Muscle & NervePub Date : 2025-04-01Epub Date: 2025-02-12DOI: 10.1002/mus.28367
Christopher G Herbosa, Ronald Perez, Alexandra Jaeger, Christopher J Dy, David M Brogan
{"title":"Inhibition of SARM1 Reduces Neuropathic Pain in a Spared Nerve Injury Rodent Model.","authors":"Christopher G Herbosa, Ronald Perez, Alexandra Jaeger, Christopher J Dy, David M Brogan","doi":"10.1002/mus.28367","DOIUrl":"10.1002/mus.28367","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The function of the sterile alpha and toll/interleukin receptor motif-containing protein 1 (SARM1) in neuropathic pain development has not yet been established. This protein has a central role in regulating axon degeneration and its depletion delays this process. This study aims to demonstrate the effects of SARM1 deletion on the development of neuropathic pain.</p><p><strong>Methods: </strong>Thirty-two wild-type (WT) or SARM1 knockout (KO) rats underwent spared nerve injury (SNI) or sham surgery. Mechanical allodynia was assessed by electronic Von Frey and cold hyperalgesia by the acetone test. Nociception was evaluated at the baseline, Day-1, Day-2, Week-1, Week-2, Week-3, and Week-4 time points. Nerve sections were examined by immunohistochemistry (IHC).</p><p><strong>Results: </strong>WT Injury rats were more sensitive to pain than WT Sham at all postoperative time points, validating the pain model. Injured SARM1 KO rats only demonstrated a difference in mechanical or cold nociception from KO Sham at Week 3. Injured KO rats demonstrated a clear trend of decreased sensitivity compared to WT Injury nociception, reaching significance at Week 4 (p = 0.044). Injured KO rats showed attenuated sensitivity to cold allodynia relative to WT at Week 2 (p = 0.019). IHC revealed decreased macrophages in spared sural nerves of injured KO animals at 2 and 4 weeks, and the proximal portion of tibial/peroneal nerves at Week 2.</p><p><strong>Discussion: </strong>This study demonstrates that SARM1 KO rats are less sensitive to mechanical and cold nociception than WT rats in an SNI model with decreased inflammatory response. Given these results, inhibition of SARM1 should be further investigated in the treatment of neuropathic pain.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"670-679"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-27DOI: 10.1002/mus.28403
Trond Sand, Martin Uglem, Gjermund Johnsen, Arnstein Grøtting, Øystein Dunker, Jorunn Sandvik, Kristian B Nilsen
{"title":"Sensory Nerve Conduction Amplitudes and Z-Compounds Depend on Body Mass Index in Overweight and Obese Individuals.","authors":"Trond Sand, Martin Uglem, Gjermund Johnsen, Arnstein Grøtting, Øystein Dunker, Jorunn Sandvik, Kristian B Nilsen","doi":"10.1002/mus.28403","DOIUrl":"https://doi.org/10.1002/mus.28403","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The association between body mass index (BMI) and nerve conduction study (NCS) amplitudes has not been adequately studied in overweight and obese subjects. Our aim was to estimate the impact of high BMI on NCS amplitudes and NCS summary measures.</p><p><strong>Methods: </strong>One hundred and seventy-five patients with previous gastric bypass surgery and 86 subjects from the general population had clinical evaluations and NCS of upper and lower limbs. Subjects with diabetes, mononeuropathy, or polyneuropathy (PNP) were excluded. Eighty-three former patients with a mean age of 48 years (SD 8), BMI mean = 34.3 kg/m<sup>2</sup> (range 22-60) and 62 control subjects from the general population with a mean age of 55 years (SD 11), BMI mean = 26.6 kg/m<sup>2</sup> (range 19-40), were analyzed. Several Z-compounds were calculated from NCS parameters. Multiple linear regression models compared the impact of BMI on NCS amplitude measurements, corrected for height and age, and NCS-based Z-compounds.</p><p><strong>Results: </strong>Most sensory amplitudes and tibial nerve motor amplitude decreased significantly with increasing BMI. The magnitudes of association were comparable for BMI and age. A new Z-compound for sensory amplitudes correlated moderately strongly with BMI for subjects with BMI < 30 kg/m<sup>2</sup> and BMI ≥ 30 kg/m<sup>2</sup> (standardized beta 0.55 and 0.47 respectively, p < 0.0005).</p><p><strong>Discussion: </strong>Sensory NCS amplitudes depend on BMI in obese and non-obese subjects. Lower reference limits for several sensory amplitudes should probably be adjusted for BMI. Reference limits for amplitude-dependent Z-compounds should be corrected for BMI. The findings have relevance for all patients investigated with nerve conduction studies, in particular, patients with suspected axonal polyneuropathies.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143720421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-27DOI: 10.1002/mus.28405
Stacey Sullivan, Carina Stafstrom, Natalie Grant, William S David, Feodor Price, Lee Rubin, Florian Eichler, Reza Sadjadi
{"title":"Prospective Dysphagia Assessment in Adult Patients With Nephropathic Cystinosis.","authors":"Stacey Sullivan, Carina Stafstrom, Natalie Grant, William S David, Feodor Price, Lee Rubin, Florian Eichler, Reza Sadjadi","doi":"10.1002/mus.28405","DOIUrl":"https://doi.org/10.1002/mus.28405","url":null,"abstract":"<p><strong>Introduction: </strong>Myopathy and dysphagia are common manifestations of nephropathic cystinosis, a rare autosomal recessive lysosomal storage disorder. We previously demonstrated that both the oral and pharyngeal phases of swallowing are affected and impact function and quality of life in patients. To better understand and characterize swallowing impairment, we evaluated patients with nephropathic cystinosis experiencing dysphagia and myopathy.</p><p><strong>Methods: </strong>We prospectively evaluated patients who had participated in a prior clinical trial readiness study of myopathy and dysphagia, using functional outcomes and video fluoroscopic swallowing studies.</p><p><strong>Results: </strong>Eight patients underwent functional and swallowing assessments. Oral and pharyngeal stages of swallowing were affected in all subjects. There was an improvement in oral stage dysphagia (Visit 3 8 [3:9]; Visit 4 5 [1:5]) between Visits 3 and 4 median of 1090 days (IQR [921:1288]) while pharyngeal stage dysphagia Modified Barium Swallow Impairment Profile scores stayed unchanged in the same amount of time. There was also improvement in M. D. Anderson Dysphagia Inventory (MDADI) scores (MDADI-C Visit 3 73 [62:87], Visit 4 96 [76:99]; MDADI-F Visit 3 18 [17:21], Visit 4 25 [23:25]), indicating a higher quality of life pertaining to swallowing.</p><p><strong>Discussion: </strong>Despite the progressive nature of the myopathic changes typically described in patients with nephropathic cystinosis, we demonstrated improvement in some swallowing metrics. This may suggest a possible late-onset effect of respiratory training or cognitive-behavioral impact from repeated swallowing measurements.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143720416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-23DOI: 10.1002/mus.28399
Alper Mengi
{"title":"Comment on Hydrodissection With or Without Corticosteroid Versus Corticosteroid-Only Injection for Carpal Tunnel Syndrome: Double-Blind Randomized Controlled Trial.","authors":"Alper Mengi","doi":"10.1002/mus.28399","DOIUrl":"https://doi.org/10.1002/mus.28399","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-21DOI: 10.1002/mus.28398
Grace Addy, Erica Scirocco, Dario Gelevski, Margot Rohrer, Aimee Roderick, Mary McCormack, Anat Weiss Sadan, Jennifer Scalia, Neil Parikh, Elisa Giacomelli, Matteo Locatelli, Dylan V Neel, Derek D'Agostino, Alex Leite, Hong Yu, Alexander V Sherman, Jarrad Mock, Andreas Kalmes, Sarah Luppino, Suma Babu, James Berry, Merit Cudkowicz, Sabrina Paganoni
{"title":"An Expanded Access Protocol of RNS60 in Amyotrophic Lateral Sclerosis.","authors":"Grace Addy, Erica Scirocco, Dario Gelevski, Margot Rohrer, Aimee Roderick, Mary McCormack, Anat Weiss Sadan, Jennifer Scalia, Neil Parikh, Elisa Giacomelli, Matteo Locatelli, Dylan V Neel, Derek D'Agostino, Alex Leite, Hong Yu, Alexander V Sherman, Jarrad Mock, Andreas Kalmes, Sarah Luppino, Suma Babu, James Berry, Merit Cudkowicz, Sabrina Paganoni","doi":"10.1002/mus.28398","DOIUrl":"https://doi.org/10.1002/mus.28398","url":null,"abstract":"<p><strong>Aims: </strong>RNS60 is an investigational product in clinical development for amyotrophic lateral sclerosis (ALS). RNS60 slowed disease progression in the ALS SOD1<sup>G93A</sup> mouse model and was safe and well tolerated both in an open-label pilot study and a randomized, placebo-controlled, multicenter phase 2 trial in people living with ALS. The objective of this ongoing expanded access protocol (EAP) was to provide RNS60 to people living with ALS who are ineligible for controlled clinical trials and to collect data on the safety and tolerability of dosing RNS60 via twice-daily nebulization rather than the previously studied daily nebulization with weekly intravenous administration.</p><p><strong>Methods: </strong>Eligible participants (≥ 18 years old, diagnosed with ALS per investigator assessment, and ineligible for an ALS clinical trial testing RNS60) were treated with twice-daily nebulization of RNS60 at home. Safety was evaluated by the assessment of adverse events and routine safety labs.</p><p><strong>Results: </strong>A total of 84 participants have been treated with RNS60 via nebulization twice daily for up to 48 months so far. The most common treatment-related adverse event was increased secretions [N = 27 (32%)]. Serious adverse events (SAEs) [69 occurrences; N = 38 (45%) with at least one SAE] and deaths [N = 24 (28%)] were deemed not related to RNS60.</p><p><strong>Discussion: </strong>This EAP supports the benign side effect profile of RNS60 when administered via twice-daily nebulization and demonstrates the feasibility of long-term EAPs as a complementary approach to controlled trials in people with advanced ALS.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143670521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-20DOI: 10.1002/mus.28377
Jeremy P Steen, Kishore S Jaiswal, Dinesh Kumbhare
{"title":"Myofascial Pain Syndrome: An Update on Clinical Characteristics, Etiopathogenesis, Diagnosis, and Treatment.","authors":"Jeremy P Steen, Kishore S Jaiswal, Dinesh Kumbhare","doi":"10.1002/mus.28377","DOIUrl":"https://doi.org/10.1002/mus.28377","url":null,"abstract":"<p><p>Myofascial pain syndrome (MPS) is a chronic regional pain condition characterized by trigger points-hyperirritable spots within taut bands of muscle fibers that cause both localized and referred pain. The pathogenesis, diagnostic criteria, and classification of MPS are still under investigation, which complicates the development of standardized treatment protocols. Although diagnostic tools have improved, MPS often remains underrecognized due to symptom overlap with other pain disorders, such as fibromyalgia, neuropathic pain, and joint disorders. Factors contributing to its onset and persistence include muscle overuse, postural imbalance, systemic conditions, and psychological and behavioral influences. This narrative review explores the primary risk factors, current hypotheses on pathogenesis, diagnosis and differential diagnosis, and both conventional and emerging treatments. Sufficient evidence supports the use of local anesthetic injections for MPS. Some evidence suggests that dry needling, acupuncture, magnetic stimulation, ultrasound therapy, laser therapy, extracorporeal shock wave therapy, and manual therapy may be effective, particularly compared to sham or placebo. However, non-steroidal anti-inflammatory drugs, diclofenac, botulinum toxin, and transcutaneous electrical nerve stimulation show insufficient evidence, while the effectiveness of muscle relaxants, antidepressants, gabapentin, opioids, topical lidocaine, capsaicin, EMLA cream, and kinesio taping remains inconclusive. Effective management of MPS requires a patient-centered approach that integrates empirically supported and evidence-based treatments tailored to individual needs. This review synthesizes the current understanding of MPS and highlights the need for high-quality research to improve clinical decision-making in managing this complex condition.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143663943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Diaphragmatic Compound Muscle Action Potentials Correlate With the Clinical Severity of Myasthenia Gravis.","authors":"Hirotomo Suehiro, Kenji Sekiguchi, Yoshikatsu Noda, Shun Matoba, Shunsuke Watanabe, Masahiro Hayashi, Masaaki Yoshikawa, Ryosuke Sugisawa, Riki Matsumoto","doi":"10.1002/mus.28397","DOIUrl":"https://doi.org/10.1002/mus.28397","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Patients with myasthenia gravis (MG) often experience impairment of respiratory function. Some case reports have explored the diaphragmatic compound muscle action potential (DCMAP) amplitudes in patients with MG using single-stimulus phrenic nerve conduction studies. Herein, we aimed to investigate whether the DCMAP amplitude correlated with the clinical severity of MG and whether this improved with treatment.</p><p><strong>Methods: </strong>We retrospectively evaluated patients with MG who underwent right phrenic nerve conduction studies. We evaluated the patients' clinical characteristics and the clinical severity of MG, as assessed by compound muscle action potential (CMAP) amplitudes, and compared the CMAP amplitudes before and after treatment in patients with generalized MG (gMG).</p><p><strong>Results: </strong>This study included 76 patients with MG (30 with ocular MG [OMG] and 46 with gMG). The median DCMAP amplitudes were significantly higher in OMG than in gMG (364 vs. 212 μV, p < 0.01). Receiver operating characteristic analysis showed that DCMAP amplitude < 256 μV distinguished gMG from OMG with 71.7% sensitivity and 93.3% specificity. The DCMAP amplitudes were moderately correlated with the MG activities of daily living score (p < 0.01, r = -0.52) and quantitative myasthenia gravis score (p < 0.01, r = -0.60). Median DCMAP amplitudes significantly increased in patients with gMG after treatment (from 184 to 336 μV, p < 0.01).</p><p><strong>Discussion: </strong>DCMAP amplitudes may correlate with the clinical severity of MG, differentiate gMG from OMG, and reflect the treatment response. We propose that DCMAP measurement be included in the evaluation of patients with MG.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143663939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-19DOI: 10.1002/mus.28393
Marc Gotkine, David A Schoenfeld, Ilana Cohen, Jeremy M Shefner, Yossef Lerner, Irun R Cohen, Colin Klein, Eran Ovadia, Merit E Cudkowicz
{"title":"Akt Activation With IPL344 Treatment for Amyotrophic Lateral Sclerosis: First in Human, Open-Label Study.","authors":"Marc Gotkine, David A Schoenfeld, Ilana Cohen, Jeremy M Shefner, Yossef Lerner, Irun R Cohen, Colin Klein, Eran Ovadia, Merit E Cudkowicz","doi":"10.1002/mus.28393","DOIUrl":"https://doi.org/10.1002/mus.28393","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Akt intracellular signal transduction pathway dysfunction has been reported in people with amyotrophic lateral sclerosis (ALS) providing a novel target for intervention in this devastating progressive disease. This first-in-human study evaluated the safety, tolerability, and preliminary efficacy of the Akt pathway activator, IPL344, in people with ALS.</p><p><strong>Methods: </strong>Nine participants with ALS and a progression rate > 0.55 points/month on the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) received open-label IPL344 treatment (once-daily) for up to 36 months. Safety was assessed through adverse event (AE) reporting. Plasma neurofilament light chain (NfL) concentrations were measured before and after treatment. Clinical outcomes were compared to historical data.</p><p><strong>Results: </strong>The mean ± SD duration of IPL344 follow-up was 14.0 ± 12.5 months. One participant developed drug hypersensitivity, two had central venous catheter-related AEs, and two had serious pneumonia AEs. The unadjusted mean ± SE slope of decline in ALSFRS-R was -0.53 ± 0.15 (48% slower progression vs. historical controls, p = 0.028). Adjustment for disease stage and rate-indicating covariates indicated a 64% slower ALSFRS-R progression (p = 0.034), with increased rather than reduced body weight (p = 0.02). Eight of nine IPL344-treated participants had a significantly improved slope compared to the median slope of a matched control group (p = 0.04). Plasma NfL concentrations were lowered by 27% (n = 6). Unadjusted median survival for participants in the IPL344 group was 43.4 months [95% CI: 20.5, NA] compared with 19.1 months [17.4, 23.0] in the historical control group.</p><p><strong>Discussion: </strong>These preliminary data indicate that IPL344 was safe and well-tolerated, and possibly effective. Our findings may merit further investigation in a larger placebo-controlled clinical trial.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143657746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-17DOI: 10.1002/mus.28395
Eleanor Greiner, Chet R Villa
{"title":"\"An Ounce of Prevention…\".","authors":"Eleanor Greiner, Chet R Villa","doi":"10.1002/mus.28395","DOIUrl":"https://doi.org/10.1002/mus.28395","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143649825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-03-14DOI: 10.1002/mus.28396
Antonio E Camelo-Filho, Pedro L G S B Lima, Rodrigo F da Rosa, Tito B S Soares, André L S Pessoa, Paulo R Nóbrega, Pedro Braga-Neto
{"title":"Nerve Ultrasound Detects Nerve Atrophy in Patients With Ataxia-Telangiectasia: A Pilot Study.","authors":"Antonio E Camelo-Filho, Pedro L G S B Lima, Rodrigo F da Rosa, Tito B S Soares, André L S Pessoa, Paulo R Nóbrega, Pedro Braga-Neto","doi":"10.1002/mus.28396","DOIUrl":"https://doi.org/10.1002/mus.28396","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Ataxia-telangiectasia (A-T) is a genetic multisystem neurodegenerative disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, extrapyramidal involvement, peripheral sensorimotor neuropathy, immunodeficiency, pulmonary disease, and an increased risk of malignancy that ultimately determines the shortened lifespan in many patients. A-T nerve ultrasonographic characteristics remain underexplored. This pilot study aimed to characterize the ultrasonographic morphology of peripheral nerves in patients with A-T.</p><p><strong>Methods: </strong>Ultrasound cross-sectional areas (CSAs) of the median, ulnar, sural, and tibial nerves were obtained from three A-T patients and were compared to reference values. Nerve conduction studies and electromyography were also performed. Given the small sample size and the exploratory nature of this study, formal statistical analyses were not performed, and descriptive statistics were presented for the data.</p><p><strong>Results: </strong>Nerve CSAs in A-T patients were smaller than in healthy controls at all measurement sites.</p><p><strong>Discussion: </strong>Nerve ultrasound revealed atrophy in the peripheral nerves of A-T patients. This reduction in nerve size may distinguish A-T and highlights the utility of nerve ultrasound as a non-invasive diagnostic tool for peripheral sensorimotor neuropathy. These findings may have important implications for early detection in clinical practice.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143630650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}