Citations

ABSTRACT Background Primary lateral sclerosis (PLS) is an ultrarare upper motor neuron syndrome with a prognosis unique from classical ALS. The study of PLS is complicated by its rarity and the difficulty distinguishing PLS from ALS. We present data from a 1‐year prospective follow‐up study on PLS and efforts to distinguish it from ALS. Methods Seventy‐six PLS participants enrolled in this prospective natural history study. EMG studies, blood neurofilament light chain levels (NfLs), and demographic characteristics were obtained at baseline. At 1‐year follow‐up, repeat EMG studies were conducted to determine which participants fulfilled criteria for ALS. Baseline characteristics were then compared to determine features that predict reclassification. Results Seventy participants completed 1‐year follow‐up. Five of the 70 were reclassified to ALS (7.1%). Those reclassified had higher trends in baseline blood NfL levels (91.4 vs. 34.0 pg/mL, p = 0.13) and shorter symptom duration (39 vs. 69 months in the PLS group, p = 0.15). Reclassification was noted in both probable and definite PLS participants. All cases with a symptomatic duration of less than 2 years retained the PLS phenotype (5 of 5). NfL levels over 90 pg/mL predicted reclassification with 94% specificity and 60% sensitivity. No other features predicted reclassification to ALS. Conclusions In our population, reclassification of PLS to ALS occurred at a low frequency at 1 year follow‐up (7.1%). Baseline NfL was the strongest predictor in differentiating UMN dominant ALS from PLS at 1‐year follow‐up. Based on our data, we propose EMG and NfL criteria for enrollment in future PLS trials.

Abstract Introduction: While evaluation approaches for community-academic research groups are established, few tools exist for academic institutional advisory groups across multi-core centers and research, education, and clinical care missions. Institutional advisory group evaluation should consider group processes and their impact on community-centered outcomes. This study describes the community-engaged development of a mixed-method evaluation approach to address this gap and presents pilot outcomes across an NIH-funded center. Methods: We utilized a Community of Practice model to co-develop a survey with 14 community and academic representatives of four advisory groups. The final survey included five categories of group process and four categories of outcomes. Storytelling sessions with community partners explored areas where the survey identified discrepancies in perspectives between community and academic team members, as well as areas with lower scores. Results: Nine community and 14 academic (staff and faculty) partners completed the survey. Respondents positively assessed group process outcomes (shared values, leadership, community-centeredness, and decision-making), and slightly less positive assessments of institutional outcomes. Storytelling sessions confirmed the overall satisfaction of community partners but highlighted actionable concerns within power-sharing, decision-making, funding equity, and trust-building. Conclusions: The results of this equity-centered evaluation suggest the utility and importance of participatory, mixed-methods approaches to evaluating community-academic institutional advisory groups.

Objective The primary lateral sclerosis ( PLS ) consensus diagnostic criteria and functional rating scale ( PLSFRS ) were recently established to facilitate and optimize future PLS clinical trials. We examined the trajectory of the PLSFRS and other functional outcome measures and biomarkers in the PLS Natural History Study ( PLS NHS ) to understand their performance in this prospective cohort. Methods The PLS NHS is a prospective, longitudinal, multicenter study of people living with PLS in different diagnostic categories: early (disease duration <2 years); probable (2–4 years); and definite PLS (4–15 years). PLSFRS scores and other functional outcome measures were collected at baseline, 3‐, 6‐, 9‐, and 12‐month follow‐up visits. Baseline characteristics were compared between the groups. The slopes of the PLSFRS and other functional outcome measures over 12 months were examined in the overall cohort and subgroups using linear mixed‐effect models. The associations between baseline characteristics and the rate of PLSFRS decline were analyzed with linear regression models. Results A total of 76 participants were included: early (n = 6); probable (n = 26); and definite (n = 44) PLS . Baseline PLSFRS total scores were highest in the early PLS group, followed by the probable and definite PLS groups. In the overall cohort, the PLSFRS total score declined by 0.33 points/month (95% confidence interval [0.27–0.39], adjusted p < 0.05). The rate of decline was steepest in the early PLS group, followed by the probable and definite PLS groups. Baseline neurofilament light chain level was associated with the rate of PLSFRS decline over 1 year ( p = 0.001). Interpretation In PLS, the rate of functional decline, as measured by the PLSFRS total score, is faster during the early phase of the disease. Neurofilament light might serve as a prognostic biomarker in PLS. ANN NEUROL 2026;99:418–428

The gracilis free functioning muscle transfer is an established procedure for reconstructing elbow, wrist, and hand function in adult traumatic brachial plexus injury patients. Elbow flexion or distal wrist or hand function can be compromised by gracilis bowstringing in up to 17.8% of patients. Two techniques for pulley reconstruction to correct bowstringing and improve the function of the gracilis transfer are described.

Abstract Symptomatic scapholunate tears are a common traumatic problem in the wrist. This article demonstrates a technique for reducing and stabilizing complete scapholunate tears. The purpose is to provide a detailed description of the technique, highlight potential advantages, and discuss preliminary clinical results. Technique Video video See video under supplementary data.

ObjectivesUpdated retrospective review of the sonographic appearance of palmar fibromatosis (PF) with evaluation of the utility of the Comb Sign previously described in plantar fibromas. Additional evaluation was conducted on the location relative to the flexor tendon, anatomic proximity of palmar fibromas to the A1 pulley and evaluate any potential association with trigger finger.MethodsMedical record and imaging review was performed from 2017 to 2023, for patients with a new onset ultrasound or clinical diagnosis of PF. Clinical associations and imaging morphology were reviewed including presence of the Comb Sign, fibroma association with the A1 pulley, and fibroma association with trigger finger.ResultsExactly 87 total fibromas in 53 patients were evaluated. The Comb Sign was present in 39% of fibromas, usually seen in transverse plane, more prevalent in multifocal disease and larger fibromas. Most (72%) palmar fibromas were within 1 cm of, contacted, or covered the A1 pulley (P < .001). Lateral extension beyond the flexor tendon axis can be seen (44%). Trigger finger and tenosynovitis were rare. However, volume and SI dimension of fibromas were associated with tenosynovitis (P < .0001) and all nine patients with concomitant trigger finger had fibromas within 1 cm from the A1 pulley.ConclusionsThe Comb Sign can aid in sonographic diagnosis of PF. Lateral extension of fibromas can occur. Most palmar fibromas have a significant intimate association with the A1 pulley, and presence of trigger finger with adjacent palmar fibroma can exist and is important for hand surgeons to know preoperatively.

Fifty cadaver digits were used to study the mechanical properties of five different methods of pulley reconstruction and the effectiveness of three different pulley reconstructive arrangements in restoring a normal relationship between tendon excursion and joint motion. All reconstructed pulleys showed less stiffness and more displacement before failure than normal intact pulleys. Although reconstruction using a length of another tendon woven through the remaining fibrous rim of the pulley and both double and triple tendon graft loops around bone absorbed more energy to failure than a normal pulley, only the triple loop around bone could withstand as much load before failure as a normal pulley. No reconstruction restored a normal tendon excursion/joint motion relationship, but reconstruction of the A2 and A4 pulleys restored a more normal relationship than either the “belt loop” reconstruction or a combination belt loop, A2 and A4 reconstruction.

Injury to the A2 pulley is caused by high eccentric forces on the flexor‐tendon–pulley system. Accurate diagnosis is necessary to identify the most appropriate treatment options. This review summarizes the literature with respect to using ultrasound (US) to diagnose A2 pulley injuries, compares ultrasound to magnetic resonance imaging and computed tomography, and identifies current knowledge gaps. The results suggest that US should be used as the primary imaging modality given high accuracy, relatively low cost, ease of access, and dynamic imaging capabilities. Manual resistance is beneficial to accentuate bowstringing, but further research is needed to determine best positioning for evaluation.