Havana syndrome: Overview for otolaryngologists.

Havana Syndrome is used to describe the array of deficits seen in diplomats stationed in Cuba from August 2016 to September 2017.1 Patients from this cohort were clinically studied, investigating their symptoms, audition and vestibular deficits, and imaging abnormalities seen on MRI.1-3 The primary studies were retrospective, looking at how these symptoms could be classified or fit into diagnostic criteria. The cohort had many similarities: a sound at the onset of symptoms, a mix of neurologic and otologic symptoms, and persistent symptoms that lasted for months.1-3 Theories of the cause of Havana Syndrome have led to no conclusive answer. Potential viral etiologies, mass psychogenic illness, and head trauma were examined as causes.1-5 Diseases with established diagnostic criteria were used to compare the patients' symptoms to try to find a disease that fits Havana Syndrome.3 Recent information from the Office of the Director of National Intelligence suggested the most likely cause to be mass psychogenic illness. With the cause of Havana Syndrome still unknown, a review of reported cases and reports helps otolaryngologists understand Havana Syndrome so they diagnose it only when appropriate and do not miss other conditions that may present with similar symptoms and that might respond well to targeted treatment.

Frequency of misdiagnosis in hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic Cardiomyopathy (HCM) is characterized by unexplained left ventricle hypertrophy (LVH) ≥15 mm. The condition is often hereditary and family screening is recommended to reduce the risk of adverse disease complications and premature death among relatives. Correct diagnosis of index patients is important to ensure that only relatives at risk of disease development are invited for family screening. PURPOSE: To investigate if patients with ICD-10 codes for HCM (DI421) or hypertrophic obstructive cardiomyopathy (DI422) fulfilled recognised diagnostic criteria. METHODS: All patients with ICD-10 codes for HCM or HOCM at a Department of Cardiology were identified and had their diagnosis validated by a cardiac investigation or a review of their medical records and previous investigations. RESULTS: Two hundred and forty patients had ICD-10 codes for HCM/HOCM, of whom 202 (84%, 202/240) underwent re-examination, while 38 (16%, 38/240) had their hospital notes reviewed. Seventy-six patients (32%, n = 76/240) did not fulfil diagnostic criteria, of whom 39, (51%, n = 39/76) had normal (10 mm) or modest LV wall thickness (11-14 mm). The remaining 37 patients (49%, n = 37/76) had LVH ≥15 mm, which was well-explained by uncontrolled hypertension, (32%, n = 24/76), aortic valve stenosis (19%, n = 7/76) or wild-type amyloidosis (16%, 6/76). CONCLUSION: One-third of patients with ICD-10 codes for HCM or HOCM did not fulfil recognised diagnostic criteria. Incorrect diagnosis of HCM may cause unnecessary family investigations which may be associated with anxiety, and a waste of health care resources. This highlights the need for specialised cardiomyopathy services to ensure correct diagnosis and management of HCM.

[Cutting edge of diagnosis and treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) based on the EAN/PNS guideline 2021].

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a most common chronic immune-mediated demyelinating neuropathy, and includes a number of clinical subtypes. The major phenotype is "typical CIDP", which is characterized by symmetric polyneuropathy and "proximal and distal" muscle weakness. During the historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (MAG) neuropathy, and autoimmune nodopathy have been excluded. Currently CIDP is considered as a syndrome including typical CIDP and CIDP variant such as distal CIDP and multifocal CIDP. In 2021, the international guideline of diagnosis and treatment for CIDP, European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) Guideline, was published. This review article introduces the putline of the guideline with medical-social situation in Japan. The diagnosis of CIDP is based on (1) phenotype of typical CIDP or variant, (2) electrophysiologic evidence of peripheral nerve demyelination, and (3) exclusion criteria. The first-line treatments are corticosteroids or immunoglobulin therapy, and plasma exchange should be considered if the 2 treatments were not effective sufficiently. This guideline recommends intravenous or subcutaneous immunoglobulin as a maintenance therapy, and suggests other immune-suppressive agents. In the near future, new treatment with biologics, such as monoclonal antibodies against neonatal Fc receptors, complements, and CD19/20 will be approved.

Evaluation of different screening tools for detection of malnutrition in hospitalised patients.

AIMS AND OBJECTIVES: To assess the prevalence of malnutrition in hospitalised adult patients, and to evaluate the accuracy of the most commonly used nutritional screening tools for identifying individuals at risk of malnutrition. METHODS: A prospective cross-sectional study was conducted on a total of 248 hospitalised patients in internal medicine wards (mean age: 75.2 years; 39.5% females). Nutritional screening was performed within 48 h of admission using the following tools: Malnutrition Universal Screening Tool (MUST), Nutrition Risk Screening Tool (NRS-2002), Malnutrition Screening Tool (MST), Short Nutritional Assessment Questionnaire (SNAQ), and Mini Nutritional Assessment Short Form (MNA-SF). The criteria of the European Society for Clinical Nutrition and Metabolism (ESPEN) were used as the gold standard for defining malnutrition. Patients were also evaluated using the Subjective Global Assessment (SGA) and the Global Leadership Initiative on Malnutrition (GLIM) criteria. Accuracy was determined by examining sensitivity, specificity, and positive and negative predictive values, and diagnostic agreement was determined by calculation of Cohen's kappa (κ). The study is reported as per the Reporting of Observational Studies in Epidemiology (STROBE) guidelines. RESULTS: The ESPEN criteria classified 20.2% of the hospitalised patients as malnourished. Overall, the MUST had the highest sensitivity (80.0%), specificity (74.7%) and positive predictive value (44.4%). For the subgroup of patients aged >65 years, the MNA-SF had high sensitivity (94.4%) but low specificity (39.0%). Based on Cohen's κ, the SGA and GLIM criteria showed low agreement with the ESPEN criteria. CONCLUSION: The MUST was the most accurate nutritional screening tool, through the MST is more easily applied in many clinical settings. A comprehensive assessment of malnutrition that considers muscle mass is crucial for the reliable diagnosis of malnutrition. IMPLICATIONS FOR THE PROFESSION AND/OR PATIENT CARE: The present findings underscore the importance of accurate assessment of the malnutrition status of hospitalised patients and the need for a reliable screening tool. No patient or public contribution.

A patient with Fibromyalgia and Chronic Recurrent Multifocal Osteomyelitis: the importance of psychosomatic assessment.

Background: Many patients affected by FM present different comorbidities, but to date no case of FM in patients with CRMO has been reported in literature. Several studies show the importance of psychosomatic assessment in FM, but only one reported the presence of allostatic overload. Case presentation: In April 2022, a 21-year-old female patient, a third-year medical student, came to our clinic to be assessed and treated for FM. She presents with a diagnosis of CRMO made in 2014 and a diagnosis of FM made in 2019. Results: At the psychiatric evaluation she presented symptoms of anxiety, depression, insomnia and reported widespread pain with the presence of almost daily headaches. From the psychosomatic point of view using DCPR-revised she presented diagnostic criteria for allostatic overload, related to study and periodic flare-ups of painful symptoms due to CRMO, persistent somatization, with musculoskeletal and gastroenterological symptoms, demoralization and type A behaviour. Conclusion: This case shows how useful a psychosomatic assessment of the patient can be for offering insights into what stressors at the origin of allostatic overload may be present in different FM patients.

Internet Addiction Management: A Comprehensive Review of Clinical Interventions and Modalities.

Internet addiction is a pervasive and complex issue that has gained increasing attention in the digital age. This comprehensive review provides an in-depth exploration of clinical interventions and modalities for managing internet addiction. It begins by examining the diagnostic criteria and assessment tools used to identify internet addiction, highlighting the diverse subtypes and varying degrees of severity. Subsequently, the review delves into various clinical interventions, including psychotherapeutic approaches like cognitive behavioral therapy (CBT), dialectical behavior therapy (DBT), and mindfulness-based interventions. Pharmacological interventions, technology-based tools, and integrative approaches are also thoroughly analyzed. The review also outlines various treatment settings and modalities such as inpatient treatment centers, outpatient clinics, telehealth, support groups, and prevention programs for schools and communities. Furthermore, it discusses the efficacy and challenges associated with managing internet addiction, emphasizing the need for effective interventions, relapse prevention, ethical considerations, and addressing stigma and access barriers. In conclusion, the review offers practical implications for clinical practice. It emphasizes future research's importance in refining diagnostic criteria, exploring emerging technologies, and adapting interventions to an ever-evolving digital landscape. This comprehensive review is a valuable resource for clinicians, researchers, and policymakers seeking to understand and address the complexities of internet addiction.

Age related disparities in sleep apnea diagnosis using a wearable device: Implications of 4% vs. 3% hypopnea scoring criteria.

STUDY OBJECTIVES: Obstructive sleep apnea (OSA) diagnosis relies on the Apnea-Hypopnea Index (AHI), with discrepancies arising from the 3% and 4% desaturation criteria. This study investigates age-related variations in OSA severity classification, utilizing data from 1201 adult patients undergoing Home Sleep Apnea Testing (HSAT) with SleepImage Ring@. METHODS: The study employs Bland-Altman analysis to compare AHI values obtained with the 3% and 4% desaturation criteria. Age-stratified analysis explores discrepancies across different age groups. RESULTS: The analysis reveals a systematic bias favoring the 3% criterion, impacting the quantification of apnea events. Age-specific patterns demonstrate diminishing agreement between criteria with increasing age. CONCLUSION: This comprehensive study underscores the importance of standardized criteria in OSA diagnosis. The findings emphasize age-specific considerations and ethical concerns, providing crucial insights for optimizing patient care and advancing sleep medicine practices.

Four-year follow-up of psychiatric and psychosomatic profile in patients with Inflammatory Bowel Disease (IBD).

Psychological characterization of patients affected by Inflammatory Bowel Disease (IBD) focuses on comorbidity with psychiatric disorders, somatization or alexithymia. Whereas IBD patients had higher risk of stable anxiety and depression for many years after the diagnosis of the disease, there is a lack of studies reporting a comprehensive psychosomatic assessment addressing factors of disease vulnerability, also in the long-term. The objective of this investigation is to fill this gap in the current literature. The aims were thus to assess: a) changes between baseline and a 4-year follow-up in psychiatric diagnoses (SCID), psychosomatic syndromes (DCPR), psychological well-being (PWB-I), lifestyle, gastrointestinal symptoms related to IBD and Irritable Bowel Syndrome (IBS)-like symptoms b) stability of psychiatric and psychosomatic syndromes at 4-year follow-up. A total of 111 IBD outpatients were enrolled; 59.5% of them participated at the follow-up. A comprehensive assessment, including both interviews and self-report questionnaires, was provided at baseline and follow-up. Results showed increased psychiatric diagnoses, physical activity, consumption of vegetables and IBS-like symptoms at follow-up. Additionally, whereas psychiatric diagnoses were no longer present and new psychopathological pictures ensued at follow-up, more than half of the sample maintained psychosomatic syndromes (particularly allostatic overload, type A behavior, demoralization) from baseline to follow-up. Long-term presence/persistence of such psychosocial burden indicates the need for integrating a comprehensive psychosomatic evaluation beyond traditional psychiatric nosography in IBD patients. Moreover, since psychosomatic syndromes represent vulnerability factors of diseases, further studies should target subgroups of patients presenting with persistent psychosomatic syndromes and worse course of the disease.

Applying the 2022 optic neuritis criteria to noninflammatory optic neuropathies with optic nerve T2-hyperintensity: an observational study.

INTRODUCTION: Recent diagnostic criteria for optic neuritis include T2-hyperintensity of the optic nerve (ON), even without associated contrast enhancement. However, isolated ON-T2-hyperintensity is a nonspecific finding found in any optic neuropathy or severe retinopathy. We applied the 2022 optic neuritis diagnostic criteria to a cohort of patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one eye, to assess the rate of optic neuritis misdiagnosis using these criteria. METHODS: Retrospective study of consecutive patients who underwent brain/orbit MRI with/without contrast between 07/01/2019 and 06/30/2022. Patients with ON-T2-hyperintensity in at least one eye were included. The 2022 optic neuritis diagnostic criteria were applied to patients with noninflammatory optic neuropathies who had an ophthalmologic examination available for review. RESULTS: Of 150 patients included, 85/150 had compressive optic neuropathy; 32/150 had glaucoma; 12/150 had papilledema; 8/150 had hereditary (3), radiation-induced (3), nutritional (1), traumatic (1) optic neuropathies (none fulfilled the criteria); 13/150 had ischemic optic neuropathy and 4 fulfilled the criteria as definite optic neuritis due to contrast enhancement of the ON head. Seven additional patients would have satisfied the diagnostic criteria if red flags for alternative diagnoses had been overlooked. DISCUSSION: The application of the 2022 optic neuritis diagnostic criteria in patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one ON resulted in misdiagnosis of optic neuritis in only 4 patients because of ON head enhancement, all with nonarteritic anterior ischemic optic neuropathy. Neuro-ophthalmologic evaluation and exclusion of the ON head as a location in the MRI criteria would have prevented optic neuritis misdiagnosis in our study.

Hemophagocytic lymphohistiocytosis in Egyptian children: diagnosis, treatment challenges, and outcome.

BACKGROUND: Hemophagocyticlymphohistiocytosis (HLH) is a spectrum of immune activation which could be genetically determined, or secondary to an underlying illness. Our aim was to present the clinico-genetic aspects of HLH among Egyptian children and to evaluate the patterns of reactivation and outcome with illustrations of overlap manifestations. RESEARCH DESIGNAND METHODS: We retrospectively collected the data of 55 patients with HLH, registered at Ain Shams University Children's Hospital,Cairo, Egypt. RESULTS: Median age at diagnosis was 19 months (range 2-180), 33 patients (60%) fulfilled the diagnostic HLH criteria at presentation. Fourteen (25.45%) patients had secondary HLH, 15 (27.27%) patients had genetically documented familial HLH (11 had variants in UNC13D gene and one in PRF1 gene), 3 had Griscelli and Chediak-Higashi syndromes. Sixteen patients (29.1%) had reactivations, 8 (50%) of them had molecularly confirmed HLH. We report the death of 40 patients, the median duration from the diagnosis to death of 5 months mostly due to disease activity. CONCLUSIONS: This study confirms that the nonspecific signs and symptoms of HLH are challenging. Genetic testing, though expensive and sophisticated, is integral for the diagnosis. The difficulty in finding non-related donors for stem cell transplantation and the early reactivations are the causes of the inferior outcome.

Recent Insights Into Native Valve Infective Endocarditis: JACC Focus Seminar 4/4.

This focused review highlights the latest issues in native valve infective endocarditis. Native valve disease moderately increases the risk of developing infective endocarditis. In 2023, new diagnostic criteria were published by the Duke-International Society of Cardiovascular Infectious Diseases group. New pathogens were designated as typical, and findings on computed tomography imaging were included as diagnostic criteria. It is now recognized that a multidisciplinary approach to care is vital, and the role of an "endocarditis team" is highlighted. Recent studies have suggested that a transition from intravenous to oral antibiotics in selected patients may be reasonable, and the role of long-acting antibiotics is discussed. It is also now clear that an aggressive surgical approach can be life-saving in some patients. Finally, results of several recent studies have suggested there is an association between dental and other invasive procedures and an increased risk of developing infective endocarditis. Moreover, data indicate that antibiotic prophylaxis may be effective in some scenarios.

Applicability of the International League Against Epilepsy (ILAE) 2022 diagnostic criteria for epilepsy syndromes in children: A retrospective review of 1550 children with epilepsy.

OBJECTIVE: Recently, the ILAE Nosology and Definitions Task Force defined diagnostic criteria for epilepsy syndromes. There is paucity of data on the use of these new diagnostic criteria in children with epilepsy, and how these criteria may lead to changes from previous practice. METHODS: This was a retrospective chart review of data of children attending the epilepsy clinic in a tertiary care children's hospital from January 2011 to January 2023. The clinical details such as age at onset, types of seizures, co-morbidities, and results of EEG, MRI and genetic testing were reviewed. Epilepsy syndrome diagnosis was made as per the ILAE 2022 criteria, and compared with the previous syndrome diagnosis as per records. RESULTS: Data from 1550 children (63 % boys) with epilepsy were analysed, and 55.4 % children were classified to have epilepsy syndromes as per the new ILAE 2022 diagnostic criteria. Application of the new 2022 ILAE diagnostic criteria was associated with a change in name alone in 676 (77.8 %) children. Hundred (11.5 %) children were newly classified under an epilepsy syndrome who had previously remained unclassified. Eleven (1.3 %) children who were previously classified into an epilepsy syndrome could not be classified using the new diagnostic criteria. Eight (0.9 %) were shifted to a new syndromic category. Overall, change in diagnosis occurred in 13.7 (11.5 + 1.3 + 0.9)%. No change in epilepsy syndrome classification/nomenclature occurred in 74 (8.5 %) children. SIGNIFICANCE: The new diagnostic criteria led to an overall change in diagnosis in 13.7 % of children with epilepsy. These criteria will hopefully lead to uniformity in diagnosis of epilepsy syndromes across diverse settings.

RECAM-J 2023-Validation and development of the Japanese version of RECAM for the diagnosis of drug-induced liver injury.

AIM: The diagnosis of drug-induced liver injury (DILI) is challenging. We modified the revised electronic version of the Roussel Uclaf Causality Assessment Method (RUCAM) for the diagnosis of DILI (RECAM), the scoring system developed in US and Spanish cohorts in 2022, and developed RECAM-J 2023 to align with the clinical practice in Japan. In the current study, we introduce RECAM-J 2023 and verify its performance in the context of Japanese patients with DILI. METHODS: After translation of RECAM into Japanese, modifications were made to develop RECAM-J 2023 without any alteration to the scores. To examine the validity and performance of RECAM-J 2023, clinical information on DILI and non-DILI cases in Japan were retrospectively collected. The diagnosis of DILI was made by expert's decision. Then we scored each case using RECAM-J 2023, and calculated area under curve (AUC) values for identification for DILI. RESULTS: We collected data from 538 DILI and 128 non-DILI cases. The sum of highly probable (HP) and probable (PR) cases categorized by RECAM-J 2023 were only 206 (38%) in DILI cases. As the primary cause of low scores was the deduction with missing hepatitis virus markers, which is unlikely to be an issue in prospective applications, we rescored without these deductions. At this time, the sum of HP and PR was raised to 421 (78%). The AUCs of RECAM-J 2023 without deductions were 0.70 and 0.88 for identifying at least HP, and at least PR, respectively. CONCLUSION: RECAM-J 2023, when prospectively used without any missing hepatitis virus markers, provides acceptable performance for identifying at least PR DILI cases in Japanese daily clinical practice.

Advances and challenges in the diagnosis and management of left ventricular noncompaction in adults: A literature review.

In the realm of cardiovascular health, isolated left ventricular noncompaction (LVNC) stands out for its distinct morphological features and the clinical challenges it presents, particularly in adults. This literature review explores the intricacies of LVNC, aiming to unravel its epidemiological spread, diagnostic hurdles, and therapeutic strategies. Despite technological advancements in cardiac imaging that have improved the recognition of LVNC, a significant gap persists alongside a fragmented understanding of its pathogenesis. The studies scrutinized reveal a broad spectrum of prevalence rates influenced by diverse diagnostic tools and demographic variables. This variation underscores the complexity of accurately identifying LVNC and the resultant implications for clinical management. The review succinctly addresses the need for precise guidelines to navigate the diagnosis of LVNC and outlines the imperative for tailored clinical management approaches that cater to the wide array of patient presentations, from asymptomatic cases to those with severe cardiac dysfunction. By highlighting the critical gaps in current literature-namely the absence of standardized diagnostic criteria and a comprehensive pathogenic model-the review sets the stage for future research directions. These endeavors are essential for enhancing diagnostic accuracy, refining management protocols, and ultimately improving patient outcomes in this complex subset of cardiomyopathy, thus contributing significantly to the advancement of cardiovascular medicine.

Clinical characteristics of hemophagocytic lymphohistiocytosis.

BACKGROUND: There is no specificity in the clinical presentation of hemophagocytic lymphohistiocytosis (HLH). OBJECTIVE: To study some clinical, etiological, and prognostic features of HLH to improve the clinical understanding of the disease. METHODS: Retrospective analysis of the clinical data of 125 patients with HLH admitted to our hospital from June 2015 to August 2021, including clinical characteristics, laboratory indicators, and survival period. Statistical analysis was performed from the overall group of study indicators, which included population, children, and adults. RESULTS: In the whole population, sex, age, blood myoglobin, and NK cell ratio of M-HLH and non-M-HLH patients (P< 0.05), serum albumin, and direct bilirubin were independent correlates of M-HLH. In the pediatric group, age and the proportion of NK cells were significantly different between M-HLH and non-M-HLH patients (P< 0.05). Multivariate Logistic regression analysis showed that all factors were not significantly associated with M-HLH. The associated regression analysis showed that all factors were not significantly associated with M-HLH. ROC curve analysis showed that the best predictive value of NK cell percentage for M-HLH diagnosis in the overall population was 4.96% in the pediatric group and 4.96% in the adult group. The best predictive value for M-HLH diagnosis was 2.08%. The univariate analysis showed that platelet count, alanine aminotransferase, aspartate aminotransferase, serum albumin, direct bilirubin and indirect bilirubin affected prognosis; COX regression showed that none of these factors had a significant relationship. The overall median survival time was 20.7 months in the adult group, 44.3 months in non-M-HLH patients, and 7.73 months in M-HLH patients (p= 0.011); univariate analysis showed that platelet count and serum albumin level affected prognosis; COX regression results in serum albumin level was an independent risk factor for prognosis. CONCLUSION: The survival rate of non-M-HLH was significantly better than that of M-HLH; the proportion of NK cells had predictive value for the diagnosis of M-HLH; in the general population, non-M-HLH was more likely to have abnormal liver function than M-HLH: lower platelet count and serum albumin level were associated with poor prognosis, and the lower the platelet count and serum albumin level, the worse the prognosis: in addition, adults with lower serum albumin levels are also associated with poor prognosis.

A novel staging system to consolidate silent sinus syndrome and chronic maxillary atelectasis: A systematic review and case series.

KEY POINTS: Silent sinus syndrome (SSS) and chronic maxillary atelectasis (CMA) represent an overlapping clinical entity, both likely lying on the spectrum of one disease process. There is widespread inconsistency of diagnosis in the literature of reported cases of SSS and CMA. We propose a novel, comprehensive staging system to simplify diagnosis and inform management.

[The association between myopia and glaucoma]. / Vzaimosvyaz' miopii i glaukomy.

Myopia and glaucoma are the leading causes of irreversible blindness in the world, and as the number of nearsighted people is predicted to increase sharply in the upcoming decades, an increase in combined pathology is expected. This article analyzes literature sources to assess the epidemiology of glaucoma and myopia both worldwide and in the Russian Federation, examine the anatomical changes of the eyeball associated with these diseases, and identify the diagnostic criteria that facilitate the verification of glaucoma when combined with myopia.

Etiologic Factors of Temporomandibular Disorders: A Systematic Review of Literature Containing Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) and Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD) from 2018 to 2022.

This study aims to conduct a systematic analysis of literature published between 1 January 2018 and 1 September 2022, exploring factors influencing the progression or development of temporomandibular disorders (TMD), diagnosed using the Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) or Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD). Three electronic databases were reviewed to identify papers that examined TMD factors using DC/TMD or RDC/TMD. Inclusion criteria encompassed original research published in English between 1 January 2018 and 1 October 2022, online, and complete DC/TMD or RDC/TMD studies on human participants aged 18 or older. Two authors independently assessed the risk of bias using The Joanna Briggs Institute (JBI) Analytical cross-sectional studies' Critical Appraisal Tool. Of 1478 articles, 11 were included. The studies revealed strong associations between TMD and factors such as female, poor sleep quality, depression, oral parafunction, anxiety, somatization, and anatomical features. However, variables such as education, living conditions, socioeconomic status, marital status, chronic pain, and stress did not exhibit statistically significant correlations. Based on the obtained data, it can be concluded that the causes of TMD are largely related to psychological factors, which supports the biopsychosocial theory of the disorder.

272nd ENMC international workshop: 10 Years of progress - revision of the ENMC 2013 diagnostic criteria for inclusion body myositis and clinical trial readiness. 16-18 June 2023, Hoofddorp, The Netherlands.

Since the publication of the 2013 European Neuromuscular Center (ENMC) diagnostic criteria for Inclusion Body Myositis (IBM), several advances have been made regarding IBM epidemiology, pathogenesis, diagnostic tools, and clinical trial readiness. Novel diagnostic tools include muscle imaging techniques such as MRI and ultrasound, and serological testing for cytosolic 5'-nucleotidase-1A antibodies. The 272nd ENMC workshop aimed to develop new diagnostic criteria, discuss clinical outcome measures and clinical trial readiness. The workshop started with patient representatives highlighting several understudied symptoms and the urge for a timely diagnosis. This was followed by presentations from IBM experts highlighting the new developments in the field. This report is composed of two parts, the first part providing new diagnostic criteria on which consensus was achieved. The second part focuses on the use of outcome measures in clinical practice and clinical trials, highlighting current limitations and outlining the goals for future studies.