Muscle & Nerve最新文献

Introduction/aims: There is limited data on the sensitivity and responsiveness of high-resolution imaging techniques in the longitudinal assessment of hereditary neuropathies. In this study, our aims were to investigate the ability of ultra-high field magnetic resonance imaging to detect longitudinal changes in the peripheral nerves of Charcot-Marie-Tooth (CMT) 1A patients, and to evaluate the potential benefits of doing so at the nerve fascicle level.

Methods: We performed magnetic resonance imaging (MRI) to simultaneously obtain high-resolution anatomical and quantitative data at ultra-high 7 Tesla field strength in peripheral nerves of four patients with CMT1A disease at baseline and follow up. We compared the resulting measurements of T2 in sciatic, tibial, and fibular nerves within individual fascicles of the three nerve regions.

Results: Analyzing individual fascicle distributions, we demonstrated a significantly elevated T2 in the fibular nerve over the course of the study, with a mean increase of 3.55 ms (p = 0.01). Changes in the sciatic nerve were marginally significant (mean increase 1.42 ms, p = 0.05), and tibial nerve changes were not significant (mean increase 1.31 ms, p = 0.18). Combining fascicles across subjects showed significant changes in all three nerves over time.

Discussion: Our results indicate that longitudinal MRI assessment of individual nerve fascicles may serve as a quantitative biomarker of disease progression in patients with hereditary neuropathies. Further, our study demonstrates that the data provided by fascicle-level analysis may provide better analytical abilities than whole-nerve imaging.

Introduction/aims: .: Primary Sjögren syndrome (SS) is a chronic autoimmune disease characterized frequently by sensory neuropathy (SN). Carbonic Anhydrase-6 (CA-6), Parotid Secretory Protein (PSP), and Salivary Protein-1 (SP-1) antibodies (Early Sjögren antibodies [ESA]) are found in 45% of SS patients who lack traditional antibodies (Ro/La). But there is a lack of information regarding whether ESA are associated with SN, and if there are any identifying characteristics associated with ESA in SN. Our goal was to close this knowledge gap.

Methods: All SN patients tested for ESA from May 2023-October 2024 were retrospectively analyzed for clinical features such as sicca symptoms, onset acuity, small fiber neuropathy (SFN)-questionnaire scores, and pathological features such as length-dependence/vasculitis on skin biopsies, as well as lip biopsy confirmation of SS. Seropositive/seronegative groups were separated for statistical analysis.

Results: Thirty-three adult patients (73% female) with cryptogenic SN had ESA testing. Eighteen (55%) had abnormal ESA. Twelve (100%) seropositive patients had significant sicca symptoms versus four seronegative patients (29%) (p = 0.0002). There were no significant associations between ESA seropositivity and SFN-questionnaires, pathological nonlength dependence (NLD)/vasculitis, or onset acuity.

Discussion: ESA may be seen in more than half of cryptogenic SN patients, but whether these patients have confirmed SS is unclear. Seropositive patients more frequently have significant sicca symptoms than seronegative patients, but no other significant identifying characteristics were seen. Further work on a larger population should be done to confirm these findings, but this study suggests the utility of checking ESA in cryptogenic SN patients with significant sicca symptoms.

Introduction/aims: Neuromuscular ultrasound (NMUS) is becoming increasingly incorporated as a complementary tool in electrodiagnostic (EDX) medicine. Competency assessment is critical to ensure adequate quality, appropriate application, and accurate interpretation. The primary objective was to develop a competency-based assessment tool of foundational NMUS skills among postgraduate medical trainees in a single center.

Methods: The assessment included written (28 multiple-choice, short answer, or yes/no) questions and practical skills (15 prompts) sections recommended by a published consensus-based survey to include the knowledge and skills necessary to achieve minimal competency for unsupervised practice of NMUS for common conditions. The written portion included interpretation of basic pathology. Core practical content focused on the most evaluated structures in the Mayo EDX laboratory: the median and ulnar nerves and diaphragm muscle. The practical portion was administered by a single examiner and scored with a five-point competency-based rating. Four experienced educators used the Angoff standard setting method to set passing scores for each section.

Results: Subjects included three EMG-track clinical neurophysiology fellows who completed a NMUS lecture series with supervised hands-on scanning and a 2-week intra-lab NMUS elective during fellowship. Composite scores ranged from 89% to 97%. All subjects met minimum passing scores for each section (77% and 88% for the knowledge and skills sections, respectively).

Discussion: This NMUS competency assessment was successfully implemented and may be incorporated into postgraduate medical trainee evaluation to ensure minimal competency in evaluation of carpal tunnel syndrome, ulnar neuropathy at the elbow, and diaphragm function. This effort integrates quality NMUS evaluations into clinical practice.

Introduction/aims: Comorbidities are frequent in myasthenia gravis (MG) and may affect treatment choices. Conversely, MG treatments may impact the risk of comorbidity. Our objective was to examine comorbidity and MG treatment patterns in nationwide MG cohorts in Denmark, Finland, and Sweden.

Methods: We included individuals with ≥ 2 MG diagnoses (ICD codes) in nationwide health registries between 2000 and 2020 and analyzed comorbidities before and after MG diagnosis and MG-related treatments during follow-up.

Results: Among 8819 people with MG (pwMG), 3159 were incident cases with data available ±5 years from diagnosis. Circulatory diseases were the most frequent comorbidity (19%-29%) before diagnosis, mostly explained by hypertension (13%-24%). After diagnosis, anemia and osteoporosis prevalence increased three to six fold. Mental health disorders were more frequent in younger (0-64 years) than older (≥ 65 years) pwMG. In the first year after diagnosis, acetylcholinesterase inhibitors (AChEIs) were the most used treatment in Finland (41%) and Sweden (34%), and corticosteroids (CSs) with nonsteroidal-immunosuppressive therapy (NS-IST) in Denmark (33%). By year 5, the proportion of pwMG receiving NS-IST, CS, or their combination was similar in the three countries (Denmark 47%, Finland 40%, and Sweden 41%). Only 5%-7% remained treatment-naive throughout follow-up.

Discussion: MG treatment was broadly similar across the three Nordic countries, while also reflecting nation-specific therapeutic guidelines. Many pwMG required multiple therapies, underscoring risks of long-term immunosuppression and highlighting the need for vigilant management and future research of safer strategies.

Introduction: Oral hygiene represents a modifiable risk factor for systemic health and pulmonary complications yet is not routinely addressed in ALS care. This study aimed to examine the relationships between oral health, disease severity and determinants of health in people living with amyotrophic lateral sclerosis (pALS), and to identify key predictors of oral hygiene outcomes.

Methods: Individuals with ALS completed an oral hygiene and bulbar screening during their multidisciplinary appointment. Disease demographics, determinants of health, oral health outcomes and bulbar disease outcomes were collected. Descriptives and one sample t-tests were performed to compare oral hygiene outcomes with healthy reference values. Multiple regression analyses were conducted to assess the relationship between disease demographics and oral health.

Results: Sixty-two pALS aged 64.0 (+/- 10.8), 40% female, 31% Hispanic/Latino and 37% bulbar onset disease were enrolled. Compared to healthy reference values, plaque index (M = 1.45, SD = 0.52, p < 0.0001), gingival index (M = 1.25, SD = 0.46, p < 0.0001) and bleeding on probing (M = 35.26%, SD = 26.1, p < 0.0001) were elevated in pALS. Lack of dental insurance was a significant predictor of bleeding on probing (BOP) (p = 0.001), plaque (p = 0.006) and gingival scores (p = 0.001). ALSFRS-R (p < 0.03) was also predictive of greater plaque, and care partner status (p < 0.04), and age (p < 0.02) were predictors BOP. Ethnicity and dysphagia severity were not significant predictors.

Discussion: Oral health screenings conducted during routine multidisciplinary visits identified periodontal disease in pALS, representing a feasible and immediately actionable pathway to improve oral care outcomes in pALS.