Citations 82

Peter James Dyck: In Memoriam

Journal of the Peripheral Nervous System | November 20, 2025 | Volume: 30 | Issue: 4 | Pages: No page available

10.1111/jns.70073

Nexiguran Ziclumeran Gene Editing in Hereditary ATTR with Polyneuropathy

New England Journal of Medicine | September 25, 2025 | Volume: 393 | Issue: 14 | Pages: 1375-1386

10.1056/nejmoa2510209 | PMID 41002250

Minimal changes in excitation-contraction coupling in spastic human muscle

Journal of Neurophysiology | September 6, 2025 | Volume: 134 | Issue: 4 | Pages: 1069-1074

10.1152/jn.00274.2025 | PMID 40912898

This study presents the first in vivo intraoperative measurement of the kinetic properties of spastic human muscle. Despite slower relaxation in spastic biceps, the force-frequency relationship was similar to that of the healthy gracilis muscle. This suggests that spasticity does not substantially alter frequency-dependent force summation, possibly due to similar fiber-type compositions and limited changes in calcium handling or myosin isoforms in human spastic muscle.
In vivo human gracilis muscle active force‐length relationship is explained by the sliding filament theory

The Journal of Physiology | May 11, 2025 | Volume: 603 | Issue: 10 | Pages: 3049-3059

10.1113/jp288322 | PMID 40349312

AbstractThe sliding filament theory explains skeletal muscle fibre force change as a function of length based on the overlap of actin and myosin filaments. Although this length‐tension (LT) relationship has been well investigated in animal models, it is not known whether this microscopic sarcomere LT property can be scaled up five orders of magnitude to explain the LT behaviour of a long human muscle such as the gracilis. The goal of this study is to validate the sarcomere LT curve in humans based on human filament length combined with in vivo experimental data. Intraoperative measurements of maximal tetanic force and muscle‐tendon unit length at four different joint configurations (JC) were obtained from 19 patients undergoing free functioning muscle transfer surgery. With physiologically measured fibre length and estimated sarcomere shortening resulting from tendon compliance, we show that 79.7% variance in isometric force data is explained by a simple human sarcomere LT model. This study demonstrates that the human whole muscle LT relationship can be modelled by the sliding filament theory given patient‐specific fibre length, filament length, tendon compliance and sarcomere shortening. imageKey points Whole human gracilis muscle isometric length‐tension relationships were measured in the operating room. Grouped whole muscle raw length‐tension curves showed no obvious form. The width of each experimental length‐tension curve was highly variable across subjects and used to predict fibre length (serial sarcomere number). After whole muscle length‐tension curves were normalized to each patient's serial sarcomere number, the whole human muscle length‐tension curve was well predicted by the sliding filament theory.
Neuromuscular Ultrasound Assessment of the Median Nerve at the Wrist and Forearm: A Reproducibility and Reliability Study in Nonphysician Sonographers

Muscle & Nerve | April 28, 2025 | Volume: 72 | Issue: 1 | Pages: 130-133

10.1002/mus.28424 | PMID 40289532

ABSTRACTIntroduction/AimsNeuromuscular ultrasound (NMUS), initially performed by physicians, is increasingly performed by technicians in many electrodiagnostic (EDX) laboratories for certain studies under physician supervision. The aims of this study were to determine: (1) the reproducibility of nonphysician sonographers' assessments of the median nerve and (2) the need for ongoing training to maintain the reproducibility and reliability of median nerve ultrasound assessments.MethodsThe median nerve was examined sonographically in a healthy volunteer by 12 nonphysicians on 3 separate occasions: Day 1 and then 2 weeks (Day 2) and 4 months (Day 3) later. Cross‐sectional area (CSA) and wrist‐to‐forearm ratios (WFRs) were calculated each time. Sonographers were retrained prior to Day 2. Reproducibility and reliability were determined using coefficients of variation calculated for each characteristic for each day and each nonphysician sonographer, comparing performance across the 3 time points.ResultsThere was overall less variation for each characteristic (wrist CSA, forearm CSA, and WFR) on Day 2 compared to Day 1 (coefficients of variation 3.0%, 5.4%, and 5.7% vs. 8.8%, 15.5%, and 11.3%, respectively), both when viewed at the group and individual levels, indicating an improvement in reproducibility and reliability after the retraining session. The improvement was similar for Day 3.DiscussionThis study demonstrates the need for training and subsequent retraining sessions to ensure the reproducibility and reliability of NMUS measurements in the median nerve.
Clinical Features and Outcomes of Autoimmune Neuromuscular Hyperexcitability Spectrum Disorders (P4-8.012)

Neurology | April 7, 2025 | Volume: 104 | Issue: 7_Supplement_1 | Pages: No page available

10.1212/wnl.0000000000212155

High‐Throughput Immunoassays for Cavin‐4 IgG: A Diagnostic Tool for Immune‐Mediated Rippling Muscle Disease

Annals of Clinical and Translational Neurology | February 17, 2025 | Volume: 12 | Issue: 4 | Pages: 876-880

10.1002/acn3.70012 | PMID 39957412

ABSTRACTCavin‐4 was identified as a potential autoantigen for immune‐mediated rippling muscle disease (iRMD). To validate this, we developed and tested various immunoassays, including a cell‐based assay (CBA), cavin‐4 recombinant protein ELISA, and multi‐peptide ELISA. Among 19 iRMD patients, all exhibited muscle rippling, and 13 had percussion‐induced mounding. All immunoassays demonstrated clinical and analytical specificities greater than 95%. The protein ELISA had the highest sensitivity (94.7%) and specificity (99.9%), outperforming CBA (sensitivity 89.5%, specificity 99.6%) and the multi‐peptide ELISA (sensitivity 79.0%, specificity 97.2%). Our results suggest that the cavin‐4 protein ELISA is a promising tool for high‐throughput clinical testing in iRMD.
Compound Muscle Action Potential and Myosin-Loss Pathology in Patients With Critical Illness Myopathy

Neurology | June 13, 2024 | Volume: 103 | Issue: 1 | Pages: No page available

10.1212/wnl.0000000000209496 | PMID 38870464

Identification of Calcium Dysregulation in Immune-mediated Rippling Muscle Disease (P8-11.001)

Neurology | April 9, 2024 | Volume: 102 | Issue: 7_supplement_1 | Pages: No page available

10.1212/wnl.0000000000206010

Compound Muscle Action Potential (CMAP) Duration and Myosin Loss in Patients with Critical Illness Myopathy: Correlation and Prognostication (P1-11.005)

Neurology | April 9, 2024 | Volume: 102 | Issue: 7_supplement_1 | Pages: No page available

10.1212/wnl.0000000000205833