Citations

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease with fluctuating activity that requires long-term visual documentation to evaluate disease severity and treatment response. A standardized protocol for photographing HS is critically needed. Objectives: To establish a standardized protocol for using photography to clinically document HS. Methods: We brought together a panel of international experts and used a modified Delphi process to reach consensus on statements about standardized practices for using photography to document HS in clinical practice and research settings. A 75% agreement level was prespecified as the cutoff for consensus. Results: Consensus for photographically documenting HS was developed within 6 key areas: patient preparation, background, lighting, size and reference scale, camera considerations, and patient positioning. The revised and optimized protocol includes 34 standardized reference illustrations. However, the proposed recommendations are for ideal scenarios, and their implementation may not always be practical in a clinical setting. Variations in resources may not have been captured. Conclusions: The proposed consensus-based protocol provides a standard for consistent photographic documentation of HS. This protocol may help clinicians provide better disease surveillance and patient care, and it may aid in more precise and reliable research and clinical trials for HS.

Abstract Objective To assess the localization and lateralization values of ictal emotional features and ictal defensive behaviors in pediatric and young adult patients with frontal lobe epilepsy (FLE). Methods Children and young adults with FLE were identified from a single institution's epilepsy surgery database. Two epileptologists determined by consensus the presence of ictal emotional features (positive‐ smile, laughter, humming, singing; negative‐ fear, crying, anger, disgust, contempt, sadness) and ictal defensive behaviors (freezing with a fearful facial expression, shielding the face with hands, burying the face in a pillow, or exhibiting hyperkinetic motor behaviors like attempting to escape from bed or resisting restraint) in each patient's first video EEG (vEEG). Historic seizure semiology and vEEG reports were compared with the consensus vEEG analysis. Longitudinal changes in ictal emotional features were defined by the emergence or resolution of ictal emotional features between historic seizure semiology and re‐review of presurgical vEEG analysis. Subgroup analyses were conducted according to the seizure lateralization (left vs. right) and sublobar localization (precentral, medial, lateral, extensive) based on surgical extent. Results Twenty‐three FLE patients were included (female 39%; mean age at vEEG analysis: 11.28 years, range 1.75–20.17). The inter‐rater reliability was very good for both ictal emotional features ( κ = 0.81, 95% CI: 0.59–1) and ictal defensive behaviors ( κ = 0.91, 95% CI: 0.79–1). Ictal emotional features were observed in 10 patients (43.4%), with negative emotions present in 70% (7/10) of these cases. vEEG reports lacked documentation of ictal emotional features in two patients. Although ictal emotional features showed no statistical correlation by sublobar regions, ictal defensive behaviors were consistently seen in medial compared to lateral FLE (100% vs. 16.6%; p = 0.004). Longitudinal changes in ictal emotional features were observed in 8 (34.7%) patients. Significance Ictal emotional features were common in pediatric and young adult FLE patients and were mainly negative. Ictal defensive behaviors were more likely associated with medial FLE. Longitudinal changes in ictal emotional features occurred in about a third of pediatric and young adult patients with FLE. Plain Language Summary This study examined emotional features and defensive behaviors during seizures in children and young adults with FLE. Negative emotions were commonly seen during seizures. Ictal defensive behaviors were more frequently observed in patients with medial FLE, and recognizing these signs could help clinicians localize the seizures. Changes in emotional features were seen over time in about one‐third of children and young adult patients.

PURPOSE Stereotactic radiation (SRS/SRT) as opposed to whole-brain radiation (WBRT) represents the standard of care for patients with a limited number of brain metastases given the relatively favorable toxicity profile associated with stereotactic treatment. However, in patients with small cell lung cancer (SCLC), WBRT remains standard because of a lack of prospective data supporting SRS/SRT and concerns related to intracranial progression and neurologic death when WBRT is omitted. We conducted a single-arm, multicenter, phase II trial of SRS/SRT in patients with SCLC and 1-10 brain metastases to assess neurologic death rates relative to historical controls managed with WBRT (ClinicalTrials.gov identifier: NCT03391362 ). METHODS Patients were eligible if they had SCLC or an extrathoracic small cell primary and 1-10 brain metastases. Previous brain-directed radiation including prophylactic cranial irradiation was not permitted. Neurologic death was defined as marked, progressive, radiographic brain progression accompanied by corresponding neurologic symptomatology without systemic disease progression or systemic symptoms of a life-threatening nature. Close imaging-based surveillance of the brain post-SRS/SRT was used. RESULTS Between February 2018 and April 2023, 100 patients were enrolled. The median number of brain metastases was 2 (IQR, 1-4; range, 1-10). The median overall survival was 10.2 months; only 22% of patients required salvage WBRT. In total, 20 neurologic deaths were observed, relative to 64 non-neurologic deaths. The neurologic death rate at 1 year was 11.0% (95% CI, 5.8 to 18.1); the historical rate in patients managed with WBRT was 17.5%. CONCLUSION Our prospective, multi-institutional study demonstrated low rates of neurologic death when SRS/SRT as opposed to WBRT is used in patients with SCLC and 1-10 brain metastases who are surveilled closely post-treatment, supporting the utility of stereotactic approaches in this population.

ABSTRACT Systemic lupus erythematosus (SLE) is an enigmatic autoimmune disease with a varied presentation that carries with it an increased risk for many potentially life‐threatening problems. When compared with the general population, the relative risk of ischemic stroke in SLE is more than doubled. Our case report highlights a rare complication of SLE, vasculitis as the cause of stroke. CNS vasculitis secondary to SLE most commonly affects the endothelium of small and medium vessel arteries through perivascular inflammation in a concentric pattern. We present a case of a 46‐year‐old female with a history of hypertension, hyperlipidemia, hypothyroidism, multiple ischemic strokes (residual left sided weakness), and elevated cardiolipin IgM levels (not on anticoagulation) who presented with left facial numbness, dysarthria, and word finding difficulty. The initial CT head revealed chronic appearing hypodensities in the right parietal and occipital lobes without hemorrhage. CT angiogram of the head and neck demonstrated multifocal stenosis in the right MCA and right PCA. She received IV tenecteplase and MRI brain revealed an infarct in the left MCA distribution along with infarcts in the right thalamus, hippocampus, and cerebellum. Transesophageal echocardiogram was negative for Libman‐Sacks endocarditis. Given concerns for an underlying vasculitis process, an MRA vessel wall imaging was obtained which demonstrated concentric enhancement and persistent vessel narrowing in the left MCA M2 along with symmetric concentric enhancement of vaso vasorum of intracranial vertebral arteries. Lumbar puncture revealed CSF protein 60, culture negative, glucose 61, white blood cells 3, IgG index 0.5, ACE negative, and no oligoclonal bands. Her ESR was 43 and CRP < 0.5. Hypercoagulability workup demonstrated RNP > 8, SSA 6.4, Smith 1.6, +ANA 1:1280, C4 11, cardiolipin IgM 60. Given serum ANA titers and anti‐cardiolipin IgM labs in the setting of intracranial vessel wall enhancement concerning for an inflammatory process, it was believed that this stroke was actually a result of lupus vasculitis. The paucity of literature on the association between SLE vasculitis and cerebrovascular disease presents an important opportunity for more research, and it may be prudent to further investigate the relative risk of stroke in patients with known SLE vasculitis. This is also important because young individuals with SLE are at a higher relative risk of stroke.

Abstract Aims To assess the performance of an artificial intelligence-enabled electrocardiogram (AI-ECG) algorithm in identifying patients with moderate to severe aortic stenosis (AS) in an Asian cohort from a tertiary care centre. Methods and results We identified a randomly selected patients ≥60 years old who underwent echocardiography and ECG within in 31 days between 2012 and 2021 at the Samsung Medical Center in Korea. Patients with previous cardiac surgery, prosthetic valves, or pacemakers were excluded. The AI-ECG model, originally developed and validated by Mayo Clinic in the USA, was applied without fine-tuning. Performance metrics, including the area under the curve (AUC), sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy, were calculated to compare AI-ECG predictions with TTE-confirmed AS status. Among 5425 patients, 1095 had moderate to severe AS, and 4330 age- and sex-matched patients without AS were included as controls. The AI-ECG model achieved an AUC of 0.85 (95% CI: 0.84–0.87) in detecting moderate to severe AS. Sensitivity, specificity, PPV, NPV, and accuracy were 0.83, 0.65, 0.37, 0.94, and 68.29%, respectively. The model's performance was consistent across various age and sex subgroups, with sensitivity increasing in older patients. Conclusion The AI-ECG model developed in the USA demonstrated comparable performance in detecting moderate to severe AS in an Asian cohort compared with its original validation population. These findings highlight the potential utility of AI-ECG as a non-invasive screening tool for AS across diverse patient populations.

Abstract Background We examined how the COVID-19 pandemic influenced influenza vaccination, healthcare engagement, and patient satisfaction. Methods We retrospectively analyzed influenza vaccination records and patient experience surveys of adult primary care patients in southeastern Minnesota during two 3-year phases: Prepandemic (1 January 2017–28 February 2020) and Pandemic-Plus (1 March 2020–31 December 2023). Vaccination status was defined as “always vaccinated” (AV), “never vaccinated” (NV), or “intermittently vaccinated” (IV) for seasonal influenza. Results During the Pandemic-Plus phase, 7.0% (n = 3556) of the Prepandemic AV and 48.7% (n = 16 710) of the Prepandemic IV patients never received another influenza vaccine. Compared to AV, IV and NV patients were more likely to reside in areas with greater socioeconomic deprivation (odds ratio [OR], 1.58 [95% confidence interval {CI}, 1.53–1.62] and 1.99 [1.94–2.05], respectively), have a high school education or less (2.86 [2.74–2.98] and 3.38 [3.23–3.53]), and report healthcare disengagement (1.59 [1.55–1.64] and 4.21 [4.09–4.33]). After adjusting for Area Deprivation Index and medical comorbidities, healthcare disengagement increased among the NV versus AV between phases (3.33 [3.24–3.41] and 4.23 [4.10–4.35]). In a subgroup analysis those with severe comorbidities were less likely AV (NV vs AV: 1.21 [1.14–1.27]) and more dissatisfied with care (NV vs AV: 1.25 [1.18–1.33]). Conclusions The COVID-19 pandemic altered vaccination behaviors and healthcare satisfaction, especially among those at high risk of developing influenza-related complications. Medical providers and public health officials should be aware of factors associated with vaccine refusal to better target interventions.

Pediatric central nervous system tumors are the leading cause of cancer-related deaths in children. The five-year survival rate for high-grade glioma in children is less than 20%. The development of new treatments is dependent upon multi-institutional collaborative clinical trials requiring reproducible and accurate centralized response assessment. We present the results of the BraTS-PEDs 2023 challenge, the first Brain Tumor Segmentation (BraTS) challenge focused on pediatric brain tumors. This challenge utilized data acquired from multiple international consortia dedicated to pediatric neuro-oncology and clinical trials. BraTS-PEDs 2023 aimed to evaluate volumetric segmentation algorithms for pediatric brain gliomas from magnetic resonance imaging using standardized quantitative performance evaluation metrics employed across the BraTS 2023 challenges. The top-performing AI approaches for pediatric tumor analysis included ensembles of nnU-Net and Swin UNETR, Auto3DSeg, or nnU-Net with a self-supervised framework. The BraTS-PEDs 2023 challenge fostered collaboration between clinicians (neuro-oncologists, neuroradiologists) and AI/imaging scientists, promoting faster data sharing and the development of automated volumetric analysis techniques. These advancements could significantly benefit clinical trials and improve the care of children with brain tumors.

The translation of AI-generated brain metastases (BM) segmentation into clinical practice relies heavily on diverse, high-quality annotated medical imaging datasets. The BraTS-METS 2023 challenge has gained momentum for testing and benchmarking algorithms using rigorously annotated internationally compiled real-world datasets. This study presents the results of the segmentation challenge and characterizes the challenging cases that impacted the performance of the winning algorithms. Untreated brain metastases on standard anatomic MRI sequences (T1, T2, FLAIR, T1PG) from eight contributed international datasets were annotated in stepwise method: published UNET algorithms, student, neuroradiologist, final approver neuroradiologist. Segmentations were ranked based on lesion-wise Dice and Hausdorff distance (HD95) scores. False positives (FP) and false negatives (FN) were rigorously penalized, receiving a score of 0 for Dice and a fixed penalty of 374 for HD95. The mean scores for the teams were calculated. Eight datasets comprising 1303 studies were annotated, with 402 studies (3076 lesions) released on Synapse as publicly available datasets to challenge competitors. Additionally, 31 studies (139 lesions) were held out for validation, and 59 studies (218 lesions) were used for testing. Segmentation accuracy was measured as rank across subjects, with the winning team achieving a LesionWise mean score of 7.9. The Dice score for the winning team was 0.65 ± 0.25. Common errors among the leading teams included false negatives for small lesions and misregistration of masks in space. The Dice scores and lesion detection rates of all algorithms diminished with decreasing tumor size, particularly for tumors smaller than 100 mm3. In conclusion, algorithms for BM segmentation require further refinement to balance high sensitivity in lesion detection with the minimization of false positives and negatives. The BraTS-METS 2023 challenge successfully curated well- annotated, diverse datasets and identified common errors, facilitating the translation of BM segmentation across varied environments and providing the tools for future development of personalized volumetric reports to patients undergoing BM treatment.

ABSTRACT Bipolar disorder’s (BD) clinical heterogeneity has an unresolved genetic basis. We meta-analyzed genome-wide association studies (GWAS) of 16 BD subphenotypes in 226,032 individuals from 57 cohorts (38,022 cases); 10 advanced to multivariate and multi-trait analyses. Four factors (compulsive, psychotic, dysregulated, internalizing) explained 82.8% of shared genetic variance. BD1 and BD2 loaded on distinct factors despite a high genetic correlation; 87.0% of common-factor loci were significant in neither subtype. Unipolar mania aligned with psychosis over internalizing, and was distinguishable from BD1, and rapid cycling showed heritable cross-domain liability. We identified 356 risk loci, 158 novel, including the first univariate-GWAS associations for psychosis, unipolar mania, rapid cycling and schizoaffective disorder—and 249 credible genes (89 high-confidence), 12 with approved-drug or clinical-phase annotations. Cell-type association showed a midbrain dopaminergic–GABAergic gradient along the psychotic factor. BD’s genetic architecture appears hierarchical—a general liability resolving into dimensions of course and comorbidity beyond diagnostic subtypes.