The Florida Scoring System for stratifying children with suspected Sjögren's disease: a cross-sectional machine learning study.

BACKGROUND: Childhood Sjögren's disease is a rare, underdiagnosed, and poorly-understood condition. By integrating machine learning models on a paediatric cohort in the USA, we aimed to develop a novel system (the Florida Scoring System) for stratifying symptomatic paediatric patients with suspected Sjögren's disease. METHODS: This cross-sectional study was done in symptomatic patients who visited the Department of Pediatric Rheumatology at the University of Florida, FL, USA. Eligible patients were younger than 18 years or had symptom onset before 18 years of age. Patients with confirmed diagnosis of another autoimmune condition or infection with a clear aetiological microorganism were excluded. Eligible patients underwent comprehensive examinations to rule out or diagnose childhood Sjögren's disease. We used latent class analysis with clinical and laboratory variables to detect heterogeneous patient classes. Machine learning models, including random forest, gradient-boosted decision tree, partial least square discriminatory analysis, least absolute shrinkage and selection operator-penalised ordinal regression, artificial neural network, and super learner were used to predict patient classes and rank the importance of variables. Causal graph learning selected key features to build the final Florida Scoring System. The predictors for all models were the clinical and laboratory variables and the outcome was the definition of patient classes. FINDINGS: Between Jan 16, 2018, and April 28, 2022, we screened 448 patients for inclusion. After excluding 205 patients due to symptom onset later than 18 years of age, we recruited 243 patients into our cohort. 26 patients were excluded because of confirmed diagnosis of a disorder other than Sjögren's disease, and 217 patients were included in the final analysis. Median age at diagnosis was 15 years (IQR 11-17). 155 (72%) of 216 patients were female and 61 (28%) were male, 167 (79%) of 212 were White, and 20 (9%) of 213 were Hispanic, Latino, or Spanish. The latent class analysis identified three distinct patient classes: class I (dryness dominant with positive tests, n=27), class II (high symptoms with negative tests, n=98), and class III (low symptoms with negative tests, n=92). Machine learning models accurately predicted patient class and ranked variable importance consistently. The causal graphical model discovered key features for constructing the Florida Scoring System. INTERPRETATION: The Florida Scoring System is a paediatrician-friendly tool that can be used to assist classification and long-term monitoring of suspected childhood Sjögren's disease. The resulting stratification has important implications for clinical management, trial design, and pathobiological research. We found a highly symptomatic patient group with negative serology and diagnostic profiles, which warrants clinical attention. We further revealed that salivary gland ultrasonography can be a non-invasive alternative to minor salivary gland biopsy in children. The Florida Scoring System requires validation in larger prospective paediatric cohorts. FUNDING: National Institute of Dental and Craniofacial Research, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Heart, Lung, and Blood Institute, and Sjögren's Foundation.

A tribute to Wartenberg's refined neurological examination / Um tributo ao refinado exame neurológico de Wartenberg

ABSTRACT Robert Wartenberg was a renowned 20th century neurologist who contributed greatly to our understanding of the neurological examination. This article aims to illustrate his legacy by highlighting five seminal neurological signs.

RESUMO Robert Wartenberg foi um renomado neurologista do século XX que contribuíu enormemente para o entendimento do exame neurológico. Este artigo tem como objetivo principal ilustrar seu legado, ressaltando cinco sinais neurológicos seminais.

Magnetic resonance spectroscopy in childhood brainstem tumors.

Five children with brainstem tumors and two control patients had magnetic resonance spectroscopy studies of the brainstem. Two of the malignant tumor patients had magnetic resonance spectroscopy studies before and after radiation therapy. The third was irradiated 14 years earlier but developed new symptoms and a new brainstem lesion on MRI. Magnetic resonance spectroscopy demonstrated a different degree of malignancy between the old and new lesion. The fourth patient had magnetic resonance spectroscopy of a chronic, large pontine lesion 6 years after diagnosis and radiation. The spectral pattern suggested a low degree of malignancy. The fifth patient had neurofibromatosis type 1 with brainstem lesions. Magnetic resonance spectroscopy suggested neoplastic tissue of low malignancy. These results suggest that magnetic resonance spectroscopy offers additional information for anticipating the degree of anaplasia in children with brainstem tumors.

Afecçöes neurológicas ocupacionais dos músicos: lª parte / Occupational neurological disorders in musicians: part I

Os músicos podem ser acometidos por afecçöes neurológicas de natureza ocupacional. O temor de palco é um distúrbio decorrente da descarga adrenérgica excessiva relacionada a apresentaçöes difícies e/ou importantes. O uso excessivo dos músculos ao tocar por períodos prolongados pode resultar em uma lesäo ultra-estrutural de resoluçäo demorada. A compressäo de nervos periféricos pode resultar do contato direto com os instrumentos musicais ou da posiçäo necessária para tocá-los. Mais raramente, os músicos säo acometidos por uma discinesia ocupacional, de origem obscura e tratamento difícil

Siringomielia: revisäo da literatura e relato de caso / Syringomyelia: review of the literature and report of a case

Os autores fazem detalhada revisäo da literatura acerca de aspectos históricos, etiopatogênicos, clínicos, diagnósticos e terapêuticos da siringomiella. Apresentam um caso desta enfermidade, cujo diagnóstico foi confirmado por ressonância magnética