Bilateral Involvement Research Articles

Clinical presentation and outcomes in patients with antiphospholipid syndrome-associated adrenal hemorrhage. A multicenter cohort study and systematic literature review

BackgroundAdrenal hemorrhage (AH) can occur in patients with antiphospholipid Syndrome (APS). We aimed to characterize the clinical manifestations, treatments, and outcomes of patients presenting with APS-associated AH (APS-AH) through a retrospective cohort and a systematic literature review (SLR). MethodsWe performed a mixed-source approach combining a multicenter cohort with an SLR of patients with incident APS-AH. We included patients from Mayo Clinic and published cases with persistent positivity for antiphospholipid antibodies and presenting with AH, demonstrated by imaging or biopsy. We extracted demographics, clinical characteristics, laboratory findings, treatment strategies, and outcomes (primary adrenal insufficiency and mortality). We used Kaplan-Meier and Cox models for survival analysis. ResultsWe included 256 patients in total, 61 (24%) from Mayo Clinic and 195 (76%) from the SLR. The mean age was 46.8 (SD 15.2) years, and 45% were female. 69% of patients had bilateral adrenal involvement and 64% presented adrenal insufficiency. The most common symptoms at presentation were abdominal pain in 79%, and nausea and vomiting 46%. Hyponatremia (77%) was the most common electrolyte abnormality. Factors associated with primary adrenal insufficiency were bilateral adrenal involvement at initial imaging (OR 3.73, CI; 95%, 1.47–9.46) and anticardiolipin IgG positivity (OR 3.80, CI; 95%, 1.30–11.09). The survival rate at five years was 82%. History of stroke was associated with 3.6-fold increase in mortality (HR 3.62, 95% CI; 1.33–9.85). ConclusionAH is a severe manifestation of APS with increased mortality. Most patients developed permanent primary adrenal insufficiency, particularly those positive for anticardiolipin IgG and bilateral adrenal involvement.

Bilateral Involvement of Age-Related Macular Degeneration in South Korea: Findings from the Korea National Health and Nutrition Examination Survey 2017–2020

ABSTRACT Purpose To evaluate the bilateral involvement of age-related macular degeneration (AMD) in South Koreans. Methods This was a cross-sectional study of the Korean National Health and Nutrition Examination Survey (2017–2020). This study included 13,737 participants aged 40 years or older. Participants were evaluated to determine the prevalence of bilateral early and late AMD. In cases in which exudative AMD or geographic atrophy (GA) was diagnosed in a single eye, the fellow eye was evaluated to determine the presence and type of late AMD. Results The overall prevalence of bilateral AMD was 6.12% (95% confidence interval [CI], 5.63–6.61). The prevalence of bilateral early AMD was 5.71% (95% CI, 5.24–6.18), while that of late AMD was 0.14% (95% CI, 0.08–0.20). The prevalence of the bilateral involvement of late AMD increased with age. A 0.02% prevalence (95% CI, 0.00–0.06) of late AMD was observed in participants aged 50–59. The prevalence increased to 0.08% (95% CI, 0.00–0.18) in participants aged 60–69, while the prevalence in participants aged 70–79 and over 80 was 0.45% (95% CI, 0.12–0.78) and 1.97% (95% CI, 0.75–3.19), respectively. The prevalence of early AMD in one eye and late AMD in the fellow eye was 0.26% (95% CI, 0.16–0.36). Conclusions An assessment of the incidence of AMD revealed that a significant number of persons had bilateral involvement. The treatment burden may significantly increase for participants with bilateral late AMD compared to those with unilateral involvement. Therefore, the study may be helpful with the establishment of private and national insurance policies.

Syndromic Involvement of Patients Presenting With Congenital Upper Limb Anomalies: An Analysis of 4,317 Cases

This study investigated the patterns of syndromic involvement for patients with congenital upper limb anomalies (CULAs). We hypothesize that patients with CULAs will present with predictable syndromic patterns. This retrospective study queried the multicenter Congenital Upper Limb Differences (CoULD) Registry. Of the 4,317 patients enrolled, 578 (13%) reported one or more syndromes. Syndromes were confirmed to be recognized by the Online Mendelian Inheritance in Man. Demographics were reviewed and compared with the full CoULD registry group. Syndromes reported by five or more patients were examined to determine the type of CULA according to Oberg/Manske/Tonkin classifications. Of the 578 children with one or more reported syndromes, 517 had Online Mendelian Inheritance in Man recognized syndromes (cohort A), In cohort A, 58 syndromes were each represented by a single patient within the registry. Forty-eight syndromes in cohort A were reported by two or more patients, which accounted for 461 of the total patients with reported syndromes. However, VACTERL and Poland syndromes were the most commonly reported syndromes. Patients with CULAs and syndromes frequently exhibited bilateral involvement (61%), compared with the entire CoULD group (47%) and other orthopedic (50%) and medical conditions (61%) compared with the entire CoULD group (24% and 27%, respectively). Additionally, they exhibited a lower frequency of family history of a congenital orthopedic condition (21%) or a family member with the same CULA (9%) compared with the entire CoULD group (26% and 14%, respectively). Associated syndromes were recorded in 578 patients (13%) in the CoULD registry as follows: 58 syndromes represented by a single patient, 48 by 2 or more patients, and 23 syndromes by 5 or more patients. Rare syndromes that are only represented by a single patient are more likely to be unknown by a pediatric hand surgeon, and consultation with a geneticist is advised. Differential Diagnosis/Symptom Prevalence Study IV.

Clinical efficacy of modified Yokoyama procedure with extraocular muscle transposition for high myopic eyes with restrictive esotropia

Objective: To investigate the clinical efficacy of the modified Yokoyama procedure with extraocular muscle transposition for high myopic eyes with restrictive esotropia. Methods: A retrospective case series study was conducted. Clinical data were collected from patients who underwent the modified Yokoyama procedure with extraocular muscle transposition for high myopic eyes with restrictive esotropia at Eye Hospital of Shandong First Medical University from February 2017 to February 2022. During the procedure, the superior rectus and lateral rectus muscles were fully separated. A longitudinal blunt incision was made in the central muscle belly extending posteriorly to 12-14 mm from the muscle insertion. The temporal half of the superior rectus muscle and the upper half of the lateral rectus muscle belly were transposed and secured to the contralateral muscle insertion. Simultaneously, medial rectus muscle recession was performed. Follow-up visits were conducted at 1 week, 1 month, 3 months, and 6 months postoperatively. Patients' ocular alignment, eye movements, improvement in compensatory head posture, objective degrees of strabismus using synoptophore, changes in extraocular muscles and globe position on orbital CT scan were recorded. Surgical complications were also documented. Results: Five patients (8 eyes) were included in this study, including 4 females (7 eyes) and 1 male (1 eye), with an average age of (63±11) years and an average axial length of (34.58±2.25) mm. The medial rectus muscle recession during surgery was (7.6±2.3) mm. Preoperatively, all patients had esotropia with a horizontal range of+15°to+45° and a vertical range of+15°to+45°. Significant limitations in lateral and upward gaze were observed, with a degree of restriction ranging from-3 (-4 to-1). Three patients with bilateral involvement and one patient with unilateral involvement exhibited significant compensatory head postures. One patient with unilateral involvement had no compensatory head posture. Preoperative orbital CT scans indicated nasal displacement of the superior rectus muscle and inferior displacement of the lateral rectus muscle, with the eyeball herniating from the muscle cone. At the 6-month follow-up, all patients achieved nearly orthophoric alignment. Objective degrees of horizontal strabismus ranged from-4°to+7°, and vertical strabismus ranged from 0°to +6°, as determined by synoptophore examination. Eye movements significantly improved, with a degree of restriction of-1 (-2 to-1) for lateral gaze and-2 (-3 to-1) for upward gaze. Compensatory head postures disappeared, and orbital CT scans indicated the eyeball was located within the muscle cone. There were no severe complications such as anterior segment ischemia, muscle adhesions, disease recurrence, secondary glaucoma, or globe penetration. Conclusion: The preliminary clinical outcomes of the modified Yokoyama procedure with extraocular muscle transposition for high myopic eyes with restrictive esotropia are promising.

Retrospective Analysis of Presymptomatic Treatment In Sturge-Weber Syndrome.

Ninety percent of infants with Sturge-Weber syndrome (SWS) brain involvement have seizure onset before 2 years of age; this is associated with worse neurologic outcome. Presymptomatic treatment before seizure onset may delay seizure onset and improve outcome, as has been shown in other conditions with a high-risk of developing epilepsy such as tuberous sclerosis complex. Electroencephalogram (EEG) may be a biomarker to predict seizure onset. This retrospective clinical data analysis aims to assess impact of presymptomatic treatment in SWS. This two-centered, IRB-approved, retrospective study analyzed records from patients with SWS brain involvement. Clinical data recorded included demographics, age of seizure onset (if present), brain involvement extent (unilateral versus bilateral), port-wine birthmark (PWB) extent, family history of seizure, presymptomatic treatment if received, neuroscore, and anti-seizure medication. EEG reports prior to seizure onset were analyzed. Ninety-two patients were included (48 females), and 32 received presymptomatic treatment outside of a formal protocol (5 aspirin, 16 aspirin and levetiracetam; 9 aspirin and oxcarbazepine, 2 valproic acid). Presymptomatically-treated patients were more likely to be seizure-free at 2 years (15 of 32; 47% versus 7 of 60; 12%; p<.001). A greater percentage of presymptomatically-treated patients had bilateral brain involvement (38% treated versus 17% untreated; p=.026). Median hemiparesis neuroscore at 2 years was better in presymptomatically-treated patients. In EEG reports prior to seizure onset, the presence of slowing, epileptiform discharges, or EEG-identified seizures was associated with seizure onset by 2 (p=.001). Presymptomatic treatment is a promising approach to children diagnosed with SWS prior to seizure onset. Further study is needed, including prospective drug trials, long-term neuropsychological outcome, and prospective EEG analysis to assess this approach and determine biomarkers for presymptomatic treatment.

Optical coherence tomography characteristics and factors affecting visual acuity in diabetic papillopathy.

To analyse baseline imaging characteristics and factors affecting poor visual acuity in diabetic papillopathy. This was a retrospective, observational study conducted at a tertiary eye care centre in eyes with a diagnosis of diabetic papillopathy. Demographic data including age, gender, duration of diabetes, type of diabetes, HbA1c values and other systemic co-morbidities were recorded. Baseline best corrected visual acuity (BCVA), and various imaging characteristics were noted. 37 eyes of 22 patients with diagnosis of diabetic papillopathy were included [mean age of 46.6 ± 13.5 years, five (22.7%) females and 17 (77.3%) males]. Bilateral involvement was seen in 15 (68.2%) patients. The mean baseline BCVA was 0.51 ± 0.49 logMAR (Snellen equivalent 20/60). Two eyes (5.4%) had features of concurrent mild NPDR, 9 eyes (24.3%) had moderate NPDR, 10 eyes (27.03%) had severe NPDR while 16 (43.2%) other had PDR. All eyes had optic disc edema while 4 had disc pallor at presentation. Telangiectatic vessels/ neovascularisation of disc was present in 17 eyes (45.9%) detected either clinically or on optical coherence tomography (OCT) angiography. 11 (29.7%) eyes had a peripapillary cuff of subretinal fluid accompanying the disc edema. Sixteen eyes (43.2%) had centre involving macular edema seen on OCT. In eyes with a macular edema, the mean central macular thickness was 407.4 ± 71.42 microns at baseline. On assessing the baseline factors that contribute to the final visual acuity, presenting visual acuity was the only variable found to be statistically significant. Although described to have good visual outcome, eyes presenting with poor visual acuity tend to have worse vision on follow up. This may aid in prognosticating and guiding management plan.

Watchful Waiting Strategy in the Treatment of Acute Otitis Media in Children

Background: Acute otitis media (AOM) is a common issue among children, often requiring antibiotic treatment. It is crucial to explore methods for the rational use of antibiotics. This study aimed to assess the effectiveness of the watchful waiting approach in treating children aged 6 months to 18 years with mild to moderate AOM. Methods: This prospective quasi-experimental study was conducted on 100 children with mild to moderate AOM. All patients, except those in severe or critical conditions, were placed on the watchful waiting strategy. A follow-up was conducted after 48 - 72 hours. If symptoms had improved, the watchful waiting approach continued. If medication was necessary for various reasons, these reasons were documented. Subsequently, children who completed the watchful waiting strategy were compared to those who received antibiotics. The collected data were analyzed using chi-square and t-tests in SPSS Statistics for Windows (version 16.0). Results: In this study, 59 patients (59%) were treated with the watchful waiting approach; nevertheless, 41 patients (41%) received antibiotics. The presence or absence of postnasal discharge showed a significant difference between the two groups (P = 0.001). No significant differences were observed between the two groups regarding fever (P = 0.066), enlarged tonsils (P = 0.740), snoring (P = 0.252), and bilateral involvement of the tympanic membrane (P = 0.313). Conclusions: Based on the results, the watchful waiting strategy is a cost-effective method for children aged over 6 months with mild to moderate symptoms, provided that parents cooperate fully. Clinicians should be cautious and consider alternative treatments if postnasal discharge is present, as it might indicate a higher likelihood of treatment failure with the watchful waiting approach.

Additional Parameters to the Briganti Nomogram for Predicting Pelvic Lymph Node Metastasis in Prostate Cancer.

Despite advanced medical technology, accurately predicting pelvic lymph node (LN) metastasis in patients with prostate cancer (PCa) remains a challenge. Various nomograms were utilised to enhance the accuracy of this prediction. Our goal was to determine if preoperative inflammation markers and transrectal prostate biopsy data offer extra insight into predicting pathological LN involvement in radical prostatectomy with extended pelvic LN dissection (RP + ePLND). This study included patients with PCa who had a Briganti score of 5 or higher and were treated with RP + ePLND between January 2016 and May 2023. Physical examination findings, blood work within 1 month before biopsy and prostate biopsy results were recorded retrospectively. The patients were divided into two groups based on the presence or absence of pelvic LN metastasis (group 1 and group 2, respectively). The preoperative data of both groups were compared for analysis. The study involved 71 patients; 17 were in group 1, and 54 were in group 2. We observed significant differences between the groups in terms of bilateral tumour involvement in prostate, presence of perineural and lymphovascular invasion (LVI) in biopsy specimens, D'Amico risk group, as well as haematological and biochemical parameters such as the absolute counts of lymphocyte and monocyte, neutrophil-to-lymphocyte ratio and the levels of total and free prostate-specific antigen. Logistic regression analysis revealed that absolute lymphocyte count, presence of LVI and bilateral tumour involvement were independent predictors of pathological pelvic LN metastasis in PCa. The cut-off value of lymphocyte count for predicting LN metastasis was 1.57 (103/mL) with a sensitivity of 0.870 and a specificity of 0.412 (area under the curve (AUC): 0.675, p = 0.030). According to our findings, LVI in biopsy specimens, bilateral lobe tumours and preoperative lymphocyte count are significant predictors of pelvic LN metastasis in patients with PCa and a Briganti score of ≥5.

Population Prevalence, Pattern and Associated Factors for Retinal Diseases at High Altitude in Nepal.

Retinal diseases are common at high altitudes due to a cascade of changes caused by hypoxia. The aim of this study is to assess the population prevalence, pattern and associated factors of retinal disorders at high altitude in Nepal. A cross-sectional study was conducted at three selected high-altitude districts (over 2500 meters) of Nepal. Subjects were at least 40 years old, and the target sample size was 309. A detailed history was taken. Visual acuity, blood sugar, blood pressure, and oxygen saturation were measured. Anterior and posterior ocular evaluations were conducted by retina specialists using slit lamp and indirect ophthalmoscopy. A total of 338 participants were recruited, with nearly equal numbers from the three districts with mean age of 57.0 (S.D 11.1) years. Two-thirds (63.9%) were females; 38.2% were illiterate, and 46.7% were farmers. Average blood oxygen saturation was 87.2% (S.D 4.1%). Systemic hypertension and diabetes mellitus were found in 58% and 11%, respectively. Retinal diseases were found in 176 (52.5%), with bilateral involvement in 157 (46.9%). The major retinal diseases were hypertensive retinopathy (32.2%), high-altitude retinopathy (10.4%), age-related macular degeneration (AMD) (8.1%), branch vein occlusion (BRVO) (2.1%), and diabetic retinopathy (DR) (1.8%). The multivariate analysis showed significant association of retinal diseases with age and hypertension. Best corrected visual acuity better than 6/18 was present in 96.7%. Over half of the study participants had retinal diseases, with hypertensive retinopathy, AMD, and high-altitude retinopathy as the most common retinal problems. A significant association of retinal diseases was found with ageing, and hypertension. Access to eye care services and control of systemic hypertension along with patient education should be emphasized among people living at high-altitude.

Corneal and anterior segment parameters in patients with clinically unilateral pseudoexfoliation syndrome.

Pseudoexfoliation syndrome (PES) is an age-related systemic condition that predominantly affects ocular structures and is characterized by the deposition of material on the lens, ciliary body, zonules, corneal endothelium, iris, and pupillary margin. We compared the corneal endothelial morphology, anterior segment parameters, corneal densitometry, and corneal topographic characteristics between the clinically affected and apparently normal fellow eyes of patients with clinically unilateral PES. This was a comparative, cross-sectional study of 34 patients with clinically unilateral PES. The anterior segment was examined using a Scheimpflug imaging system, and the corneal endothelium was assessed using a noncontact specular microscope. Corneal endothelial cell density, polymegathism, and pleomorphism were assessed using the specular microscope. Furthermore, the Scheimpflug camera was used to measure the corneal power of the flat and steep axis, mean corneal power, maximum keratometry, anterior chamber angle, anterior chamber depth, anterior chamber volume, corneal volume, and the corneal thickness at the apex point, center of the pupil, and the thinnest point. Corneal densitometry was evaluated at two concentric zones (0-2 mm and 0-12 mm). In total, 68 eyes from 34 patients were ultimately included in the study. The mean (standard deviation) age of the patients was 73.38 (8.75) years (range: 50-87 years). Among the included patients, 17 (50%) were male and 17 (50%) were female. The anterior segment parameters did not significantly differ between eyes with PES and their clinically unaffected fellow eyes (all P > 0.05). Similarly, no statistically significant difference was observed in corneal endothelial morphology (all P > 0.05). Our measured parameters do not differ between the clinically affected eye and the clinically unaffected fellow eye. This supports the theory that PES is a bilateral disorder. Considering the variety of complications associated with PES, bilateral involvement should be assumed in the clinical and surgical management of patients with clinically unilateral PES. In the future, new research could increase our understanding of this syndrome.

Retinoblastoma in Ceara: An epidemiological study in a Brazilian pediatric oncology referral center.

Although Brazil has a high prevalence of retinoblastoma, there is a lack of epidemiological data on the disease. Thus, in this study, we aimed to evaluate the epidemiological profile of patients diagnosed with retinoblastoma in the ophthalmology department of a pediatric tertiary referral hospital in Ceara, Brazil. A descriptive and cross-sectional study was conducted by retrospectively analyzing the clinical and socioeconomic data from the medical records of pediatric patients followed-up at the hospital between 2007 and 2021. Retinoblastoma was diagnosed on the basis of a fundoscopic or histopathologic examination. The data of 105 patients were included in the study, and the mean patient age at the time of diagnosis was 1.7 years. Most of the patients were women (50.5%) and hailed from rural areas (57.4%), which was associated with a higher tumor stage. Of the 150 patients, 57.1% initially presented with leukocoria. Ocular hyperemia was associated with more advanced stages of retinoblastoma (p=0.004). Bilateral involvement was observed in 25.7% of the patients and at a significantly younger age (p=0.009). The presence of retinal detachment, vascularized lesions, and vitreous seeds significantly increased the likelihood of requiring enucleation. This study presents an epidemiological description of retinoblastoma in Brazil, which highlights the significance of early detection. Delayed diagnosis is associated with a poorer visual prognosis and higher mortality rate, particularly in patients with unilateral disease. Risk factors for a more severe disease were retinal detachment, vascularized lesions, and vitreous seeds. The correlation between histopathological features and clinical outcomes was limited. Further studies are required to assess the influence of ocular hyperemia, fundoscopic assessment, and histopathologic findings on the prognosis of retinoblastoma. Moreover, it is critical to devise interventions to reduce the time-to-diagnosis in rural areas.

Bipartitism in Tarsal Bones: A Retrospective Analysis of Clinical and Radiological Features

Background: Bipartitism in the foot, especially in the tarsal bones, is a rare anatomical variant characterised by dividing one bone into two separate ossification centers. This condition can be congenital or acquired, often remains asymptomatic, and is only discovered incidentally during radiological imaging. This study aims to present a comprehensive retrospective analysis of bipartitism in tarsal bones, assessing its prevalence, type of articulation and associated clinical features in a cohort of patients. Methods: A total of 4,645 patients were retrospectively evaluated from 6,145-foot images consisting of 4,975 computed tomography (CT) and 1,170 magnetic resonance imaging (MRI) scans obtained between 2015 and 2023. 15 patients with tarsal bipartitism were identified and contacted through the hospital system for in-person medical history and examination. Radiological exams assessed partial and complete bipartition, diastasis, and exostosis in the tarsal bones. Age, sex, side, trauma history, pain, foot deformity, gait disturbance, and other clinical findings were also recorded. Results: Bipartitism was detected in 15 patients (0.32%), predominantly affecting males (87%). The most common variant was medial cuneiform bipartitism, observed in 13 patients (0.28%). Talus and calcaneus bipartitism were each observed in one patient (0.02%). Bilateral involvement was present in 67% of the cases, with 60% of patients reporting chronic pain. Complete dissociation of bipartite bones was noted in 80% of cases. Accompanying bipartitism, two patients had pes planovalgus, and one had hallux varus deformity. Conclusion: Although bipartitism of the tarsal bones is rare, it remains a diagnostic challenge due to variability in clinical presentation and possible association with other foot deformities. Accurate diagnosis through advanced imaging techniques is essential to differentiate these variants from fractures and guide appropriate treatment. This study contributes valuable insights into the prevalence and clinical impact of bipartitism in the tarsal bones, emphasising the importance of early diagnosis and individualised treatment strategies.

Simultaneous and Consecutive Drainage of Bilateral Chronic Subdural Hematoma: A Randomized Controlled Trial.

Chronic subdural hematoma (CSDH) is one of the most common types of intracranial hemorrhage, particularly in elderly individuals. Although most patients present with unilateral CSDH, bilateral involvement is not rare. Furthermore, bilateral CSDHs are associated with rapid deterioration and poorer outcomes because of a higher risk of brain herniation than unilateral hematomas. The most contentious issue is the potential herniation of the medial temporal lobe, which remains on the unevacuated side during the brief interval between right and left procedures. We compared simultaneous burr-hole craniostomy with consecutive burr-hole craniostomy for treating bilateral CSDH and to determine whether consecutive evacuation is riskier in terms of brain stem complications.Over a 6.5-year period, patients with bilateral CSDH who had an indication for operation were allocated into two groups randomly. The first group (n = 18) underwent simultaneous evacuation, and the second group (n = 25) underwent consecutive evacuation. Glasgow Coma Scale and Markwalder grades were recorded during the postoperative period. Patients were followed up during the inpatient period and postoperatively at 1, 3, 6, and 12 months after discharge. Mortality, morbidity, surgical complications, reoperation, and, as a combination of all of these, treatment success rates were compared. Treatment success rates were worse in patients with mixed-density hematomas and in female patients at the end of 12 months, but there was no significant difference between the simultaneous and consecutive evacuation groups at any time. Therefore, the choice of technique can be decided by the surgeon.

Adult Moyamoya Disease and Moyamoya Syndrome: What Is New?

Recent advances are in the genetics, diagnosis, pathophysiology, and management of moyamoya disease (MMD), and moyamoya syndrome (MMS), a term used to describe moyamoya-like vasculopathy associated with various systemic diseases or conditions. Ring finger protein (RNF213) has been reported to be a susceptibility gene not only for MMD but also for atherosclerotic intracranial arterial stenosis and ischemic stroke attributable to large artery atherosclerosis. The latest guidelines by the Research Committee on MMD of the Japanese Ministry of Health, Labor, and Welfare, removed limitations of the previous definition that required bilateral involvement of the intracranial carotid artery to make the diagnosis, given the increasing evidence of progression to bilateral involvement in unilateral MMD. 3-dimensional constructive interference in steady-state MRI is useful for the differential diagnosis of MMD from atherosclerosis. Recent advances in the pathophysiology of MMD suggest that genetic and environmental factors play important roles in vascular angiogenesis and remodeling via complex mechanisms. The latest Japanese Guidelines and American Scientific Statement described that antiplatelet therapy can be considered reasonable. Endovascular interventional stent placement fails to prevent ischemic events and does not halt MMD progression. In the Japan Adult Moyamoya trial, a randomized controlled trial for bilateral extracranial-intracranial direct bypass versus conservative therapy in patients with MMD, who had intracranial hemorrhage, recurrent bleeding, completed stroke, or crescendo transient ischemic attack was significantly fewer with direct bypass than with conservative care. This review presents updated information on genetics, diagnosis, pathophysiology, and treatment of adult MMD and MMS. Despite recent advances, many mysteries still exist in the etiologies of moyamoya vasculopathy. The diagnostic criteria and treatment guidelines have been updated but not yet been globally established. Ongoing and future studies investigating underlying pathophysiological mechanisms of MMD and MMS may clarify potentially effective medical, surgical, or endovascular treatments.

Analysis of risk factors for lymph node metastasis in 241 patients with thyroid carcinoma and establishment of a prediction model.

This study aimed to identify risk factors for cervical lymph node metastasis (LNM) in papillary thyroid carcinoma (PTC) and develop a clinical prediction model. Retrospectively, data were collected from 348 PTC patients treated at the Second Affiliated Hospital of Nanchang University between January 2019 and December 2022, with 241 patients included in the final analyses. Patients with lateral cervical LNM were categorized into a metastasis group, and those without were in a non-metastasis group. The patients were divided into a training set (n=169) and a validation set (n=72) in a 7:3 ratio. Logistic and least absolute shrinkage and selection operator (LASSO) regression models were used to identify key factors associated with lateral cervical LNM and prognosis, enabling the construction of a predictive model. The model's validity was assessed via the Hosmer-Lemeshow Test, calibration curves, ROC curves, and decision curve analysis. The metastasis group exhibited higher proportions of males, multiple lesions, bilateral involvement, tumor diameter ≥1 cm, and elevated levels of PLR, LMR, and NLR (P<0.05). Logistic regression analysis revealed that gender, multiple lesions, affected side, and tumor diameter were associated with lateral cervical LNM (P<0.05). The predictive Nomogram model, which included factors like affected side, tumor diameter, capsular invasion, central LNM, PLR, and NLR, demonstrated strong predictive accuracy and clinical utility. Thus, this study provides a practical clinical tool through an accurate Nomogram model to assess lateral cervical LNM risk in PTC patients using logistic and LASSO regression analyses.

Breaking the mould: An essential need in optic neuritis management

Aims/Purpose: To present a clinical case to highlight the importance of ophthalmological examination in the management of optic neuritis.Methods: Case report.Results: A 45‐year‐old woman with Sjögren's syndrome and a family history of MS presents to the emergency department. She complains of decreased vision OD for the past 4 days, with exacerbated‐by‐eye‐movements retroocular pain and migraine‐like headache. BCVA is amaurosis OD and 1 OI, with right RAPD and no colour vision impairment in OI. Except for optic disc oedema with splinter haemorrhages in OD the rest of the ophthalmological examination is normal. RNFL‐OCT reveals thickening in the OD (220 μm/110 μm). Brain CT and laboratory tests, including D‐dimers, are normal. A LP is performed to rule out infectious causes is normal. Neurofilament and ESR are also normal. Brain and spinal MRI show no abnormalities. Due to the severity, methylprednisolone therapy is initiated. One week later, her symptoms are stable, except for headache and retroocular pain improvement. Upon reassessment, despite preserved BCVA and Ishihara in OI, an increase in RNFL thickening is noted in OI (120 μm), with partial arcuate defect in visual field. Anti‐MOG, AQP4 and a panel of autoantibodies including anti‐Ro and anti‐La, are still pending. Considering the bilateral involvement, plasma exchange therapy is initiated. 14 days after, BCVA OU has improved to 1, and the visual field and Ishihara test have normalized. RNFL thickening is still observed OU (161/120 μm) and there is persistent optic disc edema OD. Anti‐MOG are positive, AQP4‐Ab are negative, and both negative in CSF.Conclusions: The coexistence of other autoimmune diseases, can lead to attributing the ON to that disease rather than considering the neuroinflammatory aetiology, leading to delayed diagnosis. The detection of subclinical bilateral involvement was crucial in escalating the therapeutic regimen. Seropositivity is the confirmatory diagnostic marker, but in many centres, it may be difficult to obtain reliable biomarker data.

Study of Cerebral Hemispheric Lateralization in Stroke and the Arterial Territories Involved in High-risk Patients

Introduction: According to WHO, Stroke is the 2nd leading cause of death and disability after Ischemic heart disease. Prevalence rate of stroke in India varies from 44.45 to 150 per lakh population. Studies have reported a higher frequency of stroke of the left hemisphere. The artery reported to be the most affected is Left Middle Cerebral artery(MCA) followed by Right Middle Cerebral artery. The aim of this study is to determine the side of cerebral hemisphere most affected by stroke and the arterial territories involved in high-risk patients aged above 35 years presenting to the tertiary healthcare centre, as well as to study the sex predilection of stroke. Materials and Methods: A cross-sectional prospective study of 50 high risk stroke patients with Diabetes Mellitus(DM) and /or Hypertension(HTN) presenting to General medicine OPD of a tertiary healthcare centre were investigated with CT/MRI for the side of the cerebral hemisphere affected and the arterial territories involved. The data was tabulated over the course of three months and statistical analysis was carried out. Results: The left cerebral hemisphere was affected in 50%, right in 40% and bilateral involvement was found in 10% patients. Slight predilection of stroke towards the left was seen, however it was not found to be statistically significant (P-value= 0.249). MCA territory was involved in 66% of the patients, Posterior Cerebral Artery(PCA) territory in 8%, Vertebrobasilar Artery(VB) territory in 8%, Anterior Cerebral Artery(ACA) territory in 6% and MCA+ACA in 4%, MCA+VB in 2% and MCA-PCA watershed in 6% patients. Conclusions: We found a slight predilection of stroke towards the left, which was however, not found to be statistically significant. We also found that Middle Cerebral Artery(MCA) territory is the most commonly involved in stroke.

Association of Ocular Injuries in Patients with Head Injury: A Tertiary-Level Armed Forces Experience

ABSTRACT Introduction: The incidence of trauma is on an increasing trend worldwide. It has drastically increased due to urbanization, vehicular congestion, alcoholism, and poor infrastructure facilities, especially in developing countries. In the Armed Forces, the additional burden of trauma is also seen due to various unit activities, organized sports, and other adventure sports. Head injury cases are often associated with various ocular injuries. These ocular injuries, if subtle in nature, can be missed initially and progress into ophthalmological complications and rarely into permanent visual impairment. The aim of this study was to critically analyze the association of various ocular injuries in patients with head injury. Materials and Methods: A cross-sectional study was conducted in a tertiary-level Armed Forces Hospital from July 2016 to December 2018. A total of 40 patients were evaluated who had head injury along with ocular injuries. The Glasgow Coma Scale (GCS) was used to assess the severity of the head injury. Ocular manifestations such as subconjunctival hemorrhage, ecchymosis, soft tissue injuries such as lid tear, corneal injuries, and bony and associated nerve injuries were evaluated. Results: A total of 40 patients with head injury with ocular manifestations were studied. Road traffic accident (RTA) was the most common cause, followed by accidental fall from height and sports injuries. There was a male preponderance (n = 32 (80%)) over females (n = 8 (20%)). Unilateral ocular manifestation (n = 32 (80%)) was more common than bilateral involvement (n = 8 (20%)). The most frequent ocular manifestations were subconjunctival hemorrhage and ecchymosis (35 (87.5%)), followed by lid laceration (6 (15%)), corneal tear (04 (10%)), vitreous hemorrhage (01 (2.5%)), evisceration (2 (05%)), and cranial nerve injury (06 (12.5%)). Orbital wall fracture was seen in 15 (37.5%) patients, including lateral wall fracture (n = 08 (20%)), medial wall fracture (n = 04 (10%)), orbital floor fracture (n = 02 (05%)), and orbital roof fracture (n = 01 (2.5%)). Conclusion: Ocular injuries can occur in patients with head injury irrespective of the mode and kinematics involved (severe head injury may present with minimal ocular manifestations or vice versa). These patients require hospitalization, observation, thorough ophthalmic evaluation, and periodic assessment to minimize morbidity and complications.

GM1 and GM2-Gangliosidosis: Clinical Features, Neuroimaging Findings and Electroencephalography.

Gangliosidosis is one of the hereditary metabolic diseases caused by the accumulation of Gangliosid in the central nervous system, leading to severe and progressive neurological deficits. Regarding phenotype, GM1 and GM2-Gangliosidosis are divided into Infantile, Juvenile, and Adult. In this study, thirty-seven patients with GM1 and GM2-Gangliosidosis were referred to the neurology department of Mofid Children's Hospital in Tehran, Iran, whose disease was confirmed from September 2019 to December 2021. This study assessed age, sex, and developmental status before the onset of the disease, clinical manifestations, brain imaging, and electroencephalography. 97.20% of patients were the result of family marriage. Approximately 80% of juvenile patients were developmentally normal before the onset of the disease. Developmental delay was more common among infantile GM1-Gangliosidosis than infantile GM2-Gangliosidosis, but in total, more than 50% of GM1&GM2-Gangliosidosis patients had reached their developmental milestone before the onset of the disease. With the onset of disease symptoms, 100% of patients regressed in terms of movement, 97.20% of them mentally, and 75% of them had seizures during the disease. The most common clinical findings were cherry-red spot, Mongolian spot, macrocephaly, organomegaly, hyperacusis, and scoliosis. The most common brain imaging findings included bilateral thalamus involvement, brain atrophy, PVL, and delayed myelination. The most common finding in electroencephalography was background low voltage with abnormal sharp waves. This study concluded that most of the patients are the result of family marriage, and most of the juvenile patients are developmentally normal before the onset of the disease. In addition, more than 50% of infantile patients reach their developmental milestones before the onset of the disease. The most common clinical findings of these patients are seizures, cherry-red spot, macrocephaly, hyperacusis, Mongolian spot, and bilateral involvement of the thalamus.

Effect of delamanid on interim outcomes of bacteriological conversion amongst pediatric drug resistant tuberculosis cases in India.

To determine the bacteriological conversion rate after 6 months of Delamanid (DLM) based treatment in children with drug-resistant tuberculosis (DR-TB) and determine factors associated with bacteriological conversion. This is a descriptive retrospective study done in children between the age of 6-17 years with DR-TB who received DLM-based therapy from October 2018 to May 2021. The drug resistance pattern of TB was detected using Xpert RIF/MTB and phenotypic drug sensitivity testing (DST) on TB-MGIT culture reports. Follow-up sputum TB MGIT culture was carried out monthly after DLM initiation for 6 months. Factors associated with sputum bacteriological conversion such as age, gender, pulmonary TB (PTB) versus disseminated TB, unilateral or bilateral lung involvement, type of DR-TB, prior treatment failure, and type of DR-TB regimen were analyzed. Sixty patients received DLM of which two had extrapulmonary TB (EPTB) and sputum conversion could not be assessed. The mean age at presentation was 12.69 ± 3.03 years. Five patients (8.3%) died while on DLM treatment. On follow-up, 8 (13.7%) out of 58 patients had no sputum bacteriological conversion after 6 months of DLM initiation of which three patients were on salvage therapy; 46 (79.3%) had sputum bacteriological conversion within 6 months of DLM initiation. Sputum bacteriological conversion rate was almost 80% at the end of 6 months of DLM-based treatment.