Confluent Pattern Research Articles

A Familial Cohort of Adult-Onset Alexander Disease (P12-3.016)

<h3>Objective:</h3> To describe a familial cohort of adult-onset Alexander Disease. <h3>Background:</h3> Leukodystrophies are a group of rare, hereditary disorders characterized by abnormal myelination. Each leukodystrophy is caused by abnormalities in one or more genes leading to abnormal production of myelin (hypomyelination) or destruction (demyelination). Leukodystrophies are also classified by the region of the brain or in some cases peripheral nerves that are affected. Alexander Disease is a leukodystrophy characterized by a confluent pattern of demyelination. It is caused by autosomal dominant gain-of-function mutation in the <i>GFAP (glial fibrillary acidic protein)</i> gene. <i>GFAP</i> serves an intermediate filament protein of mature astrocytes; incorrect formation of the protein can lead to astrocyte cytoskeletal collapse and abnormal protein aggregation. Clinically, this can lead to innumerable neurologic manifestations in the affected individual. Alexander Disease predominantly affects neonates, infants, children and rarely adults. We describe a familial cohort of adult-onset Alexander Disease associated with a c.758C&gt;G (p.A253G) mutation of GFAP. This mutation has previously been described in neonates. In this case series we describe the clinical and radiologic features of three sisters with adult-onset symptoms. The first patient is a 53-year-old female who presented with progressive myelopathy and later developed bulbar symptoms. Her 51-year-old sister developed progressive, bilateral leg weakness and gait disturbance. She progressed to have profound cognitive dysfunction. The MRI features include T2 hyperintensities with atrophy in the midbrain and upper cervical spine with peripheral rim brainstem and periventricular garlands. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> These cases describe the clinical and radiologic features of a familial cohort of adult-onset Alexander disease in a mutation previously described only in childhood onset. This also illustrates the importance of considering a diagnosis of Alexander Disease in adult patients and the necessity of genetic analysis of patients and their family members. <b>Disclosure:</b> Dr. Wolf has nothing to disclose. Dr. Gettings has nothing to disclose. Dr. Goodrich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono.

The use of optical coherence tomography angiography in differential diagnosis of conjunctival melanocytic tumors

BACKGROUND: Optical coherence tomography angiography (OCTA) is a noninvasive method of eye microcirculation evaluation. Few reports are published on the use of OCTA for anterior segment (AS) vessels analysis in healthy eyes and in conjunctival tumors, and their vascular characteristics are still not thoroughly investigated. These questions are of importance, as it is known that tumors vasculature is indicative of the patients vital prognosis.&#x0D; AIM: The aim of our study was to investigate the potential of AS-OCTA in evaluation of normal conjunctival vessels architecture as well as that in melanocytic neoplasms.&#x0D; MATERIALS AND METHODS: 20 healthy volunteers (20 eyes) and 20 patients (20 eyes) with conjunctival nevi and melanomas were examined. AS optical coherence tomography (OCT) and AS-OCTA were performed. Scan analysis included qualitative assessment (vessels pattern, lumen, tortuosity) and quantitative assessment [perfusion density (PD, %) index]. Mean (MPD), maximum (MaxPD) and perifocal PD (PPD) were determined.&#x0D; RESULTS: In normal group, predominantly radial pattern of the vessels was revealed, their caliber remaining the same along their entire length; larger vessels were more often discovered in deep conjunctival layers. The lowest PD value (29.9%) was registered in the inferior conjunctival segment, and the highest (36.7%) in the nasal one. In the conjunctival tumors area tortuosity of the vessels, uneven vessels caliber along their length, and increase in the PD value were observed. Melanomas were characterized by an increase in the lace-like pattern and by presence of confluent pattern zones; MaxPD value was more than 50%. Significant difference was found between MPD values of normal conjunctiva and MPD values in conjunctival melanoma.&#x0D; CONCLUSIONS: AS-OCTA is an informative method for the visualization of vessels in normal conjunctiva and in conjunctival tumors. If the tumors vessels are unevenly distributed, MaxPD should be measured.

Pathophysiology of myelin oligodendrocyte glycoprotein antibody disease.

Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD) is a spectrum of diseases, including optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, and cerebral cortical encephalitis. In addition to distinct clinical, radiological, and immunological features, the infectious prodrome is more commonly reported in MOGAD (37-70%) than NMOSD (15-35%). Interestingly, pediatric MOGAD is not more aggressive than adult-onset MOGAD, unlike in multiple sclerosis (MS), where annualized relapse rates are three times higher in pediatric-onset MS. MOGAD pathophysiology is driven by acute attacks during which T cells and MOG antibodies cross blood brain barrier (BBB). MOGAD lesions show a perivenous confluent pattern around the small veins, lacking the radiological central vein sign. Initial activation of T cells in the periphery is followed by reactivation in the subarachnoid/perivascular spaces by MOG-laden antigen-presenting cells and inflammatory CSF milieu, which enables T cells to infiltrate CNS parenchyma. CD4+ T cells, unlike CD8+ T cells in MS, are the dominant T cell type found in lesion histology. Granulocytes, macrophages/microglia, and activated complement are also found in the lesions, which could contribute to demyelination during acute relapses. MOG antibodies potentially contribute to pathology by opsonizing MOG, complement activation, and antibody-dependent cellular cytotoxicity. Stimulation of peripheral MOG-specific B cells through TLR stimulation or T follicular helper cells might help differentiate MOG antibody-producing plasma cells in the peripheral blood. Neuroinflammatory biomarkers (such as MBP, sNFL, GFAP, Tau) in MOGAD support that most axonal damage happens in the initial attack, whereas relapses are associated with increased myelin damage.

Enlarged left atrium may be associated with laterality of anterior circulation cardioembolic stroke

BackgroundPropensity for lesion lateralization in atrial fibrillation-related cardiac embolic stroke (AF-stroke) remains controversial. In this study, we compared the hemispheric differences among patients with AF-stroke and identified factors associated with lesion laterality. MethodsWe retrospectively reviewed patients with acute AF-stroke admitted from January, 2017 to March, 2022. Patients were grouped based on whether lesions were right or left hemispheric in the anterior circulation territory, based on diffusion-weighted imaging. Factors associated with right-side propensity were analyzed. ResultsAmong 385 patients, the mean age was 74±11 years and 52.5 % were male. Right and left hemispheric lesions were observed in 189 (49.1 %) and 196 (50.9%) patients, respectively. In the multivariate analysis, enlarged left atrium (LA) (adjusted odds ratio [aOR]=1.03, 95% confidential interval [CI], 1.007–1.061; p=0.013) and single confluent lesion pattern (aOR= 1.55, 95% CI, 1.012–2.381; p=0.044) were associated with right hemispheric lesions. ConclusionsEnlarged LA and single confluent lesion pattern were strongly related to right-sided propensity in patients with AF-stroke.

Utility of Lung Ultrasound in Decision-making to Prioritize Hospital Admission for COVID-19 Patients: A Developing Country Perspective.

In the midst of this pandemic, planning the prioritization of hospital admissions for patients affected with COVID-19 should be of prime concern, particularly in healthcare settings with limited resources. Thus, in this study, we aimed to develop a novel approach to triage COVID-19 patients and attempt to prioritize their hospital admission using Lung Ultrasonography (LUS). The efficacy of LUS in triaging suspected COVID-19 patients and assessing the severity of COVID-19 pneumonia was evaluated; the findings were then compared with those obtained by chest computed tomography (CT). This multicenter, cross-sectional study comprised 243 COVID-19 patients who presented to the emergency department in 3 major university hospitals in Egypt. LUS was performed by an experienced emergency or chest physician, according to the local protocol of each hospital. Demographic, clinical, and laboratory data were then collected from each patient. Each patient was subjected to chest CT scans and LUS. The mean age of the 243 patients was 46.7 ± 10.4 years. Ground-glass opacity, subpleural consolidation, translobar consolidation, and crazy paving were reported in the chest CT scans of 54.3%, 15.2%, 11.1%, and 8.6% of the patients, respectively. B-line artifacts were observed in 81.1% of the patients (confluent pattern, 18.9%). The LUS findings completely coincided with the CT findings (Kappa agreement value, 0.77) in 197 patients (81.1%) and offered a diagnostic sensitivity of 74%, diagnostic specificity of 97.9%, positive predictive value of 90.2%, and negative predictive value of 93.6% for the COVID-19 patients. Following the addition of O2 saturation to the lung imaging findings, the ultrasound method was able to demonstrate 100% sensitivity and specificity in accurately differentiating between severe and non-severe lung diseases. LUS with oxygen saturation might prove to be effective in prioritizing the hospital admission of COVID-19 patients, particularly in healthcare settings with limited resources.

A Study on Synoptic Conditions Leading to the Extreme Rainfall in Taiwan during 10–12 June 2012

During 10–12 June 2012, heavy rainfall occurred three days in a row in southern and central Taiwan, with daily rainfall maxima exceeding 500 mm on each day. In the Mei-yu season (May–June) during 1993–2000, only two other rainfall events had a comparable amount and duration, but this case was the only one that occurred well before the arrival of the Mei-yu front. The synoptic conditions and their evolution leading to this unique event are thus important and are the foci of this study. Our analysis indicates that the 10–12 June 2012 event in Taiwan was caused by the strong and persistent west-southwesterly low-level jet (LLJ) that transported warm, moist, and unstable air from upstream and then impinged on the island. The LLJ developed due to the enhanced horizontal pressure (or height) gradient when the pressure at low-levels fell significantly (by ~8 hPa) in South China (north of the jet) during 8–10 June, but the subtropical high to the southeast maintained its strength. Further, through a diagnosis using the pressure tendency equation, it is found that both warm air advection and the dynamic effects (column divergence and transport of mass by vertical motion) contributed to the pressure fall in South China. The warm air advection occurred in the southern part of a large-scale confluent pattern in China, and the persistent west-southwesterly flow through deep layer (mainly above 800 hPa) in South China transported warmer and less dense air into the region from lower latitudes. On the other hand, South China was also located under the diffluent zone in the northeastern quadrant of the South Asian upper-level anticyclone, which strengthened during 5–10 June and provided divergence aloft, which exceeded the low-level convergence and upward transport of mass (at a fixed height) into the column by vertical motion on 9 June. As a result, the dynamic effects also contributed to the pressure fall, although secondary to the warm air advection. The destabilization process in South China during 8–10 June was also helpful to increase convective activity and upper-level divergence.

18F-FDG PET/CT imaging features of patients with multicentric Castleman disease.

ObjectiveThe aim of this study is to investigate the role of 18F-fluorodeoxyglucose (FDG) PET/computed tomography (CT) in the evaluation of multicentric Castleman disease (MCD).MethodsThirty-five patients with pathologically confirmed MCD who underwent 18F-FDG PET/CT were retrospectively included. The FDG uptake and CT findings of lymph nodes, pulmonary involvement, spleen, and bone marrow were assessed and the maximum standardized uptake value (SUVmax) of each lesion was measured. The locations of lymph nodes were also evaluated.Results18F-FDG PET/CT showed increased uptake in multiple nodal regions in 34 out of 35 MCD patients. The most frequently involved nodal sites were the cervical, iliac, axillary, and inguinal areas, and the least common was paraaortic and abdominal nodes. The involved lymph nodes were not confluent and presented a relatively symmetric pattern on PET/CT images. The highest SUVmax of lymph nodes per patient ranged from 2 to 19 with a mean value of 5.61 ± 3.12. Pulmonary manifestation including cysts, nodules, and interstitial lung disease were found in 10 patients, eight of whom demonstrated mild to moderate uptake in the lungs. 18F-FDG PET/CT also revealed other findings including hypermetabolic spleen (n = 8) and bone marrow (n = 23), elevated uptake in salivary glands (n = 8). Four patients also underwent follow-up PET/CT scans after therapy, and three of them displayed decreased metabolism.Conclusion18F-FDG PET/CT is a useful tool in the diagnosis, evaluation, and follow-up of MCD by providing systemic manifestations of lymphadenopathy, pulmonary involvement, and hypermetabolic spleen or bone marrow. Furthermore, the lymphadenopathy in MCD presented a predominantly peripheral distribution, relatively symmetric, moderately hypermetabolic, and not confluent pattern on 18F-FDG PET/CT.

Treatment Approaches for Atypical CIDP.

The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. High quality evidence is lacking as far as the management of these atypical variants is concerned. In this review, we summarize the treatment approaches to each of these CIDP variants based on existing data. Distal acquired demyelinating symmetric polyneuropathy (DADS) has the phenotype of a symmetric, demyelinating sensory, length-dependent polyneuropathy and is frequently associated with paraproteinemia and anti myelin associated glycoprotein (MAG) antibodies. While the management of idiopathic DADS (DADS-I) is the same as CIDP, DADS-M responds suboptimally and has a favorable response to rituximab. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) manifests as a chronic progressive demyelinating mononeuropathy multiplex which can evolve to a confluent pattern indistinguishable from CIDP. Evidence favors treating MADSAM with conventional immunomodulatory therapy (IMT), but this disorder responds less favorably than CIDP. Some patients present with purely sensory symptoms, known as pure sensory CIDP or chronic inflammatory sensory polyradiculoneuropathy (CISP), the latter localizing to a pre-ganglionic pathology. Both respond well to first line IMT, particularly to intravenous immunoglobulin (IVIG), but patients relapse without maintenance therapy. Pure motor CIDP resembles multifocal motor neuropathy with conduction block (MMNCB), but the previously reported worsening status after steroid treatment was not reproduced in recent studies, and IVIG remains the first-line therapy. Some focal forms of CIDP defy exact classification, but respond well to first-line IMT including IVIG. Overall, atypical CIDP responds to treatment with first-line IMT, but has a suboptimal response compared to CIDP. There is evidence for effectiveness with agents such as rituximab, especially in DADS-M, and this medication can also be used in cases refractory to conventional IMTs. Rituximab is also effective in CIDP with IgG4 antibodies which has distinct clinical features and is mostly refractory to first-line IMT.

Confluent and Reticulate Papillomatosis: A Retrospective Study from southern India.

Background:Confluent and reticulate papillomatosis (CRP) is an uncommon benign, acquired keratinization disorder. Studies on this disorder are lacking except for a few case reports and there is a paucity of Indian literature on the condition.Objectives/Methods:To study and describe the various morphological patterns and histopathological findings, as well as assess the response to treatment of 30 patients diagnosed with CRP.Results:Thirtypatients with a diagnosis of confluent and reticulate papillomatosis were included in the study. The male to female ratio was 1:1.5. Mean age at onset of skin eruptions was 27.3 years and mean duration of skin eruptions was 8.2 months. Most of the patients (60%) were asymptomatic. The majority (66.7%) had lesions distributed over upper trunk. Two-thirds of patients had typical brown macules in confluent and reticulate pattern. KOH mount was done in 24 cases and was positive in three cases (12.5%) for yeast-like hyphae. Biopsy demonstrated variable degrees of hyperkeratosis, papillomatosis, and moderate acanthosis. Thirteen out of eighteenpatients on minocycline showed complete clearance within 3 weeks andthreepatients had more than 50% improvement at the end of 3 weeks. Doxycycline showed satisfactory response but results were less satisfactory with azithromycin.Conclusion:CRP is an uncommon condition. There is a paucity of large studies in Indian literature. The present study highlightssuch a large cohort of cases. Prevalence of CRP was more in female in contrast to western studies. Association of CRP with hyperthyroidism was described in many studies but the present study highlights the association with hypothyroidism. Morphological variants like shiny atrophic lesions, verrucous lesions, and involvement of atypical sites like forearm have been described. Role of minocycline in the management of chronic and recurrent cases has been reinforced.

Composite Vertical-Motion Patterns near North American Polar–Subtropical Jet Superposition Events

AbstractA polar–subtropical jet superposition is preceded by the development of a polar cyclonic potential vorticity (PV) anomaly at high latitudes and a tropical anticyclonic PV anomaly at subtropical latitudes. A confluent large-scale flow pattern can lead to the juxtaposition of these respective PV anomalies at middle latitudes, resulting in the addition of the nondivergent circulations induced by each PV anomaly and an increase in upper-tropospheric wind speeds at the location of jet superposition. Once these PV anomalies become juxtaposed, vertical motion within the near-jet environment facilitates the advection and diabatic redistribution of tropopause-level PV, and the subsequent formation of the steep, single-step tropopause structure that characterizes a jet superposition. Given the importance of vertical motion during the formation of jet superpositions, this study adopts a quasigeostrophic (QG) diagnostic approach to quantify the production of vertical motion during three types of jet superposition events: polar dominant, eastern subtropical dominant, and western subtropical dominant. The diagnosis reveals that the geostrophic wind induced by polar cyclonic QGPV anomalies is predominantly responsible for QG vertical motion in the vicinity of jet superpositions. The QG vertical motion diagnosed from the along-isotherm component of the Q vector, which represents the vertical motion associated with synoptic-scale waves, is dominant within the near-jet environment. The QG vertical motion diagnosed from the across-isotherm component of the Q vector, which represents the vertical motion associated with frontal circulations in the vicinity of the jet, is subordinate within the near-jet environment, but is relatively more important during eastern subtropical dominant events compared to polar dominant and western subtropical dominant events.

Evaluation of Serum levels of epidermal growth factor (EGF) in Patients with alopecia areata

Alopecia areata is a common cause of non-scarring alopecia that occurs in a patchy, confluent or diffuse pattern. The EGF can promote growth of the ORS compartment of mink hair follicles in vitro, and enhance proliferation and migration of the ORS cells via activation of the Wnt/β-catenin signaling pathway.Evaluation of serum levels of EGF in patients with alopecia areata.Serum EGF concentrations were measured in 60 consecutive patients with alopecia areata in comparison with 25 controls using enzyme-linked immunosorbent assay (ELISA).Serum levels of EGF were found to be higher in patients than in healthy controls.increased serum levels of EGF in patients than in controls probably reflect the role of EGF in the pathogenesis of alopecia areata.

Abstract P3-08-54: Pattern of hepatic metastasis as a prognostic index in breast cancer liver metastasis

Abstract Background Liver is one of a common site of metastasis in metastatic breast cancer, and associated with visceral crisis in breast cancer. The clinical course and prognosis of breast cancer with liver metastasis (BCLM) is quite different with other solid cancers presenting with liver metastasis. BCLM patients frequently develop hepatic failure due to liver metastasis, but there are no clinicopathologic or radiologic parameters estimating the prognosis of BCLM during their clinical course. In this study, we analyzed the relationship of radiologic, clinicopathologic characteristics and survival outcomes in BCLM patients. Methods Between January 2009 to May 2019, 156 BCLM patients were analyzed. Baseline and final abdomen CT scan or liver MRI during treatment was reviewed by experienced radiologist. The pattern of liver metastasis was classified as oligometasis (3 or less metastatic lesions), non-confluent mass formation, confluent mass formation, infiltrative and pseudocirrhosis pattern. Clinicopathologic characteristics including child-pugh score (CPS) and survival outcomes including liver metastasis associated overall survival (LMOS) was analyzed Results Among 156 patients, 77 patients were initially presented with oligometastasis, 58 patients with non-confluent mass-forming pattern, 14 patients with confluent mass formation, and 7 patients with infiltrative liver metastasis. Patients who showed liver metastasis at initial recurrence or metastasis showed confluent mass-forming (9 patients, 64.3%) or infiltrative liver metastasis (6 patients, 85.7%) as presentation. At patient’s death, initial confluent mass-forming or infiltrative liver metastasis patients showed hepatic dysfunction with CPS 2 or 3. Patients with oligometastasis showed superior LMOS compared to non-confluent mass-forming, confluent mass-forming and infiltrative liver metastasis with worst survival outcome (median LMOS 55.03 months vs. 36.87, 36.93 and 8.8 months, P=0.0021). In luminal B patients, radiologic liver metastasis pattern at patient’s death or last follow-up were associated to cause of patient’s death such as hepatic failure (P=0.044). There were no survival benefit of patients who received local treatment for liver metastasis, compared to patients who only received systemic chemotherapy. Conclusion Initial hepatic metastasis pattern were associated with LMOS, especially infiltrative liver metastasis associated to poor survival outcome. There were no survival benefit of local treatment of liver metastasis during follow-up. Authors are planning on subsequent analysis based on pathologic classification of liver metastasis. Citation Format: Jieun Lee, Moon Hyung Choi, Ahwon Lee, Sung Hak Lee. Pattern of hepatic metastasis as a prognostic index in breast cancer liver metastasis [abstract]. In: Proceedings of the 2019 San Antonio Breast Cancer Symposium; 2019 Dec 10-14; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2020;80(4 Suppl):Abstract nr P3-08-54.

Abstract WP65: Clinical and Radiographic Characterization of Cerebral Hyperdensities on Post-thrombectomy Conventional Non-contrast Ct Brain: Differentiating Between Contrast Staining and Post-thrombectomy Hemorrhage

Background: Post-interventional cerebral hyperdensities (PCHD) are present 31.2 to 87.5% of the time on post-thrombectomy (PT) CT. It can be difficult for radiologists to differentiate if PCHD represents intracerebral hemorrhage (ICH) or contrast staining using conventional CT. The ability to accurately determine the etiology of PCHD may be important for a patient’s outcome. Methods: We retrospectively investigated clinical risk factors, imaging findings, and interventional technique of patients who had a thrombectomy from 2011-2017 (n=238) at a Comprehensive Stroke Center. 112 patients with anterior circulation infarcts and immediate PT CTs as well as either a PT MRI or follow-up CT within 48 hours were included. Two experienced neuroradiologists interpreted all imaging. Baseline demographics and imaging characteristics were collected. The presence of ICH was determined by dephasing on gradient echo or by its persistence &gt; 2 days on repeat CT. Chi-square and Fisher’s exact tests were used for statistical significance, and logistic regression to determine risk estimates. Results: 84% of patients had PCHD, of which 56% had ICH. Patients had similar demographics (ICH: median age 69, 47.5% female; non-ICH: median age 71, 42.5% female). Higher pre-thrombectomy ASPECTS score (OR 0.62, p &lt; 0.05) decreased the risk of ICH, and higher Hounsfield units (HU) of PCHD both on immediate (OR 1.04, p &lt; 0.05) and intermediate (OR 1.1, p &lt; 0.0005) PT CT increased the risk of ICH. PCHDs in the deep grey matter (OR 2.84, p &lt; 0.05) and demonstrating a confluent pattern (OR 3.9 p &lt; 0.05) increased the likelihood of ICH. 10% increase risk of ICH was seen for every HU increase in density on PT CT at 24 or 48 hours. A 50% decrease in ICH was observed for each unit increase of the pre-thrombectomy ASPECTS score. ROC revealed the optimal cut-off for predicted probability as 0.64, sensitivity 70.2% and specificity 69.2%. The AUC for the predicted model was 0.84 (95%CI= (0.75- 0.92, p &lt;0.0001). Conclusion: Several imaging characteristics of PCHDs and ASPECTS score can help differentiate between the PT ICH and contrast staining. Application of these variables to patients in the immediate PT period may change clinical management.

Multivariate analyses of MRI findings for predicting osteomyelitis of the foot in diabetic patients

There have been no previous magnetic resonance imaging (MRI) studies using multivariable analysis to diagnose osteomyelitis in patients with diabetic foot. To retrospectively investigate the MRI findings of osteomyelitis in patients with diabetic foot using multivariate analyses. From November 2015 to March 2018, 118 patients who underwent MRI of the foot to evaluate suspected osteomyelitis were included in this study. The patients were categorized into the presence or absence of osteomyelitis. The primary and secondary MRI findings were retrospectively reviewed. To identify independent predictive MRI findings, multivariate analyses with binary logistic regression and receiver operating characteristic curve analyses were performed including all 118 patients and 93 patients presenting decreased T1 signal intensity, respectively. T1 signal intensity, T1 marrow pattern, T1 marrow distribution, T2 signal intensity, concordance of marrow signal intensity, cortical interruption, ulcer depth, abscess, and wet gangrene were significantly different between the two groups (P < 0.05). Multivariate analyses indicated that fluid equivalent T2 signal intensity, deep ulcer, and confluent T1 marrow pattern were major factors associated with osteomyelitis. The area under the curve of predicted probabilities for the combination of these factors was 0.799 across all 118 patients and 0.761 across 93 patients with decreased T1 signal intensity. Confluent T1 marrow pattern is a reliable finding to suggest osteomyelitis in patients with diabetic foot. In addition, fluid equivalent T2 signal intensity and deep ulcer are important findings that may suggest osteomyelitis, irrespective of T1 signal intensity change.

Phenotypic Spectrum of Pentosan Polysulfate Sodium–Associated Maculopathy

A unique pigmentary maculopathy was recently described in 6 patients with long-term exposure to pentosan polysulfate sodium (PPS), a long-standing oral therapy for interstitial cystitis. To characterize the exposure characteristics and clinical manifestations of PPS-associated maculopathy. In this multi-institutional case series, medical records of patients who exhibited the characteristic maculopathy in the setting of prior PPS exposure were retrospectively reviewed. Data were collected from August 1, 2012, to October 1, 2018, and data were analyzed from October 2018 to January 2019. Drug exposure, visual acuity, and retinal imaging characteristics. Of the 35 included patients (70 eyes), 34 (97%) were female, and the median (range) age was 60 (37-79) years. The median (range) duration of PPS intake was 15 (3-22) years, and the median (range) cumulative exposure was 1.61 (0.44-4.31) kg. The leading visual symptoms were metamorphopsia, blurred vision, and prolonged dark adaptation. Median (range) logMAR visual acuity of all eyes was 0.10 (-0.12 to 1.18). Fundus examination often revealed hyperpigmented macular spots (34 of 64 eyes [53%]) with interspersed pale-yellow deposits, although less commonly in eyes that exhibited retinal pigment epithelial atrophy (6 of 26 eyes [23%]; P < .001). Optical coherence tomography showed foci of retinal pigment epithelium elevation or thickening associated with hyperreflectance on near-infrared reflectance imaging. Fundus autofluorescence imaging typically revealed a symmetric, confluent pattern of hyperautofluorescent and hypoautofluorescent spots that involved the fovea in all eyes and extended to the retinal periphery in 24 eyes (36%). Longitudinal evaluation demonstrated dynamic changes in pigmentary abnormalities. These findings suggest that PPS-associated maculopathy is a vision-threatening condition that can manifest in the setting of long-term exposure to the drug. Multimodal imaging posits a distinctive clinical phenotype, characterized in this cohort by dynamic alterations within the retinal pigment epithelium and at the retinal pigment epithelium-photoreceptor interface. Ongoing work might explore causality and direct screening guidelines.

Expanded study on the risk of lymphovascular space invasion and lymph node metastasis of endocervical adenocarcinoma using Pattern Classification: a single-centre analysis of 213 cases

A new three-tiered Pattern Classification system for usual-type endocervical adenocarcinomas (U-EACs) recommends using tumour invasive patterns rather than depth of invasion (DOI) and horizontal spread to categorise tumours. Tumours categorised by Pattern Classification are associated with lymph node (LN) metastasis and adverse outcomes. The aim of this study is to further explore the potential of Pattern Classification in surgical pathology practice. A total of 213 consecutive cases [201 U-EACs and 12 gastric-type adenocarcinomas (GACs)] diagnosed between 2006 and 2017 was retrospectively analysed. Clinicopathological data included age at diagnosis, DOI measurement, the status of lymphovascular space invasion (LVSI) and LN metastasis, and the number of LVSI foci, dissected and metastatic LNs. Immunostaining for CD34 and D2-40 was performed to identify LVSI in 14 challenging cases. Overall, mean age at diagnosis was 51 years (range 23-75). LVSI and LN metastasis occurred in 128 (60.1%, 128/213) and 42 (20.5%, 42/205) cases, respectively. Also, 28 (13.1%), 21 (9.9%), and 164 (77.0%) patients had pattern A, B, and C tumours, respectively. Patients with pattern C tumours had the oldest age at diagnosis (p=0.007), the highest incidence of LVSI and LN metastasis, and the highest DOI (p<0.001). Due to a highly heterogeneous growth pattern, pattern C U-EACs were stratified into four subgroups: C1, C2 and C3 corresponded to solid, extensive linear destructive, and band-like lymphocytic infiltrate growth patterns, respectively, and C4 included diffuse destructive, confluent, micropapillary and mixed growth pattern. C2 and C3 subgroup tumours had lower incidence of LVSI (20% and 40%, respectively) than the other two subgroups (p<0.001). None of the patients with C2 and C3 subgroup had LN metastasis and ≥3 LVSI foci. All GACs belonged to pattern C and had deeper stromal invasion (p=0.008), higher incidence of LN metastasis (p=0.001), and larger quantity of LVSI foci (p=0.008) and metastatic LNs (p=0.004) than those of pattern C U-ECAs. Number of LVSI foci were moderately positively correlated with LN metastasis status (p<0.001, γ=0.489) or number of metastatic LNs (p<0.001, γ=0.409). Our study further supports that Pattern Classification is a system easy to follow, which has a strong correlation to LVSI and an effective predictability for LN metastasis. Extensive linear destructive and band-like lymphocytic infiltrate growth patterns in pattern C U-EACs need to be recognised, as they behave less aggressively than that for the other growth pattern subgroups. Our study supports that Pattern Classification can be routinely applied to guide therapies for patients with U-EACs.

Distribution Pattern Making Sense: Patients Achieve Rapider Pain Relief with Confluent Rather Than Separated Bilateral Cement in Percutaneous Kyphoplasty for Osteoporotic Vertebral Compression Fractures

It has been reported the distribution of bone cement in percutaneous kyphoplasty (PKP) has an impact on the curative effect. No studies have compared between confluent and separated cement pattern of bilateral bone cement in PKP for patients with osteoporotic vertebral compression fractures. Between 2010 and 2016, 1341 patients were enrolled and divided into 2 groups. Group A (n= 723), bilateral cement was confluent; Group B (n= 618), bilateral cement was separated. The visual analogue scale (VAS), Oswestry Disability Index (ODI), anterior vertebral height (AVH), and local kyphotic angle (LKA) were obtained preoperatively, 2 days after surgery, and at the final follow-up to assess the functional and radiographic efficacy of the surgery. The VAS, ODI, AVH, and LKA 2 days after operation and at the final follow-up were significantly improved compared with the preoperative for both groups (P < 0.05). There existed no significant difference between groups at various time point in ODI, AVH, and LKA (P > 0.05). Group A showed better VAS than group B 2 days after surgery (1.91 ± 0.98 vs. 2.35 ± 0.78, P < 0.001), also with better pre-postoperative VAS change (6.23 ± 0.76 vs. 5.75 ± 1.02, P < 0.001). Multiple linear regression for pain relief degree revealed group A (P < 0.001), older age (P < 0.001), and more cement volume (P < 0.001) contribute to rapid improvement of back pain. The cement leakage rate was 3.7% in group A and 2.9% in group B, with no significant difference (P= 0.405). Patients achieved rapider pain relief with confluent rather than separated bilateral bone cement pattern in PKP for osteoporotic vertebral compression fracture.

MRI Diagnosis for Post-Traumatic Osteomyelitis of Extremities Using Conventional Metal-Artifact Reducing Protocols: Revisited

To assess the diagnostic power of MR imaging features for post-traumatic osteomyelitis (PTOM) of the extremities with metal implants using a 3.0-T machine with conventional metal-artifact reducing (MAR) protocols. Between December 2012 and September 2016, 261 consecutive patients underwent MRI for clinical suspicion of PTOM in the appendicular skeleton at our hospital. A total of 72 enrolled patients with metal implants were divided into two groups, one with surgical, histologic, or microbiologic proven to be PTOM, and the other proven not to be PTOM. Their MR images were reviewed by two musculoskeletal radiologists who qualitatively analyzed various findings regarding PTOM, and concluded diagnosis of PTOM or without PTOM for each patient. All MR images were obtained using conventional MAR protocols. The sensitivity, specificity, positive and negative predictive values, and accuracy of MR diagnosis by two observers were 55%/38%, 81%/93%, 67%/40%, 73%/78%, and 71%/71%, respectively. Among the findings, T1 low signal intensity, medullary location, confluent pattern, typical features (concurrence of T1 low signal intensity, medullary, and confluent patterns), heterogeneous or no enhancement, and cortical destruction were statistically significant by univariate analysis (p<0.05). Among these features, only medullary involvement was significant by multivariate analysis (p = 0.007). Medullary involvement and no enhancement pattern were significant by step-wise discrimination analysis. Interobserver correlation was moderate with a weighted kappa value of 0.512 (confidence interval: 0.3-0.723). The overall accuracy for diagnosis of PTOM of the extremities using 3.0-T MRI with conventional metal-artifact reduction parameters was 71%. The strongest diagnostic MR imaging feature was medullary involvement of T1 low signal intensity. PTOM of the extremities can be accurately diagnosed with a 3.0-T MR machine using conventional MAR protocols.

Electrophysiological function in eyes with reticular pseudodrusen according to fundus distribution.

Reticular pseudodrusen (RPD) could be present not only in the posterior pole but extramacular area also as a confluent morphological pattern. Thus RPD can be classified by the fundus distribution for the assessment of visual prognosis. The electrophysiological function in eyes with reticular pseudodrusen (RPD), showing various fundus distribution were evaluated using full-field electroretinogram (ERG). Retinal distribution of RPD was divided into three types (localized, intermediate, and diffuse) according to the extent of involvement of retinal areas by fundus photograph montages. RPD were present with the diffuse type in 21 eyes (25.6%), with the intermediate type in 30 eyes (36.6%), and with the localized type in 31 eyes (37.8%). The average age was 74.76 ± 4.52 (range, 65–81) years in the diffuse type, 72.47 ± 9.13 (range, 55–91) years in the intermediate type, and 70.26 ± 7.77 (range, 61–89) years in the localized type. The mean amplitudes of the scotopic rod response, scotopic maximal combined response, oscillatory potentials (OP), photopic cone response, and 30Hz cone flicker response were more decreased in the diffuse, intermediate, and localized types in order, except for the photopic cone a-wave response. The diffuse type showed reduced amplitudes of ERG responses than the normal control group under all testing conditions except for the photopic a-wave response, and differences were statistically significant with the age restriction and adjustment methods (Bonferroni-corrected P < 0.05). The mean implicit times of ERG responses were significantly delayed in the diffuse type in the photopic b-wave. (Bonferroni-corrected P < 0.05). Extensive retinal involvement of RPD correlates with severely reduced electrophysiological retinal function. This acquired form of decreased electrophysiological function should be regarded as different from those of hereditary retinal degeneration.

A Case of Gastric Neuroendocrine Neoplasm with Mixed Grade: a Distinct Type of “High”-grade Well-Differentiated Neuroendocrine Neoplasm

Grade 3 neuroendocrine tumor (NET G3) is a rare new entity that has recently been introduced in the classification of neuroendocrine neoplasms of the pancreas. It is a well-differentiated form, with a high proliferative activity (Ki67 > 20%), and it represents a category whose prognosis is intermediate between NET G2 and poorly differentiated neuroendocrine carcinoma (NEC G3). In sites other than the pancreas, this new category is by far less coded. Herein is reported a case of NET G3 of the stomach, the 13th described in literature. The patient was a 71-year-old man with a mass in the gastric fundus. Histological examination revealed a neuroendocrine tumor that was mostly composed by areas with conventional well-differentiated features and intermediate proliferative rate (< 20 mitoses/10 HPFs and 7% Ki67); part of the tumor was characterized by increased nuclear atypia, a confluent growth pattern, intermediate mitotic activity, and high Ki67 value (25%). The tumor was studied immunohistochemically and molecularly, and the only alteration that came out was MEN1 gene mutation (Lau584SerfsTer5) in both the high- and low-grade components. According to the actual classification of gastric neuroendocrine neoplasms, this case is labeled as NEC G3 but it should be named NET G3, with a much better prognosis than the former. Overall, both morphological pattern and the Ki67 value should be mentioned in the histological report in cases of high-grade neuroendocrine neoplasms because of its clinical significance in defining the prognosis and for new potential therapeutic approaches.