Wasp sting-induced bilateral thalamic and midbrain infarction.

A male patient in his early 40s presented to the emergency department with an acute onset of respiratory distress and facial oedema, indicative of anaphylaxis. These symptoms emerged 2 hours subsequent to a wasp sting on the left side of his face. Despite initial stabilisation, the patient's state deteriorated into somnolence and disorientation. Notably, he denied any history of seizures, sensory or motor deficits, or bowel/bladder complications. Physical examination unveiled no focal neurological deficits. Routine laboratory tests and drug screening yielded no significant findings. Subsequent brain MRI with angiography exposed bilateral thalami diffusion restriction, strongly implying an acute infarction within the artery of Percheron territory, an atypical vascular variant. The sequence of events, alongside the absence of other conclusive aetiologies, indicated a wasp sting-induced thalamic infarction driven by vasogenic and thrombogenic effects of inflammatory substances.

Pediatric frequent relapsing nephrotic syndrome with multiple cerebral infarctions accompanied by patent foramen ovale and cerebral venous sinus thrombosis: a case report.

BACKGROUND: Idiopathic nephrotic syndrome (NS) presents as a hypercoagulable state, of which thromboembolism (TE) is a well-known life-threatening complication. Although TE is more likely to occur in venous vessels than arterial vessels, arterial TE is important because it may cause after-effects, including tissue necrosis and cerebral infarction (CI); therefore, prompt diagnosis and appropriate treatment are required. We report a pediatric NS case with multiple CIs. CASE PRESENTATION: A 14-year-7-month-old Japanese girl was diagnosed with frequent relapsing NS, accompanied by headache and disturbance of consciousness during the second relapse. Brain magnetic resonance imaging (MRI) and four-dimensional computed tomography revealed multiple CIs, vasogenic edema, and cerebral venous sinus thrombosis (CVST). The patient had no underlying thrombophilia other than hypercoagulability due to NS and prednisolone (PSL), and no cardiac arrhythmia; however, a right-to-left shunt through the patent foramen ovale (PFO) was observed with the Valsalva maneuver by echocardiography. Therefore, we assumed that a potential cause of multiple CIs might be an embolic stroke, caused by thrombosis formed from a hypercoagulable state due to NS and PSL treatment and reached through PFO. Antiplatelet and anticoagulant therapies were administered for TE. She was treated with PSL and mycophenolate mofetil (MMF) for NS. Rituximab (RTX) was administered to prevent NS relapse after complete remission (CR). She underwent transcatheter PFO closure at age 14 years and 9 months because we considered that the right-to-left shunt through the PFO would be one of the risks for recurrent cerebral embolism when NS relapses. One year after the onset of CIs, an MRI indicated that the CVST had resolved, leaving no neurological sequelae due to CI; therefore, anticoagulant therapy was discontinued. And then she has been in CR for NS with only MMF therapy. CONCLUSIONS: CI is a serious complication in patients with NS. The pathogenesis of multiple CIs is various, including right-to-left shunt through PFO, in addition to the hypercoagulability due to NS. It is important to investigate and manage underlying risks such as PFO, besides preventing the relapses of NS by aggressive treatments using MMF and RTX, in patients with NS.

A novel score to predict progression in anterior circulation single subcortical infarction patients.

OBJECTIVE: Progressive infarction (PI) has a negative effect on functional prognosis. Our study aimed to develop and validate a risk score for predicting PI in patients with anterior circulation single subcortical infarction (ACSSI). METHODS: Between January 2020 and October 2022, we retrospectively enrolled 638 eligible patients with ACSSI. Two-thirds of the eligible patients were randomly allocated to the training cohort (n = 425). Another resampling sample was formed through the bootstrap method and was used as the validation group (n = 425). Multivariate logistic regression analysis was used to identify the independent factors associated with PI. Each factor was then point assigned based on ß-coefficient and a risk scoring system was developed. This scoring system was internally validated through 1000-bootstrap resamplings. The C-statistic and Hosmer-Lemeshow test were used to assess model discrimination and calibration. RESULTS: PI occurred in 121 patients, accounting for 19.0% of the total patients. A 7-point NTS score system based on the initial NIHSS score, triglyceride-glucose index, and the number of infarct slices on axial diffusion-weighted imaging was developed. The NTS score showed good discrimination and calibration in the training cohort (C-statistic = 0.686; p value of Hosmer-Lemeshow test = 0.797) and validation cohort (C-statistic = 0.681; p value of Hosmer-Lemeshow test = 0.451). The three risk levels for predicting PI in the training and validation cohorts based on NTS score were as follows: low (0-2, 9.6% vs. 9.3%), intermediate (3-5, 28.2% vs. 26.7%), and high risk (6-7, 60.2% vs. 57.4%). INTERPRETATION: The NTS score is a valid and convenient risk score for predicting PI in ACSSI patients.

[Dystypia in a patient with subcortical ischemic stroke].

A 58-year-old, right-handed man noticed difficulty in typing and speech. On day 3 after onset, the day of admission, he had frontal lobe dysfunction including verbal fluency impairment and impairment of recent memory, although he did not have apraxia or visual agnosia. Moreover, he had difficulty typing in romaji, especially words containing contracted or double consonant sounds, although he was able to do this before onset by visually checking the keyboard. He had mild dysgraphia. MRI showed an infarct in the genu and posterior limb of the left internal capsule. SPECT revealed low-uptake lesions in the left frontal lobe. In the present case, we consider that the subcortical infarction disrupted the network between the thalamus and frontal lobe, resulting in dystypia due to difficulty with recalling romaji spelling.

Risk Factors for Massive Cerebral Infarction in Pediatric Patients With Moyamoya Disease.

BACKGROUND: To explore the risk factors for preoperative massive cerebral infarction (MCI) in pediatric patients with moyamoya disease (MMD). METHODS: Pediatric patients with MMD treated between 2017 and 2022 were enrolled. Logistic regression analysis was performed to identify risk factors for MCI among the patients, and a nomogram was constructed to identify potential predictors of MCI. Receiver operating characteristic (ROC) curves and areas under the curves were calculated to determine the effects of different risk factors. RESULTS: This study included 308 pediatric patients with MMD, including 36 with MCI. The MCI group exhibited an earlier age of onset than the non-MCI group. Significant intergroup differences were observed in familial MMD history, postcirculation involvement, duration from diagnosis to initiation of treatment, Suzuki stage, magnetic resonance angiography (MRA) score, collateral circulation score, and RNF213 p.R4810K variations. Family history, higher MRA score, lower collateral circulation score, and RNF213 p.R4810K variations were substantial risk factors for MCI in pediatric patients with MMD. The nomogram demonstrated excellent discrimination and calibration capabilities. The integrated ROC model, which included all the abovementioned four variables, showed superior diagnostic precision with a sensitivity of 67.86%, specificity of 87.01%, and accuracy of 85.11%. CONCLUSIONS: This study showed that family history, elevated MRA score, reduced collateral circulation score, and RNF213 p.R4810K variations are risk factors for MCI in pediatric patients with MMD. The synthesized model including these variables demonstrated superior predictive efficacy; thus, it can facilitate early identification of at-risk patients and timely initiation of appropriate interventions.

Inflammation-associated D-dimer predicts neurological outcome of recent small subcortical infarct: A prospective clinical and laboratory study.

OBJECTIVE: Elevated level of D-Dimer often indicates a worse prognosis in cerebral infarction. However, there is limited research on this impact within recent small subcortical infarction (RSSI). We aim to explore the role of inflammation and the total magnetic resonance imaging (MRI) burden of cerebral small vessel disease (cSVD) in this process. METHODS: 384 RSSI patients and 189 matched healthy controls were strictly registered in the current research. We evaluated short-term and long-term outcomes by measuring the percentage of the National Institutes of Health Stroke Scale (NIHSS) improvement and the modified Rankin Scale (mRS) at 3 months, respectively. We also assessed the chronic, sustained brain damage associated with cSVD using the total MRI burden and confirmed the relationship between prognosis and the total MRI burden of cSVD. Furthermore, we explored the associations between D-dimer and C-reactive protein (CRP) levels with NIHSS improvement and mRS at 3 months, as well as their relationships with both the total MRI burden of cSVD and its 4 imaging features. RESULTS: Both NIHSS improvement and the mRS at 3 months were found to be correlated with the total MRI burden of cSVD. Higher D-dimer and CRP levels showed a linear correlation, indicating worse prognosis and a higher total MRI burden of cSVD. The four imaging features of the total MRI burden of cSVD did not exhibit entirely consistent patterns when exploring their correlations with prognosis and laboratory indicators. CONCLUSION: Inflammation-associated D-dimer predicts neurological outcomes in patients with recent small subcortical infarct, and reflects a more severe total MRI burden of cSVD.

FLAIR signal intensity ratio predicts small subcortical infarct early neurologic deterioration: a cross-sectional study.

PURPOSE: Prior studies have used the fluid-attenuated inversion recovery sequence signal intensity ratio (FLAIR-SIR) to predict those with an incomplete infarct that may safely receive acute thrombolytics. Clinical early neurologic deterioration (END) of small subcortical infarcts (SSIs) is suspected to occur due to delayed infarct completion. We aimed to understand if a lower FLAIR-SIR, suggestive of an incomplete infarct, would have a higher likelihood of SSI-related END. METHODS: A cross-sectional retrospective study was performed of those with an acute SSI (anterior or posterior circulation) without significant parent vessel steno-occlusive disease. END was defined as a new or worsened disabling neurologic deficit during the index hospitalization. Standard-of-care brain MRIs were reviewed from the hospitalization, and a FLAIR-SIR cutoff of ≤ 1.15 was used based on prior studies. Adjusted logistic regression models were used for analysis. RESULTS: We identified 252 patients meeting inclusion criteria: median (IQR) age 68 (12) years, 38.5% (97/252) female, and 11% (28/252) with END. Tobacco use was more common in those without END (32%) compared with END (55%, p = 0.03). In adjusted analyses, a FLAIR-SIR cutoff of ≤ 1.15 yielded an odds ratio of 2.8 (95% CI 1.23-6.13, p = 0.012) of early neurological deterioration. CONCLUSION: Those with a FLAIR-SIR ≤ 1.15 are nearly threefold more likely to develop SSI-related END.

Effect of the Factor XIa Inhibitor Asundexian According to Baseline Infarct Pattern and on MRI Covert Infarct Outcomes.

BACKGROUND: Exploratory analysis of the phase 2 PACIFIC-Stroke (Program of Anticoagulation via Inhibition of FXIa by the Oral Compound BAY 2433334-Non-Cardioembolic Stroke) randomized trial suggested that asundexian, an oral factor XIa inhibitor, prevents recurrent stroke and transient ischemic attacks in patients with atherosclerotic stroke. In this post hoc exploratory analysis, we hypothesized that asundexian would be more effective in patients enrolled with large, multiple, or cortical acute infarcts on magnetic resonance imaging than in patients enrolled with a single small subcortical acute infarct, and asundexian would prevent incident cortical covert infarcts. METHODS: In this placebo-controlled double-blinded randomized controlled trial, patients with mild-to-moderate noncardioembolic ischemic stroke were assigned to asundexian (10, 20, or 50 mg once daily) or placebo, in addition to antiplatelet therapy. Brain magnetic resonance imagings were required within 72 hours of randomization and repeated at 26 weeks or at discontinuation of the study drug. RESULTS: Of 1808 randomized patients, 1780 (98.5%) had interpretable baseline magnetic resonance imagings, of which 1628 (91.5%) had ≥1 diffusion-weighted imaging positive acute infarcts. Magnetic resonance imaging follow-up was obtained in 1439 patients, of whom 1358 had no symptomatic stroke during the trial period. Compared with placebo, asundexian 50 mg daily conferred a trend toward reduced risk of recurrent ischemic stroke or incident covert infarcts (hazard ratio, 0.71 [95% CI, 0.45-1.11]) and recurrent ischemic stroke or transient ischemic attack (secondary outcome; hazard ratio, 0.59 [95% CI, 0.33-1.06]) that was not evident in patients with single small subcortical infarcts (hazard ratios, 1.14 [95% CI, 0.62-2.10] and 0.93 [95% CI, 0.28-3.06]). Incident cortical covert infarcts were reduced in patients taking asundexian 50 mg, but the difference was not statistically significant (crude incidence ratio, 0.56 [95% CI, 0.28-1.12]). CONCLUSIONS: These exploratory, unconfirmed results suggest that asundexian may prevent new embolic infarcts but not small artery occlusion. The hypothesis that subtypes of covert brain infarcts respond differently to anticoagulant prevention should be tested in future trials. REGISTRATION: URL: https://clinicaltrials.gov; Unique identifier: NCT04304508.

Association between substantia nigra degeneration and functional outcome in patients with basal ganglia infarction.

BACKGROUND AND PURPOSE: Cerebral infarction in the basal ganglia may cause secondary and delayed neuronal degeneration in the substantia nigra (SN). However, the clinical significance of SN degeneration remains poorly understood. METHODS: This retrospective observational study included patients with acute ischemic stroke in the basal ganglia on initial diffusion-weighted imaging who underwent follow-up diffusion-weighted imaging between 4 and 30 days after symptom onset. SN degeneration was defined as a hyperintensity lesion in the SN observed on diffusion-weighted imaging. We compared functional outcomes at 3 months between patients with and without SN degeneration. A poor outcome was defined as a score of 3-6 (functional dependence or death) on the modified Rankin Scale. RESULTS: Of 350 patients with basal ganglia infarction (median age = 74.0 years, 53.7% male), 125 (35.7%) had SN degeneration. The proportion of functional dependence or death was 79.2% (99/125 patients) in patients with SN degeneration, which was significantly higher than that in those without SN degeneration (56.4%, 127/225 patients, p < 0.001). SN degeneration was more frequent in patients with functional dependence or death (99/226 patients, 43.8%) than in those with functional independence (26/124 patients, 21.0%, p < 0.001). Multivariable logistic regression analysis showed a significant association between SN degeneration and functional dependence or death (odds ratio = 2.91, 95% confidence interval = 1.17-7.21, p = 0.021). CONCLUSIONS: The study showed that patients with degeneration of SN were associated with functional dependence or death at 3 months, suggesting that secondary degeneration is a predictor of poor stroke outcomes and a potential therapeutic target.

A deep learning and radiomics based Alberta stroke program early CT score method on CTA to evaluate acute ischemic stroke.

BACKGROUND: Alberta stroke program early CT score (ASPECTS) is a semi-quantitative evaluation method used to evaluate early ischemic changes in patients with acute ischemic stroke, which can guide physicians in treatment decisions and prognostic judgments. OBJECTIVE: We propose a method combining deep learning and radiomics to alleviate the problem of large inter-observer variance in ASPECTS faced by physicians and assist them to improve the accuracy and comprehensiveness of the ASPECTS. METHODS: Our study used a brain region segmentation method based on an improved encoding-decoding network. Through the deep convolutional neural network, 10 regions defined for ASPECTS will be obtained. Then, we used Pyradiomics to extract features associated with cerebral infarction and select those significantly associated with stroke to train machine learning classifiers to determine the presence of cerebral infarction in each scored brain region. RESULTS: The experimental results show that the Dice coefficient for brain region segmentation reaches 0.79. Three radioactive features are selected to identify cerebral infarction in brain regions, and the 5-fold cross-validation experiment proves that these 3 features are reliable. The classifier trained based on 3 features reaches prediction performance of AUC = 0.95. Moreover, the intraclass correlation coefficient of ASPECTS between those obtained by the automated ASPECTS method and physicians is 0.86 (95% confidence interval, 0.56-0.96). CONCLUSIONS: This study demonstrates advantages of using a deep learning network to replace the traditional template registration for brain region segmentation, which can determine the shape and location of each brain region more precisely. In addition, a new brain region classifier based on radiomics features has potential to assist physicians in clinical stroke detection and improve the consistency of ASPECTS.

Ischemic Stroke with Positive Antiphospholipid Antibodies in Bloom Syndrome: A Case Report.

OBJECTIVE: Bloom syndrome is a chromosomal breakage disorder associated with immune deficiency, characterized by short stature, predisposition to early-onset cancer, and immune defects. Currently, there have been no reports of acute cerebral infarction in patients with Bloom syndrome. Here, we report a case of Bloom syndrome complicated by elevated antiphospholipid antibodies and acute cerebral infarction. MATERIALS AND METHODS: A 23-year-old male with a known genetic diagnosis of Bloom syndrome was admitted to the Respiratory Department due to pulmonary aspergillosis. The patient experienced sudden dizziness, and subsequent cranial MRI revealed a newly developed infarction in the right cerebellar hemisphere. RESULTS: Six days later, the patient presented with sudden right visual field loss, and a repeat cranial MRI showed new infarctions in the left occipital and temporal lobes. Positive lupus anticoagulant and prolonged activated partial thromboplastin time suggested elevated antiphospholipid antibodies causing thrombus formation. Unfortunately, anticoagulant treatment was not administered due to recurrent hemoptysis. CONCLUSION: This study reports the first case of a Bloom syndrome patient with elevated antiphospholipid antibodies and acute cerebral infarction, suggesting that the immune and coagulation abnormalities caused by Bloom syndrome may contribute to the development of acute cerebral infarction.

Cerebral infarction in Behçet's syndrome caused by obliterative focal arteritis.

We present a case in which cerebral infarction was seen and Behcet's syndrome was diagnosed. Following investigation and monitoring, we identified that the cause was a progressive obliterative focal arteritis of the middle cerebral artery. Such an entity has not previously been reported, although similar cases are described which explain the pathophysiology of the disorder.

Prolonged paradoxical reaction requiring over 5 years of corticosteroid administration in a patient with severe tuberculous meningitis.

Tuberculous meningitis (TBM) is a rare disease in low-incidence countries like Japan, where general physicians have fewer experience with TBM. Despite its proper treatment and early improvement of the condition, TBM often causes paradoxical reactions (PRs), which can lead to severe complications such as stroke. As PRs in the brain are difficult to detect without regular neuroimaging surveillance and have a later onset than in other organs, delayed treatment can be fatal. We report a case of a 54-year-old, human immunodeficiency virus (HIV)-negative man who presented with TBM and miliary tuberculosis (TB) in an unconscious state. Standard anti-tuberculous therapy with adjunctive systemic high-dose dexamethasone brought rapid clinical and microbiological improvement, which allowed the dexamethasone to be tapered. However, he developed cerebral infarction with left hemiplegia due to a TBM-related PR five months after admission. Therefore, the initial high-dose dexamethasone was again added to the anti-tuberculous drugs, achieving the significant effects on the PR-related lesions. Anti-tuberculous drugs had been administered for 3 years and the dexamethasone was carefully tapered. Nevertheless, enlargement of PR-related lesions in the brain recurred 5 years later. Accordingly, the dose of corticosteroid was again increased, resulting in resolving the lesions. It is important to note that severe TBM may cause prolonged PRs, which require a long-term neuroimaging follow-up and anti-inflammatory drugs for the successful management of the TBM-related PR.

Iatrogenic Air Embolisms During Endovascular Interventions: Impact of Origin and Number of Air Bubbles on Cerebral Infarctions.

PURPOSE: Cerebral infarctions caused by air embolisms (AE) are a feared risk in endovascular procedures; however, the relevance and pathophysiology of these AEs is still largely unclear. The objective of this study was to investigate the impact of the origin (aorta, carotid artery or right atrium) and number of air bubbles on cerebral infarctions in an experimental in vivo model. METHODS: In 20 rats 1200 or 2000 highly calibrated micro air bubbles (MAB) with a size of 85 µm were injected at the aortic valve (group Ao), into the common carotid artery (group CA) or into the right atrium (group RA) using a microcatheter via a transfemoral access, resembling endovascular interventions in humans. Magnetic resonance imaging (MRI) using a 9.4T system was performed 1 h after MAB injection followed by finalization. RESULTS: The number (5.5 vs. 5.5 median) and embolic patterns of infarctions did not significantly differ between groups Ao and CA. The number of infarctions were significantly higher comparing 2000 and 1200 injected MABs (6 vs. 4.5; p < 0.001). The infarctions were significantly larger for group CA (median infarction volume: 0.41 mm3 vs. 0.19 mm3; p < 0.001). In group RA and in the control group no infarctions were detected. Histopathological analyses showed early signs of ischemic stroke. CONCLUSION: Iatrogenic AEs originating at the ascending aorta cause a similar number and pattern of cerebral infarctions compared to those with origin at the carotid artery. These findings underline the relevance and potential risk of AE occurring during endovascular interventions at the aortic valve and ascending aorta.

Recurrent Cerebral Infarction Due to Moyamoya Disease Complicated With Systemic Lupus Erythematosus: A Case Report and Literature Review.

INTRODUCTION: We report a rare case of moyamoya disease caused by an RNF213 mutation, complicated with systemic lupus erythematosus. CASE REPORT: A 32-year-old woman experienced 4 cerebral ischemia stroke events within 6 months. The main symptom was left limb weakness with blurred vision in the right eye. Results of digital subtraction angiography conducted at another hospital were consistent with moyamoya disease. On genetic testing, we found that the patient carried 2 mutations in the moyamoya disease-related gene RNF213 (p.R4810K, p.T1727M). On the basis of the laboratory immunologic indicators, such as positive antibodies and abnormal immunoglobulin levels and imaging examinations, the patient was finally diagnosed as moyamoya disease complicated with systemic lupus erythematosus. She was treated with aspirin, butylphthalide, urinary kallidinogenase, and sodium methylprednisolone. CONCLUSIONS: This was a 32-year-old young patient diagnosed with moyamoya disease carrying RNF213 gene mutation and accompanied by lupus with cerebral ischemic event as the first occurrence. The patient's condition was complex; therefore, comprehensive analysis and in-depth consideration were needed to avoid a missed diagnosis and misdiagnosis. When the primary disease cannot be identified, genetic testing can help to clarify the diagnosis of moyamoya disease.

The Piano Man: A Case Report of Anterior Thalamic Infarct with Dementia and Preserved Music Ability.

OBJECTIVE: The thalamus is the integrative hub of the brain with reciprocal connections throughout the cortex. This case report describes a right-handed 81-year-old male patient who experienced sudden onset cognitive impairment following a focal left anterior thalamic infarct. METHODS: With consent/assent, the patient was seen for a short neuropsychological assessment 6 weeks post stroke. Neuropsychological assessment included review of medical history, collateral intake, the Toronto Cognitive Assessment, Frontal Systems Behavior Scale-Family Rating Form, the Neuropsychiatric Inventory Questionnaire, and piano performance. RESULTS: The assessment revealed impaired performance on measures of orientation, memory, executive function, and language, as well as symptoms including hallucinations, apathy, and hypersomnolence, consistent with thalamic dementia. Remarkably, in this context, the patient maintained an ability to play piano and read music. CONCLUSIONS: The case has implications for understanding the complex integrative functions of the thalamus, including how profound impairment can simultaneously present with cognitive strengths that may not be captured by performance on neuropsychological testing. This case also suggests that magnetic resonance imaging may be indicated in cases presenting with vascular risk factors and sudden onset cognitive impairment, given that computed tomography may not be sensitive to small subcortical infarcts.