Patterns of Retinal Damage Facilitate Differential Diagnosis between Susac Syndrome and MS

Susac syndrome, a rare but probably underdiagnosed combination of encephalopathy, hearing loss, and visual deficits due to branch retinal artery occlusion of unknown aetiology has to be considered as differential diagnosis in various conditions. Particularly, differentiation from multiple sclerosis is often challenging since both clinical presentation and diagnostic findings may overlap. Optical coherence tomography is a powerful and easy to perform diagnostic tool to analyse the morphological integrity of retinal structures and is increasingly established to depict characteristic patterns of retinal pathology in multiple sclerosis. Against this background we hypothesised that differential patterns of retinal pathology facilitate a reliable differentiation between Susac syndrome and multiple sclerosis. In this multicenter cross-sectional observational study optical coherence tomography was performed in nine patients with a definite diagnosis of Susac syndrome. Data were compared with age-, sex-, and disease duration-matched relapsing remitting multiple sclerosis patients with and without a history of optic neuritis, and with healthy controls. Using generalised estimating equation models, Susac patients showed a significant reduction in either or both retinal nerve fibre layer thickness and total macular volume in comparison to both healthy controls and relapsing remitting multiple sclerosis patients. However, in contrast to the multiple sclerosis patients this reduction was not distributed over the entire scanning area but showed a distinct sectorial loss especially in the macular measurements. We therefore conclude that patients with Susac syndrome show distinct abnormalities in optical coherence tomography in comparison to multiple sclerosis patients. These findings recommend optical coherence tomography as a promising tool for differentiating Susac syndrome from MS

High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study

One view of sarcoidosis is that the term covers many different diseases. However, no classification framework exists for the future exploration of pathogenetic pathways, genetic or trigger predilections, patterns of lung function impairment, or treatment separations, or for the development of diagnostic algorithms or relevant outcome measures. We aimed to establish agreement on high-resolution CT (HRCT) phenotypic separations in sarcoidosis to anchor future CT research through a multinational two-round Delphi consensus process. Delphi participants included members of the Fleischner Society and the World Association of Sarcoidosis and other Granulomatous Disorders, as well as members' nominees. 146 individuals (98 chest physicians, 48 thoracic radiologists) from 28 countries took part, 144 of whom completed both Delphi rounds. After rating of 35 Delphi statements on a five-point Likert scale, consensus was achieved for 22 (63%) statements. There was 97% agreement on the existence of distinct HRCT phenotypes, with seven HRCT phenotypes that were categorised by participants as non-fibrotic or likely to be fibrotic. The international consensus reached in this Delphi exercise justifies the formulation of a CT classification as a basis for the possible definition of separate diseases. Further refinement of phenotypes with rapidly achievable CT studies is now needed to underpin the development of a formal classification of sarcoidosis

Brachial plexus birth palsy shoulder deformity treatment using subscapularis release combined to tendons transfer

SummaryIntroductionOne possible sequela of obstetric brachial plexus palsy (OP) is impaired external rotation (ER) of the shoulder which, in addition to its functional consequences, can generate a posterior humeral head subluxation or dislocation. The goal of the present study was to assess medium-term clinical and radiological results of release of the subscapularis muscle with transfer of the latissimus dorsi and teres major muscles.Patients and methodsFrom 1985 to 1995, a continuous series of 32 OP patients underwent subscapularis muscle release, associated in 24 cases to muscle transfer. Mean age was 2.5 years (range, 1–9.2 years). Shoulder function was assessed by measurement of passive ER and graded according to the modified Mallet classification at 1, 5 and 10 years’ follow-up or before revision. The evolution of the glenohumeral deformity was assessed on CT images of glenoid retroversion and the humeral head subluxation (% of humeral head covered), before and 5 years after surgery.ResultsMean postoperative follow-up was 9.5±5.6 years. Treatment brought significant improvement in passive ER (mean preoperative and 1-year follow-up values: −10° and 52°, respectively). This explained the good modified Mallet score at 1 year: mean=18.4/25. Subsequent significant progressive degradation was noted: 10 years postoperatively, mean ER amplitude and modified Mallet score were respectively 13° and 15.8. The CT study showed correction of the glenoid retroversion (mean preoperative and 5-year follow-up values: 29° and 18°, respectively), and of the humeral head subluxation (mean preoperative and 5-year follow-up values: 25 and 39%, respectively). Surgical revision was indicated six times (five patients): two latissimus dorsi and teres major transfers (not performed initially) and four derotational humeral osteotomies. Three-quarters of the patients who did not initially have muscle transfer had to be reoperated or else showed ER insufficiency at last follow-up.Discussion and conclusionSurgical treatment produces objective functional gain, even if this diminishes over time. Moreover, it prevents or corrects posterior subluxation of the shoulder. It is indicated when passive ER amplitude is negative. It seems advisable to associate release to muscle transfer.Level of evidenceLevel IV Retrospective study

Atteinte coronarienne et syndrome néphrotique au cours du lupus systémique : à propos d’une observation

International audienceIntroduction : Heart failure during systemic lupus erythematosus has various causes.Case report : A 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. The young age and the absence of chest pain were not suggestive of coronary artery disease initially. Coronary thrombosis was probably favored by the nephrotic syndrome, in which the arterial thrombotic risk is increased.Conclusion : Coronary artery disease should be systematically evoked in the presence of ventricular dysfunction in patients with systemic lupus, including when they are young and in the absence of chest pain. Nephrotic syndrome should be identified as a risk factor for arterial thrombosis.Introduction : Les atteintes cardiaques au cours du lupus systémique ont des causes variées.Observation : Une patiente de 29 ans présentait 3 mois après une grossesse une poussée de lupus systémique précédemment connu, avec un syndrome néphrotique, puis un choc cardiogénique nécessitant la pose d’une assistance cardiaque extracorporelle. La dysfonction ventriculaire était liée à un infarctus myocardique massif par thrombose de l’artère interventriculaire antérieure, associée à un athérome sous-jacent. L’âge jeune et l’absence de douleur thoracique étaient peu évocateurs d’une atteinte coronarienne initialement. La thrombose coronarienne était probablement favorisée par le syndrome néphrotique, au cours duquel le risque thrombotique artériel est augmenté, et moins connu que le risque thrombotique veineux.Conclusion : Une atteinte coronarienne doit être systématiquement évoquée devant une dysfonction ventriculaire chez les patients lupiques, y compris lorsqu’ils sont jeunes et en l’absence de douleur thoracique. Le syndrome néphrotique doit être identifié comme un facteur de risque de thrombose artérielle

Association entre les performances cliniques des étudiants et leur réussite aux Épreuves Classantes Nationales informatisées : une étude de cohorte rétrospective monocentrique

International audienceIntroduction: Before attending residency, 6th-year French medical students must validate a final examination including a practical clinical test in their faculty. However, the national ranking that determines their future specialty and region solely relies on a computerized knowledge test. Our goal was to investigate the association between the final faculty test and the national ranking test.Methods: In our faculty, the final examination includes a computerized theoretical test (similar to the national one) and a practical test: a standardized evaluation of semiology skills at the bedside and a standardized assessment of relational skills with role plays. The agreements between the national test and faculty computerized and practical tests were analyzed by intraclass correlation coefficients (ICC).Results: Data from 1806 students who underwent the three examinations from 2017 to 2021 were analyzed. There was a good agreement between the ranks in the faculty and national computerized tests: ICC 0.83 (95% CI 0.81-0.85). By contrast, the agreement between the ranks in the faculty practical test and the national computerized test was poor: ICC 0.13 (95% CI 0.08-0.17). Results were stable over the years.Conclusion: The agreement between the ranking of the current national test and the clinical skills assessed by a specific faculty test is poor. This could relate to a true independence or to different levels of motivation to perform well. Indeed, the result of the national test is the most important one as it determines their career. Incorporating a clinical assessment into the national ranking test will motivate students to acquire clinical skills and value those who perform well this practical dimension