revascularization of the bilateral ACA regions was considered necessary, but the left STA was already used in the previous surgery. Therefore, STA-radial artery (RA)-A3 bypass using RA graft combined with right STA-MCA bypass was performed. STA-A3 bypass using an RA graft may be the optimal treatment for ischemia of the ACA region that progresses after STA-MCA bypass.

The Role of Stereotactic Frame-Based Biopsy for Brainstem Tumors in the Era of Molecular-Based Diagnosis and Treatment Decisions.

Stereotactic frame-based brain tumor biopsy (SFB) is a potent diagnostic tool considering its minimal invasiveness, though its diagnostic power and safety for brainstem lesions remain to be discussed. Here, we aimed to examine the usefulness of SFB for brainstem tumors. Twenty-two patients with brainstem tumors underwent 23 SFBs at our institution during 2002-2021. We retrospectively analyzed patient characteristics, tumor pathology, surgical procedures, and outcomes, including surgery-related complications and the diagnostic value. Seven (32%) tumors were located from the midbrain to the pons, eleven (50%) in the pons only, and four (18%) from the pons to the medulla oblongata. The target lesions were in the middle cerebellar peduncles in sixteen procedures (70%), the cerebellum in four (17%), the inferior cerebellar peduncles in two (9%), and the superior cerebellar peduncles in one (4%). A definitive diagnosis was made in 21 patients (95%) at the first SFB. The diagnoses were glioma in seventeen (77%) cases, primary central nervous system lymphoma in four (18%), and a metastatic brain tumor in one (5%). The postoperative complications (cranial nerve palsy in three [13%] cases, ataxia in one [4%]) were all transient. SFB for brainstem tumors yields a high diagnostic rate with a low risk of morbidity.

RNF213 p.Arg4810Lys Heterozygosity in Moyamoya Disease Indicates Early Onset and Bilateral Cerebrovascular Events.

The relationship between RNF213 c.14429G > A (p.Arg4810Lys) heterozygous variants and clinical manifestation in patients with Moyamoya disease (MMD) remains unclear. We performed a retrospective cohort analysis to clarify the genotype-phenotype correlation of this RNF213 hotspot variant in MMD patients, especially between wild-type (GG) and heterozygous (GA) genotypes. Clinical and genetic data were obtained from patients diagnosed with MMD in our institutions between October 2011 and November 2020. Clinical data included age, sex, neurological status at diagnosis, medical history, smoking history, alcohol intake, and family history. Of the 225 enrolled patients, 160 (71.1%) were symptomatic, 3 (1.3%) had the homozygous variant, and 149 (66.2%) had the heterozygous variant (GA). Analysis of all enrolled patients showed that the GA group was prone to present bilateral symptoms (p = 0.008) and progressive status (Suzuki grade ≥ 4; p = 0.017). Analysis limited to symptomatic patients revealed that the GA group had bilateral symptoms (p = 0.017), younger age at onset (p = 0.043), and, in particular, a higher proportion of onset before 25 years of age (p = 0.021). Multivariate logistic regression analysis of overall patients revealed that earlier age at diagnosis (p < 0.001, OR 0.936, 95% CI 0.914-0.959) and GA group (p = 0.017, OR 3.326, 95%CI 1.237-8.941) were significantly associated with bilateral symptoms. MMD patients diagnosed at a young age with the RNF213 heterozygous variant should be followed up with consideration of possible contralateral stroke if one hemisphere is already symptomatic or of early cerebrovascular events if bilateral hemispheres are asymptomatic.

Clinical Features and Prognostic Factors for Primary Anaplastic Large Cell Lymphoma of the Central Nervous System: A Systematic Review.

Primary anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) is a subtype of primary CNS lymphoma (PCNSL). There are very few comprehensive reports on this extremely rare tumor. Therefore, it is necessary to investigate the clinical features and prognostic factors for primary ALCL of the CNS. We performed a systematic review of the published literature. Past cases were comprehensively searched using PubMed, Cochrane Library, and Web of Science. Clinical information, such as age, sex, anaplastic lymphoma kinase (ALK) status, lesion sites, treatment methods, and survivorship were extracted. Thirty-nine cases with information on ALK status and treatment course were identified. The average observation period was 13 months, and the overall 2-year survival rate was 58%. Univariate analyses showed a statistically significantly better prognosis among patients < 40 years of age (p = 0.039, HR 0.32 (0.11-0.95)) and in relation to ALK positivity (p = 0.010, HR 0.24 (0.08-0.71) and methotrexate treatment (p = 0.003, HR 0.17 (0.05-0.56)). Because of the sparsity of cases, it is necessary to accumulate cases in order to perform more detailed analyses.

Differences in Clinical Features among Different Onset Patterns in Moyamoya Disease.

Moyamoya disease is characterized by severe stenosis at the ends of the bilateral internal carotid arteries and the development of collateral circulation. The disease is very diverse in terms of age at onset, onset patterns, radiological findings, and genetic phenotypes. The pattern of onset is mainly divided into ischemic and hemorrhagic onsets. Recently, the opportunity to identify asymptomatic moyamoya disease, which sometimes manifests as nonspecific symptoms such as headache and dizziness, through screening with magnetic resonance imaging has been increasing. Various recent reports have investigated the associations between the clinical features of different onset patterns of moyamoya disease and the corresponding imaging characteristics. In this article, we have reviewed the natural history, clinical features, and imaging features of each onset pattern of moyamoya disease.