Neuro-Behçet's disease: A review.

Behcet disease (BD) is a systemic vasculitis which can involve many different organ systems. Neurological involvement (NBD) occurs in 5.3% to 59% of BD patients. The diagnosis is challenging especially in case of inaugural neurological presentation, and is based on a constellation of clinical, laboratory, and neuroimaging findings. NBD can be subdivided into parenchymal NBD through an immune mediated meningoencephalitis with a predilection to the brainstem, basal ganglia, thalamus, cranial nerves, and spinal cord involvement, and extraparenchymal NBD encompassing cerebral veinous thrombosis and intracranial arterial involvement. Brain magnetic resonance shows ill-defined areas of oedema with high signal intensity on T2-FLAIR images, isointense or hypointense in T1-weighted images in the basal ganglia area or in the brainstem, which may extend to the diencephalic structures. Swelling might be noticed. Hemorrhages can be seen, such as contrast enhancement (blood brain barrier disruption). Magnetic resonance venography and computerized tomographic angiography can be used to diagnose extraparenchymal NBD. Treatment of parenchymatous forms is based on glucocorticoids associated with oral immunosuppressants (azathioprine, mycophenolate mofetil or methotrexate) in mild forms, and intravenous cyclophosphamide or infliximab in severe forms. The management of cerebral thrombosis consists of steroids course associated with an oral anticoagulation. An early recognition of this condition is mandatory to initiate adequate therapies in order to improve outcomes and limit the risk of sequelae, relapses, or death. The aim of this review is to summarize a comprehensive review on the various neurological presentations of BD with emphasizes on diagnostic tools, prognosis, and therapeutic issues.

Annular Leukocytoclastic Vasculitis: A New Feature of IgA Vasculitis.

Background: Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases. Case description: A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse. Conclusion: An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis. LEARNING POINTS: Annular erythema may reveal an IgA vasculitis.Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.This presentation is treated with topical steroids.

Chlamydia psittaci-related pleuro-myocarditis.

Chlamydia psittaci ‒ related community-acquired pneumonia associated to acute myocarditis was diagnosed in a young man with no medical history, and a professional exposition to birds. The diagnosis was confirmed with positive specific polymerase chain reaction in bronchoalveolar lavage. The patient was treated with spiramycin for two weeks with anti-inflammatory treatment for myocarditis for three months. Clinical and biological improvement was rapidly observed followed by normalization of electrocardiogram and chest CT scan. No relapse was reported for over a two-year follow-up.

Successful Introduction of Benralizumab for Eosinophilic Ascites.

Eosinophilic ascites is a rare disorder, reported in both adult and pediatric patients, characterized by high eosinophil counts in the peritoneal fluid. Eosinophilic ascites appears as a manifestation of various diseases such as parasitic and fungal infections, malignancy, and hypereosinophilic syndrome. It also represents an uncommon manifestation of eosinophilic gastroenteritis, usually treated with corticosteroids. We present the case of a 16-year-old woman with abdominal distention related to abundant ascites. Further work-up concluded that it was eosinophilic gastroenteritis complicated with eosinophilic ascites. The patient was on oral steroids for three weeks, but various abdominal relapses were observed, leading to the introduction of benralizumab, as a steroid-sparing therapy with a favorable outcome.

Lactobacillus rhamnosus a cause of Gram-positive rods bacteremia after prophylactic probiotic consumption.

•Lactobacillus rhamnosus bacteremia was diagnosed in a patient with radiation enteritis after prophylactic probiotic consumption.•L. rhamnosus was identified by culture and MALDI-TOF-MS.•L. rhamnosus may have high MIC values for amoxicillin.•In case of L. rhamnosus bacteremia, bowel diseases should be considered as a possible source of infections.

Benralizumab for eosinophilic granulomatosis with polyangiitis.

BACKGROUND: Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series. METHODS: We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab. The primary endpoint was the rate of complete response defined as no disease activity (Birmingham Vasculitis Activity Score=0) and a prednisone dose ≤4 mg/day. Partial response was defined as no disease activity and a prednisone dose ≥4 mg/day. RESULTS: Sixty-eight patients were included, including 31 (46%) who had previously received mepolizumab. The use of benralizumab was warranted by uncontrolled asthma in 54 (81%), persistent ear, nose and throat (ENT) manifestations in 27 (40%) and persistent glucocorticoids (GCs) use in 48 (74%) patients. Median (IQR) follow-up after starting benralizumab was 23 (9-34) months. Thirty-three patients (49%) achieved a complete response, 24 (36%) achieved a partial response and 10 (15%) did not respond. Among the 57 patients who initially responded, 10 (18%) eventually required further line treatments. GCs were discontinued in 23 patients (38%). Prior mepolizumab use was associated with a higher rate of primary failure (26.7% vs 5.4%, p=0.034) and less frequent GCs discontinuation (14.8% vs 55.9%, p=0.001). Vasculitis flares occurred in 7 patients (11%) and were associated with histological evidence of vasculitis and/or antineutrophil cytoplasmic antibodies positivity at benralizumab initiation (p=0.004). CONCLUSIONS: Benralizumab appears to be an effective treatment for refractory asthma or ENT manifestations in EGPA and allows GC-sparing. However, its efficacy was lower after prior failure of mepolizumab.

State of the Art of Cardiac Amyloidosis.

Cardiac amyloidosis is defined by extracellular deposition of misfolded proteins in the heart. The most frequent cases of cardiac amyloidosis are caused by transthyretin and light chain amyloidosis. This condition is underdiagnosed, and its incidence has been continuously rising in recent studies because of the aging of the population and the development of noninvasive multimodal diagnostic tools. Amyloid infiltration affects all cardiac tunics and causes heart failure with preserved ejection fraction, aortic stenosis, arrythmia, and conductive disorder. Innovative, specific therapeutic approaches have demonstrated an improvement in affected organs and the global survival of patients. This condition is no longer considered rare and incurable. Thus, better knowledge of the disease is mandatory. This review will provide a digest of the clinical signs and symptoms of cardiac amyloidosis, the diagnostic tools used to confirm the diagnosis, and current symptomatic and etiopathogenic management considerations according to guidelines and recommendations.

Atrial Septal Defect Associated With Right Bundle Branch Block Masking Brugada Syndrome.

A type 1 Brugada electrocardiogram pattern may be masked by typical right bundle branch block aspect. We present 2 cases (male patients, aged 18 and 22 years) of associated ostium secundum atrial septal defect with a right bundle branch block aspect and symptomatic confirmed Brugada syndrome. Both patients underwent cardiac defibrillator implantation. (Level of Difficulty: Advanced.).

Consecutive Severe Orofacial Complications in Intensive Care Unit Patients: Quincke's Disease and Macroglossia due to Prolonged Prone Positioning for Management of Acute Respiratory Distress Syndrome.

Critically ill patients admitted into the intensive care units are susceptible to a wide array of complications that can be life-threatening, or lead to long-term complications. Some complications are inherent to the patient's condition and others are related to therapeutics or care procedure. The prolonged prone positioning and mechanical ventilation devices are the first risk factors for orofacial complications. We report the case of a 47-year-old male patient, with a history of sleep apnoea syndrome, morbid obesity (body mass index of 43 kg/m2), and gastroesophageal reflux disease, presented to the emergency department with recent otorhinolaryngological symptoms of dysphonia and exertional dyspnoea lasting two days, and complicated with Quincke's disease. First-line treatment consisted of a compilation of intravenous antihistamines and corticosteroids. The patient's condition worsened. He developed an acute respiratory distress syndrome secondary to ventilator-acquired pneumonia with prone positioning ventilation, complicated by severe macroglossia. Soaked gauze dressings were placed around his tongue. Progressively, the size of his tongue reduced. LEARNING POINTS: Intensive care unit (ICU) patients are susceptible to a wide array of life-threatening complications that can be linked.Oral severe acquired Quincke's disease is an isolated form of angioneurotic oedema that is induced by several factors including gastroesophageal reflux disease, sleep apnoea, inhalation exposure, or drug reactions. Macroglossia is rare life-threatening complication due to prolonged prone positioning of unknown pathogenesis. An experienced critical care staff with standardised protocol is needed to prevent such a complication.Because of possible consecutive severe orofacial complications, prolonged prone positioning for management of acute respiratory distress syndrome (ARDS) is not recommended in patients with inaugural oral angioedema.

Massive vulvar edema revealing idiopathic systemic capillary leak syndrome.

Idiopathic systemic capillary leak syndrome is a rare disease characterized by recurrent episodes of hypotension, hypoalbuminemia and peripheral edema caused by capillary hyperpermeability with approximately 350 documented cases worldwide. We report herein the case of a 22-year-old primiparous patient with an unusual compartment syndrome represented with spontaneous massive vulvar edema. Treatment consisted of volume replacement and intravenous polyvalent immunoglobulins perfusions. Vulvar edema was treated surgically by incision and drainage with favorable outcome. The pregnancy follow-up did not show any fetal abnormalities. The childbirth at 35 weeks was natural and the newborn was healthy.

Leg-type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease.

Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disease of unknown etiology. Deciphering mechanisms involved in CD pathogenesis may help improving patients' care. Six cases of stereotyped sub-diaphragmatic iMCD affecting lower limb-draining areas and associated with severe and often ulcerative lower extremity chronic dermatological condition were identified in our cohort. Pathological examination revealed mixed or plasma-cell type MCD. In three patients, shotgun metagenomics failed to identify any pathogen in involved lymph nodes. Antibiotics had a suspensive effect while rituximab and tocilizumab failed to improve the condition. This novel entity requires a specific approach and exclusion of potentially harmful immunomodulation.

Cardiovascular Magnetic Resonance Imaging Pattern in Campylobacter jejuni-related Myocarditis.

BACKGROUND: Campylobacter jejuni (C. jejuni) is a common cause of mostly self-limiting enterocolitis. Although rare, myocarditis has been increasingly documented as a complication following campylobacteriosis. Such cases have occurred predominantly in younger males and involved a single causative species, namely C. jejuni. CASE REPORT: We report herein a case of myocarditis complicating gastroenteritis in a 23-year-old immunocompetent patient, caused by this bacterium with a favorable outcome. Cardiac magnetic resonance imagining was useful in establishing an early diagnosis. CONCLUSIONS: Myocarditis should be considered in younger patients presenting with chest pain and plasmatic troponin elevations. The occurrence of myocarditis complicating C. jejuni is reviewed.

The Predictive Value of Cell Blood Count Parameters to Diagnose Pulmonary Embolism in Patients with SARS-CoV-2 Infection: A Case Control Study.

Introduction: Acute pulmonary embolism (aPE) is frequently associated with coronavirus infectious disease-2019 (COVID-19) with an incidence of more than 16%. Among the new promising biomarkers of aPE, neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) showed correlations with aPE prognosis. The aim of this study was to conduct an exploratory analysis to check the possible role of cell blood count (CBC) parameters as diagnostic and prognostic biomarkers of aPE in COVID-19 patients. Materials and Methods: A case control study was conducted. Two populations were compared: (i) patients hospitalised from 31 January 2020 to 30 June 2021 with severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection and aPE confirmed at angio computed tomography (aCT) or pulmonary scintigraphy (COVID-19 aPE group); (ii) patients hospitalised from 31 January 2017 to 30 June 2021 without SARS-CoV-2 infection whose suspicion of aPE was excluded by aCT or pulmonary scintigraphy (no-aPE group). Results: Overall, 184 patients were included in the study, 83 in COVID-19 aPE group and 101 in no-aPE group. At the univariate analysis, COVID-19 patients with aPE had higher NLR, PLR, neutrophil and lymphocyte counts than patients without aPE (p < 0.05). No significant difference was found in mean platelet volume and platelet counts. No difference in mortality rate was detected. At the multivariate analysis, neutrophil and lymphocyte counts were both associated with diagnostic of aPE while no CBC parameters were associated with mortality at day#7. Conclusions: Neutrophiland lymphocyte counts could be predictors of the early detection of aPE in COVID-19 patients. The value of CBC indices as biomarkers of aPE in daily clinical practice needs to be investigated in further studies.

Risk of Multidrug Resistant Bacteria Acquisition in Patients with Declared ß-Lactam Allergy during Hospitalization in Intensive Care Unit: A Retrospective Cohort Study (2007-2018).

INTRODUCTION: The risk of extended spectrum ß-lactamase (ESBL) bacterial acquisition in patients with ß-lactam allergy has been poorly investigated. In a previous study conducted over a 6-year long period (2007-2012), we found that patients with declared ß-lactam allergy had a higher risk of ESBL bacterial carriage at admission in intensive care unit (ICU), but they had not a higher risk of ESBL bacterial acquisition. We present the final results of the study which was eventually conducted over a 12-year long period (2007-2018). MATERIALS AND METHODS: The study included all patients admitted in ICU and receiving antibiotic treatment from January 2007 to December 2018. ESBL bacterial acquisition was the main clinical outcome. Mortality in ICU, multidrug resistant bacterial carriage at admission and discharge were the secondary outcomes. RESULTS: Overall, 3332 patients were included, 132/3332 (3.9%) were labelled ß-lactam allergic, while 3200/3332 (96.1%) did not presented ß-lactam allergy. No significant difference in rates of ESBL acquisition was detected (4/132, 3% vs. 78/3200, 2.4%; p = 0.17). Patients with ß-lactam allergy had higher rates of ESBL bacterial carriage at admission (19/132, 14.4% vs. 248/3200, 7.8%, p = 0.01) and at discharge (22/132, 16.7% vs. 351/3200, 11%, p = 0.04) than nonallergic patients. No differences in mortality, duration of hospitalization, and carriage of methicillin resistant Staphylococcus aureus were reported. Female gender was the only factor associated with ß-lactam allergy at the multivariate analysis. CONCLUSIONS: This study confirms that patients with declared ß-lactam allergy had not a higher risk of ESBL bacterial acquisition during hospitalization in ICU. However, they had a higher ESBL bacterial carriage at admission.

Atypical miller-fisher syndrome after COVID-19 and sleeve gastrectomy: Contribution of neurochemical markers to early diagnosis.

Background: The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), began in late 2019. More recently, there have been sporadic case reports on development of Miller-Fisher Syndrome , a rare variant of Guillain-Barré Syndrome in COVID-19 patients. Case report: We reported herein the case of a French young women presenting with ophtalmoplegia, cerebellar ataxia, and universal areflexia following a bariatric surgery (sleeve gastrectomy). A concomitant COVID-19 diagnosis was retained based on microbiological testing. The patient was successfully treated after high-dose intravenous thiamine, but areflexia persisted. Underlying COVID-19 related Miller-Fisher Syndrome was established on physical examination and confirmed by pathologic neurophysiological findings and elevated level of phosphorylated neurofilament heavy chain protein in cerebrospinal fluid analysis. Conclusions: Guillain-Barré Syndrome and its variants after SARS-CoV-2 infection are extremely rare. The measurement of phosphorylated neurofilament heavy chain protein should be considered as an easy tool to detect an early affection of the peripheral nervous system.

Pulmonary cavitary disease due to Mycobacterium xenopi.

Non-tuberculous mycobacteria are a significant cause of pulmonary infections worldwide and can be clinically challenging. Mycobacterium xenopi (M. xenopi) has a low pathogenicity and usually requires immunosuppression or lung diseases. We describe herein a scarce case of pulmonary cavitary disease attributed to M. xenopi in a 57-year-old Caucasian patient with underlying structural lung disease successfully treated with rifampicin, ethambutol, clarithromycin, and amikacin.

Extreme gonococcal susceptibility associated with acquired complement deficiency secondary to hypocomplementemic urticarial vasculitis and systemic lupus erythematosus.

Gonococcal infection is rarely associated with septic shock. We describe a recurrent case of septic shock related to disseminated gonococcemia in a patient with systemic lupus erythematosus and hypocomplementemic urticarial vasculitis, and discuss the implication of profound acquired complement deficiency secondary to these auto-immune diseases. This case raises the question of systematic antibioprophylaxis in patients with acquired complement deficiency.