Muscle & Nerve最新文献

{"title":"Exploring the Value of Quantitative Muscle Ultrasound in Neuromuscular Junction Disorders: A Pilot Study.","authors":"Artor Pogosean, Maarika Liik, Nens van Alfen, Anna Rostedt Punga","doi":"10.1002/mus.70361","DOIUrl":"10.1002/mus.70361","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Quantitative muscle ultrasound (QMUS) is a validated technique for assessing muscle pathology, yet its role in disorders primarily affecting the neuromuscular junction (NMJ) remains unclear. This pilot study aimed to explore whether QMUS is associated with structural muscle changes in patients with acquired or genetic NMJ disorders.</p><p><strong>Methods: </strong>We conducted a retrospective study of 18 patients with confirmed NMJ disorders who underwent electrodiagnostic testing (EDx), including repetitive nerve stimulation (RNS) and/or stimulated single-fiber electromyography (SFEMG), combined with QMUS. Patients were grouped into congenital myasthenic syndromes (CMS) and acquired autoimmune myasthenia (myasthenia gravis [MG] or Lambert-Eaton myasthenic syndrome [LEMS]). QMUS scores were classified as normal, borderline, or abnormal based on muscle echogenicity z-scores derived from a validated algorithm.</p><p><strong>Results: </strong>Abnormal EDx was present in 13 of 18 (72%) patients. Abnormal decrement (> 10%) was observed in 7/18 patients, and abnormal jitter in the orbicularis oculi muscle was found in 6/8 patients. QMUS demonstrated normal echogenicity in 14/18 (78%) patients, while 4/18 (22%) showed abnormal or borderline echogenicity, including 2/5 (40%) with generalized MG. All patients with borderline or abnormal QMUS findings had concomitant abnormal RNS and/or SFEMG.</p><p><strong>Discussion: </strong>Structural muscle changes, reflected by altered echogenicity, may occur in a subset of patients with NMJ disorders, although QMUS shows limited sensitivity compared with EDx. Future studies using standardized protocols, dynamic assessments, and targeted evaluation of clinically relevant muscles, such as facial and bulbar, are needed to determine whether QMUS may serve as a complementary tool for disease characterization or monitoring.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1281-1287"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549164/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148630917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Imaging of Facial Muscles in Facioscapulohumeral Muscular Dystrophy: An Exploratory Study Comparing Magnetic Resonance Imaging and Ultrasound.","authors":"Federico Pistoia, Elena Faedo, Marta Macciò, Giovanni Marcenaro, Maria Elena Susi, Mehrnaz Hamedani, Veronica Caramellino, Sara Massucco, Andrea Francesco Maria Vollaro, Paola Mandich, Fabio Gotta, Riccardo Picasso, Federico Zaottini, Marina Grandis, Carlo Martinoli","doi":"10.1002/mus.70360","DOIUrl":"10.1002/mus.70360","url":null,"abstract":"<p><strong>Aims: </strong>Facioscapulohumeral muscular dystrophy (FSHD) is a genetic progressive muscle disorder often presenting with facial weakness. However, imaging studies specifically evaluating facial muscle involvement and its relationship with clinical severity remain limited. This preliminary study explored magnetic resonance imaging (MRI) and ultrasound (US) assessment of facial muscle involvement and correlations with clinical severity.</p><p><strong>Methods: </strong>This single-center prospective study enrolled 12 genetically confirmed FSHD1 patients (mean age 57.8 years). Clinical evaluation included the FSHD score, Comprehensive Clinical Evaluation Form (CCEF), and Facial Weakness Score (FWS). Muscle thickness of the orbicularis oris (OOr) and zygomaticus major (ZMj) was measured on both MRI and US, while MRI was additionally used for qualitative assessment of muscle bulk. Inter-rater reliability was assessed for imaging measurements.</p><p><strong>Results: </strong>MRI and US showed comparable facial muscle thickness, with mean ZMj thickness of 2.34 ± 0.75 mm and 2.12 ± 0.55 mm, and OOr thickness of 2.28 ± 0.70 mm and 2.20 ± 0.67 mm, respectively. Inter-rater reliability for MRI was high (ICC = 0.84), and reduced ZMj thickness showed moderate to strong correlation with greater disease severity (ρ up to 0.87, p < 0.001) and impaired facial gestures (ρ = 0.50-0.77, p < 0.05).</p><p><strong>Discussion: </strong>Despite the small sample size, MRI, and US assessment of facial muscles correlated with clinical severity and functional outcomes in FSHD, supporting their use as complementary tools. Longitudinal studies are required to establish imaging's role in monitoring changes over time.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1256-1262"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549152/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148679085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Myotonia: Recognition, Evaluation, and Differential Diagnosis.","authors":"Jesse I Crayle, Muhammad Al-Lozi, Timothy M Miller","doi":"10.1002/mus.70250","DOIUrl":"10.1002/mus.70250","url":null,"abstract":"<p><p>Myotonia is a non-neurotypical muscle physiology of sarcolemmal hyperexcitability due to alterations in the structure and/or function of ion channels in the muscle cell membrane. This hyperexcitability can be observed electrically as spontaneous myotonic discharges during needle electromyography. Myotonic discharges consist of continuous sequential fibrillation or positive sharp wave morphology potentials which exhibit unstable, gradually changing, firing frequencies and amplitudes. In myotonic disorders, a clinical correlate of muscle stiffness is often present. Myotonic disorders are broadly divided into myotonic dystrophies (Types 1 and 2) and non-dystrophic myotonias (myotonia congenita, paramyotonia congenita, and sodium channel myotonias). The myotonic dystrophies are systemic disorders of dysregulated RNA splicing clinically exhibiting fixed weakness, cataracts, diabetes and cardiac disease. Non-dystrophic myotonic disorders are due to specific sarcolemmal ion channel genetic variants and generally clinically limited to muscle stiffness (myotonia) sometimes with muscle weakness which can be fixed or periodic. Although electrical myotonia is a distinctive feature of myotonic disorders, it is nonspecific and may occur in other neuromuscular conditions. Widespread myotonic discharges strongly suggest a myotonic disorder or a handful of other conditions including necrotizing autoimmune myopathy, some toxic myopathies, Pompe disease and several congenital myopathies. This monograph reviews clinical myotonia and mimics, electrical myotonic discharges, electrodiagnostic testing in myotonic disorders and clinical features of myotonic disorders and other myopathies with myotonia.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1174-1195"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549170/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sensory Cortical Hyperexcitability in Amyotrophic Lateral Sclerosis Involves a Broad Hand Representation Within the Primary Somatosensory Cortex.","authors":"Yoshiyuki Matsuki, Kota Bokuda, Ryo Morishima, Tomoya Kawazoe, Hideki Kimura, Kazushi Takahashi, Yuki Nakayama, Toshio Shimizu","doi":"10.1002/mus.70371","DOIUrl":"10.1002/mus.70371","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Sensory cortical hyperexcitability, reflected by enlarged median nerve somatosensory evoked potentials (SEPs), has been reported in amyotrophic lateral sclerosis (ALS) and is associated with shorter survival. This study investigated whether similar changes involve the ulnar nerve representation within the hand area of the primary somatosensory cortex and examined their relationship with survival.</p><p><strong>Methods: </strong>Ninety-nine patients with sporadic ALS and 42 healthy controls were retrospectively studied. Sensory nerve action potentials (SNAPs) and SEPs were recorded following median and ulnar nerve stimulation. SNAP amplitudes and the peak-to-peak amplitudes between N20 and P25 (N20p-P25p) were compared between groups. Patients were followed until death or tracheostomy, and associations between SEP amplitudes and survival were analyzed using Kaplan-Meier and Cox proportional hazards analyses.</p><p><strong>Results: </strong>SNAP amplitudes did not differ between patients and controls. In contrast, patients with ALS showed larger N20p-P25p amplitudes for both median and ulnar nerve SEPs. N20p-P25p amplitudes were positively correlated between the two nerves, and the ulnar-to-median amplitude ratio did not differ from controls. Patients with ulnar N20p-P25p ≥ 4.89 μV had significantly shorter survival than those with lower amplitude (log-rank test, p = 0.035). Multivariate Cox analysis identified increased N20p-P25p amplitude as an independent predictor of shorter survival for both nerves.</p><p><strong>Discussion: </strong>Sensory cortical hyperexcitability in ALS extends beyond the median nerve to the ulnar nerve hand area of the somatosensory cortex. Its association with survival supports the notion that sensory cortical dysfunction represents a fundamental pathophysiological feature of ALS and a potential electrophysiological prognostic marker.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1246-1255"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148670230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"\"Abductor Sparing\": A New Selective Involvement in ALS.","authors":"Amuro Kondo, Keiichi Hokkoku, Takamichi Kanbayashi, Takahiro Nakayama, Takuya Sasaki, Hiromasa Matsuno, Yasuyuki Iguchi, Toshio Fukutake, Tetsuo Ando, Tsutomu Kamiyama, Pyou Kim, Takahiro Hasegawa, Yuki Hatanaka, Shunsuke Kobayashi, Masahiro Sonoo","doi":"10.1002/mus.70374","DOIUrl":"10.1002/mus.70374","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Various signs of selective muscle involvement have been reported in amyotrophic lateral sclerosis (ALS) but such studies for the lower limbs are scarce. We formed a preliminary impression that hip abductors (Ab) are often preserved in ALS. We named this phenomenon \"abductor sparing\", and this study aimed to verify our findings.</p><p><strong>Methods: </strong>Patients with a confirmed diagnosis of ALS (ALS group) and patients with pyramidal weakness other than ALS (pyramidal group) were retrospectively identified. Medical Research Council (MRC) scores of 10 muscle groups in the lower limbs were evaluated. The proportion of patients with weakness (MRC score 4 or less) was compared between different groups.</p><p><strong>Results: </strong>We enrolled 61 patients in the ALS group and 27 patients in the pyramidal group. The most frequently weak muscle groups in both groups were big toe extensors and hip flexors. Ab was the third (70%) in the pyramidal group, whereas it was weak only in 30% of patients with ALS. This held true also for patients with ALS with shorter duration or less severity. \"The lower limb flexor pattern\", i.e., flexor muscles being weaker than extensor muscles, was observed both in ALS and pyramidal groups.</p><p><strong>Discussion: </strong>Patients with ALS generally showed similar muscle weakness patterns to those with pyramidal syndrome, except for abductor sparing. The reason for the latter phenomenon is unclear. Abductor sparing may be useful for early diagnosis of ALS, although larger studies with blinded evaluators are needed to confirm these findings.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1263-1269"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549161/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148697903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Proceedings of the 25^th Annual Meeting of the Northeast ALS Consortium.","authors":"","doi":"10.1002/mus.70436","DOIUrl":"https://doi.org/10.1002/mus.70436","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"74 Suppl 2 ","pages":"S1-S112"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149245613","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":"Myasthenia Gravis and Thymoma.","authors":"Benjamin Claytor, Lukas Delasos, Daniel P Raymond, Erik Seroogy, Jason Lempel, Yuebing Li","doi":"10.1002/mus.70318","DOIUrl":"10.1002/mus.70318","url":null,"abstract":"<p><p>Thymoma is the most common tumor of the anterior mediastinum. Approximately 20%-30% of patients with a thymoma develop myasthenia gravis (MG), and an additional one third may possess positive acetylcholine receptor (AChR) antibodies without MG. Historical estimates suggest that thymoma associated MG (TAMG) represents roughly 15%-20% of MG patients, although the proportion of TAMG has been decreasing. Virtually all TAMG patients are seropositive for AChR antibodies. In comparison to MG without thymoma, TAMG is mostly generalized and more refractory to treatment. Chest CT with contrast is the imaging modality of choice in evaluating thymoma, while MRI and PET scan may have an ancillary role in defining tumor invasiveness, staging and further differentiating thymoma from other mediastinal masses. Surgical resection should be considered for all patients with thymoma, regardless of MG status as all thymomas are malignant. Minimally invasive surgery appears equally efficacious as sternotomy for patients with early stage thymoma, and has become more prevalent due to its lower complication rates. Preoperative optimization with intravenous immunoglobulin or plasmapheresis is unnecessary in patients with stable MG. New onset MG could occur in thymoma patients following thymectomy, and the presence of AChR antibody preoperatively may be a predictive risk factor. Patients with advanced stage thymoma may require neoadjuvant or adjuvant chemotherapy and/or radiation, typically managed in a multidisciplinary setting. The recurrence rate of thymoma is mainly dependent upon its pathological stage, and worsening myasthenic symptoms may herald a recurrence of thymoma.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1156-1173"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148284081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lumbosacral Plexopathy: A Single-Center Review of Etiology, Clinical Presentation, Electrodiagnostic Findings, and Long-Term Outcomes.","authors":"Bradley Ong, Jad El Ahdab, Nicolas Thompson, Yuebing Li, Nirmal Andrapalliyal","doi":"10.1002/mus.70359","DOIUrl":"10.1002/mus.70359","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Lumbosacral plexopathy (LSP) results from a spectrum of etiologies and can cause substantial functional impairment. Limited data exist on the relative distribution of etiologies and outcomes across broad tertiary-care cohorts spanning multiple causes of LSP. This study aimed to describe the etiologic distribution and characterize the clinical, electrodiagnostic (EDx), and outcome features of LSP.</p><p><strong>Methods: </strong>We retrospectively reviewed records of patients diagnosed with LSP at our center from January 2000 to September 2024. Definite LSP was defined by a clinical and electrodiagnostic pattern localizing predominantly to the lumbosacral plexus, with supportive sensory and/or imaging findings. Probable LSP met similar clinical and EDx criteria but lacked sufficient sensory or imaging confirmation.</p><p><strong>Results: </strong>Of 1139 screened records, 124 patients met criteria for LSP (38 definite [30.6%], 86 probable [69.4%]). Median age was 64 years (IQR 54-72), and 59 (47.6%) were female. Symptom onset was acute in 47 (37.9%), subacute in 9 (7.3%), and chronic in 68 (54.8%). Sensorimotor symptoms predominated (71.0%). Leading etiologies included diabetic lumbosacral radiculoplexus neuropathy (34.7%), perioperative injury (25.8%), idiopathic LSP (14.5%), radiation-induced plexopathy (12.1%), and autoimmune LSP (6.5%). Among 85 patients with follow-up, 35 (41.2%) achieved full or near-complete recovery.</p><p><strong>Discussion: </strong>These findings are consistent with prior descriptions of LSP and radiculoplexus neuropathy and emphasize the heterogeneity of etiologies seen in tertiary care. Recovery was often incomplete, supporting the need for careful diagnostic evaluation and realistic counseling regarding outcome.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1220-1229"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549159/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148664102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic Performance of the Strength-Duration Test for Bedside Screening of Critical Illness Polyneuropathy and/or Myopathy: A Prospective, Cross-Sectional Study.","authors":"José Roberto de Deus Macedo, Emerson Fachin-Martins, Marilia Mendes Rodrigues, Marta Rodrigues de Carvalho, Rubens Nelson Morato Fernandez, Paulo Eugênio Silva","doi":"10.1002/mus.70368","DOIUrl":"10.1002/mus.70368","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Critical illness polyneuropathy and/or myopathy (CIP/CIM) is a major cause of weakness in the intensive care unit (ICU). The availability of conventional electrodiagnostic testing may be limited. Alternative electrophysiologic methods, including the strength-duration test (SDT) and the stimulus electrodiagnosis test (SET), have been proposed as bedside screening adjuncts. This study aimed to evaluate the SDT's diagnostic metrics for CIP/CIM screening and to compare with the SET's performance.</p><p><strong>Methods: </strong>This prospective, cross-sectional study included adult, mechanically ventilated ICU patients. Single assessment per patient comprised the peroneal simplified electrophysiological test (PENT), SDT, and SET. Using PENT as the reference test, we calculated the accuracy, sensitivity, specificity, predictive values, and likelihood ratios of SDT. Receiver operating characteristic curves were constructed to assess the discriminative performance and optimal cutoff.</p><p><strong>Results: </strong>Sixty participants were enrolled, yielding 101 assessments, including bilateral evaluations when feasible. The SDT demonstrated an area under the curve (AUC) of 0.8 (95% CI: 0.7-0.8, p < 0.001), with an optimal cutoff of 600 mC. At this cutoff, SDT showed a sensitivity of 73% (95% CI: 61-82), specificity of 68% (95% CI: 53-81), accuracy of 71% (95% CI: 62-79), and positive likelihood ratio of 2.3 (95% CI: 1.41-3.78) for screening probable CIP/CIM. The SET yielded an AUC of 0.7 (95% CI: 0.6-0.8, p = 0.001).</p><p><strong>Discussion: </strong>The SDT showed potential as a bedside screening adjunct for CIP/CIM in the ICU setting. Further multicenter studies are required to validate these findings and to define the role of SDT in clinical practice.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1212-1219"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549163/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148630980","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quantitative Assessment of Tongue Atrophy in Amyotrophic Lateral Sclerosis Using Transoral Tongue Ultrasonography (TOTU): A Retrospective Single-Center Study.","authors":"Naoki Iijima, Yuta Hagiwara, Seiya Ishii, Wataru Kaneko, Takashi Araga, Yoshihisa Yamano","doi":"10.1002/mus.70370","DOIUrl":"10.1002/mus.70370","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease accompanied by bulbar dysfunction, in which tongue atrophy contributes to dysarthria and dysphagia. Conventional tongue assessments rely on inspection, palpation, or electrophysiological testing but are limited by subjectivity and invasiveness. A rapid, objective, and repeatable imaging-based method for evaluating tongue size would be valuable. We quantified tongue atrophy in ALS using a standardized protocol for transoral tongue ultrasonography (TOTU) and assessed discrimination.</p><p><strong>Methods: </strong>This retrospective, cross-sectional study included 15 patients with ALS and 15 controls who underwent TOTU. Using standardized mid-sagittal images, tongue cross-sectional area (cm²) and tongue thickness (mm) were measured three times per subject and averaged. Associations with age, body mass index (BMI), disease duration, and ALS Functional Rating Scale-Revised (ALSFRS-R) scores were explored. Discrimination was evaluated using receiver operating characteristic (ROC) analyses.</p><p><strong>Results: </strong>Both tongue cross-sectional area (6.26 ± 1.37 vs. 9.44 ± 1.02 cm²) and tongue thickness (31.47 ± 3.0 vs. 39.44 ± 1.39 mm) were significantly lower in the ALS group than in controls (both p < 0.001). These indices showed no significant associations with age, BMI, disease duration, or ALSFRS-R scores. ROC analyses showed high discriminative ability, with an area under the curve of 0.982 for tongue cross-sectional area and 1.000 for tongue thickness.</p><p><strong>Discussion: </strong>Standardized TOTU enables rapid, quantitative, noninvasive assessment of tongue size and reliably detects tongue atrophy in ALS, supporting bedside evaluation of bulbar involvement.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1238-1245"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549162/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148670253","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}