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Timing of intravenous immunoglobulin treatment and outcome in Guillain-Barré syndrome: Is time nerve? 静脉注射免疫球蛋白的时机与格林-巴利综合征的治疗效果:时间就是神经吗?
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-12-01 Epub Date: 2024-10-03 DOI: 10.1002/mus.28271
Young Gi Min, Yoon-Ho Hong, Yusuf A Rajabally, Jung-Joon Sung
{"title":"Timing of intravenous immunoglobulin treatment and outcome in Guillain-Barré syndrome: Is time nerve?","authors":"Young Gi Min, Yoon-Ho Hong, Yusuf A Rajabally, Jung-Joon Sung","doi":"10.1002/mus.28271","DOIUrl":"10.1002/mus.28271","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Despite treatment, a considerable proportion of patients with Guillain-Barré syndrome (GBS) experience poor recovery, highlighting a therapeutic need. There is a lack of evidence that treatment timing affects recovery. This study aims to investigate the effects of intravenous immunoglobulin (IVIg) timing on disability and speed of recovery in GBS.</p><p><strong>Methods: </strong>We performed a retrospective study of 136 IVIg-treated GBS patients admitted to two Korean centers between 2010 and 2021. We analyzed the effect of time to IVIg on the GBS disability scale (GBS-DS) and the degree of improvement from nadir (∆GBS-DS) at 1, 3, 6, and 12 months, as well as the time to regain the ability to walk or run unaided. Time to IVIg was treated either as a continuous variable or categorized into 1-week intervals to explore critical time windows. Known prognostic factors, the modified Erasmus GBS Outcome Scores on admission and pre-treatment serum albumin levels were adjusted as covariates.</p><p><strong>Results: </strong>Shorter time to IVIg was independently associated with better GBS-DS, greater ∆GBS-DS, and shorter time to walk or run unaided at all time points. The therapeutic effect of IVIg was notably diminished when administered beyond the first 2 weeks of onset.</p><p><strong>Discussion: </strong>Our study highlights the timing of IVIg as a modifiable prognostic factor in GBS. The earlier IVIg is initiated, the better the outcomes, with the ideal time window being within the first 2 weeks. These findings underscore the importance of prompt diagnosis and early intervention to optimize recovery in GBS patients.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365860","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}
引用次数: 0
Reliability of a new stabilization device for measurement of muscle strength using a hand-held dynamometer. 使用手持式测力计测量肌肉力量的新型稳定装置的可靠性。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-12-01 Epub Date: 2024-10-05 DOI: 10.1002/mus.28266
Zeynal Yasacı, Sezen Karaborklu Argut, Derya Celik
{"title":"Reliability of a new stabilization device for measurement of muscle strength using a hand-held dynamometer.","authors":"Zeynal Yasacı, Sezen Karaborklu Argut, Derya Celik","doi":"10.1002/mus.28266","DOIUrl":"10.1002/mus.28266","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The hand-held dynamometer (HHD) is a reliable and portable tool for quantitatively assessing muscle strength. However, a number of variables, including the strength of the tester, the adequacy of stabilization, and the selected testing position, can affect the reliability of the device. The objective of the present study was to examine the reproducibility and inter-rater reliability of strength assessments using HHD with and without a stabilization device.</p><p><strong>Methods: </strong>Strength assessments were conducted with and without the stabilization device by two independent raters. Healthy volunteers with no history of musculoskeletal disorders were included in the study. The strength of the shoulder flexion, scapula elevation, knee extension, and hip abduction muscle groups was evaluated using HHD. The reliability of the measurements was evaluated using intra-class correlation (ICC), standard error of measurement, and minimal detectable change.</p><p><strong>Results: </strong>Fifty-two healthy volunteers (age 21.51 ± 2.02 years) participated in the study. The reproducibility was found to be excellent (ICC = 0.89-0.99). While reliability between the assessors was good to excellent (ICC = 0.85-0.93), reliability between assessors and device was found moderate to good (ICC = 0.48-0.78).</p><p><strong>Discussion: </strong>The strength values obtained for all directions of movement with the stabilization device were found to be significantly higher than those obtained without fixation of the HHD. It can be concluded that the data obtained from the assessors and HHD with stabilization device are not comparable. Moreover, the utilization of a stabilization device may serve to enhance the reliability of the measurements by eliminating the rater effect.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375655","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}
引用次数: 0
Volumetric muscle composition analysis in sporadic inclusion body myositis using fat-referenced magnetic resonance imaging: Disease pattern, repeatability, and natural progression. 利用脂肪参考磁共振成像对散发性包涵体肌炎的肌肉成分进行容积分析:疾病模式、可重复性和自然进展。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-12-01 Epub Date: 2024-09-24 DOI: 10.1002/mus.28252
John Heerfordt, Markus Karlsson, Midori Kusama, Seiya Ogata, Ryuta Mukasa, Naoki Kiyosawa, Noriko Sato, Per Widholm, Olof Dahlqvist Leinhard, André Ahlgren, Madoka Mori-Yoshimura
{"title":"Volumetric muscle composition analysis in sporadic inclusion body myositis using fat-referenced magnetic resonance imaging: Disease pattern, repeatability, and natural progression.","authors":"John Heerfordt, Markus Karlsson, Midori Kusama, Seiya Ogata, Ryuta Mukasa, Naoki Kiyosawa, Noriko Sato, Per Widholm, Olof Dahlqvist Leinhard, André Ahlgren, Madoka Mori-Yoshimura","doi":"10.1002/mus.28252","DOIUrl":"10.1002/mus.28252","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Fat-referenced magnetic resonance imaging (MRI) has emerged as a promising volumetric technique for measuring muscular volume and fat in neuromuscular disorders, but the experience in inflammatory myopathies remains limited. Therefore, this work aimed at describing how sporadic inclusion body myositis (sIBM) manifests on standardized volumetric fat-referenced MRI muscle measurements, including within-scanner repeatability, natural progression rate, and relationship to clinical parameters.</p><p><strong>Methods: </strong>Ten sIBM patients underwent whole-leg Dixon MRI at baseline (test-retest) and after 12 months. The lean muscle volume (LMV), muscle fat fraction (MFF), and muscle fat infiltration (MFI) of the quadriceps, hamstrings, adductors, medial gastrocnemius, and tibialis anterior were computed. Clinical assessments of IBM Functional Rating Scale (IBMFRS) and knee extension strength were also performed. The baseline test-retest MRI measurements were used to estimate the within-subject standard deviation (s<sub>w</sub>). 12-month changes were derived for all parameters.</p><p><strong>Results: </strong>The MRI measurements showed high repeatability in all muscles; s<sub>w</sub> ranged from 2.7 to 18.0 mL for LMV, 0.7-1.3 percentage points (pp) for MFF, and 0.2-0.7 pp for MFI. Over 12 months, average LMV decreased by 7.4% while MFF and MFI increased by 3.8 pp and 1.8 pp, respectively. Mean IBMFRS decreased by 2.4 and mean knee extension strength decreased by 32.8 N.</p><p><strong>Discussion: </strong>The MRI measurements showed high repeatability and 12-month changes consistent with muscle atrophy and fat replacement as well as a decrease in both muscle strength and IBMFRS. Our findings suggest that fat-referenced MRI measurements are suitable for assessing disease progression and treatment response in inflammatory myopathies.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350508","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}
引用次数: 0
The endocrine manifestations of adults with spinal muscular atrophy. 成人脊髓性肌肉萎缩症患者的内分泌表现。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-12-01 Epub Date: 2024-10-05 DOI: 10.1002/mus.28275
Matej Rakusa, Blaž Koritnik, Lea Leonardis, Katja Goricar, Tjasa Rudolf, Dejan Firbas, Žiga Snoj, Mojca Jensterle
{"title":"The endocrine manifestations of adults with spinal muscular atrophy.","authors":"Matej Rakusa, Blaž Koritnik, Lea Leonardis, Katja Goricar, Tjasa Rudolf, Dejan Firbas, Žiga Snoj, Mojca Jensterle","doi":"10.1002/mus.28275","DOIUrl":"10.1002/mus.28275","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Changes in body composition in patients with spinal muscular atrophy (SMA) can cause endocrine abnormalities that are insufficiently studied in adults. We aimed to assess the endocrine profile in a cohort of adults with SMA. Second, we compared body composition and endocrine profiles between nonambulatory and ambulatory patients and between different types of SMA.</p><p><strong>Methods: </strong>The cross-sectional study included 29 SMA patients (18 [62.1%] males and 11 [37.9%] females) of median age 44 (IQR 30-51.5) years with type 2, 3, or 4. Body composition was measured by bioimpedance. Morning blood samples were drawn for glycated hemoglobin (HbA1c), lipid profile, testosterone, cortisol, and insulin-like growth factor-1 (IGF-1). Blood glucose, insulin, and beta-hydroxybutyrate (BHB) were measured during a 75 g oral glucose tolerance test. The homeostatic model assessment for insulin resistance index was calculated.</p><p><strong>Results: </strong>In total, 75.9% of patients had increased fat mass (FM), with 51.7% having an increase despite normal body mass index. Ambulation was the most important discriminating factor of body composition. 93.1% of patients had metabolic abnormalities, including hyperglycemia, insulin resistance, and dyslipidemia. Increased BHB, a marker of ketosis, was present in more than a third of patients. Functional hypogonadism was present in half of male patients. Testosterone and IGF-1 negatively correlated with FM.</p><p><strong>Discussion: </strong>Adult patients with SMA had abnormal body composition and highly prevalent metabolic disturbances that might increase cardiometabolic risk. Because treatments have modified the course of SMA, it is important to investigate whether these observations translate into clinically relevant outcomes.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375656","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}
引用次数: 0
Efficacy and safety of maintenance intravenous immunoglobulin in generalized myasthenia gravis patients with acetylcholine receptor antibodies: A multicenter, double-blind, placebo-controlled trial. 乙酰胆碱受体抗体全身性肌无力患者静脉注射免疫球蛋白的疗效和安全性:一项多中心、双盲、安慰剂对照试验。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-07 DOI: 10.1002/mus.28289
Vera Bril, Tomasz Berkowicz, Andrzej Szczudlik, Michael W Nicolle, Josef Bednarik, Petr Hon, Antanas Vaitkus, Tuan Vu, Csilla Rozsa, Tim Magnus, Gyula Panczel, Toomas Toomsoo, Mamatha Pasnoor, Tahseen Mozaffar, Miriam Freimer, Ulrike Reuner, László Vécsei, Nizar Souayah, Todd Levine, Robert M Pascuzzi, Marinos C Dalakas, Michael Rivner, Rhonda Griffin, Montse Querolt Coll, Elsa Mondou
{"title":"Efficacy and safety of maintenance intravenous immunoglobulin in generalized myasthenia gravis patients with acetylcholine receptor antibodies: A multicenter, double-blind, placebo-controlled trial.","authors":"Vera Bril, Tomasz Berkowicz, Andrzej Szczudlik, Michael W Nicolle, Josef Bednarik, Petr Hon, Antanas Vaitkus, Tuan Vu, Csilla Rozsa, Tim Magnus, Gyula Panczel, Toomas Toomsoo, Mamatha Pasnoor, Tahseen Mozaffar, Miriam Freimer, Ulrike Reuner, László Vécsei, Nizar Souayah, Todd Levine, Robert M Pascuzzi, Marinos C Dalakas, Michael Rivner, Rhonda Griffin, Montse Querolt Coll, Elsa Mondou","doi":"10.1002/mus.28289","DOIUrl":"10.1002/mus.28289","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Prospective, randomized, controlled trials of intravenous immunoglobulin (IVIG) maintenance therapy in myasthenia gravis (MG) are lacking. In this trial, we evaluated the safety and efficacy of caprylate/chromatography-purified IVIG; (IGIV-C) in patients with generalized MG undergoing standard care.</p><p><strong>Methods: </strong>Sixty-two patients enrolled in this phase 2, multicenter, international, randomized trial (1:1 IGIV-C [2 g/kg loading dose; 1 g/kg every 3 weeks through week 21] or placebo). Efficacy was assessed by changes in Quantitative MG (QMG) score at week 24 versus baseline (primary endpoint) and percentage of patients with clinical improvement in QMG, MG Composite (MGC), and MG-Activities of Daily Living (MG-ADL) scores (secondary endpoints). Safety assessments reported all adverse events (AEs).</p><p><strong>Results: </strong>The change in QMG at 24 weeks was -5.1 for IGIV-C and -3.1 for placebo (p = .187). Seventy percent of patients in the IGIV-C group had improvement in MG-ADL (≥2-point decrease) versus 40.6% in the placebo group (p = .025). Patients showing clinical improvement in QMG and MGC (≥3-point decrease) were 70.0% for IGIV-C versus 59.4% for placebo (p = .442) and 60.0% for IGIV-C versus 53.1% for placebo (p = .610). IGIV-C was well tolerated; serious AEs were similar between arms. Three of four MG exacerbations requiring hospitalizations occurred in the IGIV-C arm with one death.</p><p><strong>Discussion: </strong>Several efficacy parameters showed numerical results greater than those seen in the placebo group. This was a small study and may have been underpowered to see significant differences. Additional studies may be warranted to fully determine the efficacy of IVIG maintenance therapy in MG.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142590961","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}
引用次数: 0
Musculoskeletal mimics for lumbosacral radiculopathy. Part 1: Theoretical considerations. 腰骶神经根病的肌肉骨骼模拟治疗。第一部分:理论考虑。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-05 DOI: 10.1002/mus.28280
Anthony E Chiodo, Shawn P Jorgensen
{"title":"Musculoskeletal mimics for lumbosacral radiculopathy. Part 1: Theoretical considerations.","authors":"Anthony E Chiodo, Shawn P Jorgensen","doi":"10.1002/mus.28280","DOIUrl":"https://doi.org/10.1002/mus.28280","url":null,"abstract":"<p><p>The diagnosis of lumbosacral radiculopathy includes the exclusion of common musculoskeletal conditions that can cause similar symptoms. Neurology and physiatry physicians use history taking and physical examination findings to develop a differential diagnosis. Appropriate diagnostic testing is then utilized to narrow down this differential diagnosis to determine a working hypothesis of the cause of a patient's symptoms, leading to a treatment plan. There are stark limitations of patient symptoms and physical examination findings in making the diagnosis of lumbosacral radiculopathy and added value of a combination of symptoms and signs to distinguish patients with lumbosacral radiculopathy from patients with mimic disorders. Diagnostic tests have variable strengths and limitations in helping to confirm this diagnosis, contrasting the high sensitivity and lower specificity of magnetic resonance imaging (MRI) with the high specificity but lower sensitivity of electromyography (EMG). Further complexity is added to the task of making a diagnosis and setting a treatment plan by the fact that these disorders are common and interact with each other; they are present concomitantly in up to 25% of patients presenting for electrodiagnostic evaluation. A companion paper will review common musculoskeletal mimics of lumbosacral radiculopathy and provide tools to anchor testing for those conditions to the traditional neurological evaluation of lumbosacral radiculopathy.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583831","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}
引用次数: 0
Musculoskeletal mimics for lumbosacral radiculopathy. Part 2: Specific disorders. 腰骶神经根病的肌肉骨骼模拟。第二部分:特定疾病。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-05 DOI: 10.1002/mus.28279
Shawn P Jorgensen, Anthony E Chiodo
{"title":"Musculoskeletal mimics for lumbosacral radiculopathy. Part 2: Specific disorders.","authors":"Shawn P Jorgensen, Anthony E Chiodo","doi":"10.1002/mus.28279","DOIUrl":"https://doi.org/10.1002/mus.28279","url":null,"abstract":"<p><p>Lumbosacral radiculopathy is a common disorder evaluated by the electrodiagnostic medicine (EDX) consultant. Making this task difficult is the abundance of radiculopathy mimics. Peripheral neurologic mimics are common, but musculoskeletal mimics are not rare and may be less familiar to many EDX consultants. Awareness of the most common musculoskeletal mimickers-particularly key historical and physical examination features that can distinguish them from radiculopathies-can lead to an accurate diagnosis for the patient and referring provider. Part 1 of this monograph covered theoretical issues surrounding why radiculopathy mimics occur. This second part reviews the most common musculoskeletal mimics, including facet arthropathy, myofascial pain syndrome, hip pathology, greater trochanteric pain syndrome, piriformis syndrome, sacroiliac joint dysfunction, hamstring pathology, iliotibial band syndrome, and plantar fasciitis. Diagnosis of these musculoskeletal mimickers is complicated by nonspecific physical examination and imaging findings, and diagnostic injections are frequently necessary to confirm the diagnosis. Treatment for most mimickers includes physical therapy, anti-inflammatory medications, guided injections, and other conservative measures, only rarely followed by surgical intervention. EDX consultants can efficiently incorporate a few high-yield maneuvers into their physical examination based on the location of the pain to provide answers to patients presenting with a musculoskeletal mimic of a lumbosacral radiculopathy.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583836","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}
引用次数: 0
Chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin M paraprotein, cold agglutinins, and disialosyl antibodies presenting after COVID19 mRNA vaccination. 接种 COVID19 mRNA 疫苗后出现的慢性共济失调性神经病、眼球震颤、免疫球蛋白 M 副蛋白、冷凝集素和二ialosyl 抗体。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-05 DOI: 10.1002/mus.28288
Benjamin Beland, Theodore Mobach
{"title":"Chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin M paraprotein, cold agglutinins, and disialosyl antibodies presenting after COVID19 mRNA vaccination.","authors":"Benjamin Beland, Theodore Mobach","doi":"10.1002/mus.28288","DOIUrl":"https://doi.org/10.1002/mus.28288","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583807","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}
引用次数: 0
Treatment response in patients with clinical and supportive laboratory features of chronic inflammatory demyelinating polyneuropathy without demyelinative findings on nerve conduction studies: A retrospective study. 具有慢性炎症性脱髓鞘性多发性神经病的临床和实验室支持性特征,但神经传导检查未发现脱髓鞘病变的患者的治疗反应:一项回顾性研究。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-07-03 DOI: 10.1002/mus.28198
Patrick Curry, David N Herrmann, Michael Stanton, Phillip Mongiovi, Chary Akmyradov, Eric Logigian
{"title":"Treatment response in patients with clinical and supportive laboratory features of chronic inflammatory demyelinating polyneuropathy without demyelinative findings on nerve conduction studies: A retrospective study.","authors":"Patrick Curry, David N Herrmann, Michael Stanton, Phillip Mongiovi, Chary Akmyradov, Eric Logigian","doi":"10.1002/mus.28198","DOIUrl":"10.1002/mus.28198","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with \"supportive\" evidence of CIDP on cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), ultrasound (US), or nerve biopsy but not on NCS, often receive immunomodulating therapy. We evaluated the treatment response of patients with clinical and supportive features of CIDP lacking NCS evidence of demyelination.</p><p><strong>Methods: </strong>Retrospective chart review was conducted on 232 patients who met CIDP clinical criteria and were treated with disease-modifying therapy. Patients included did not have NCS criteria of demyelination, but did have supportive CSF, MRI, or US findings consistent with CIDP. A positive treatment response was defined as at least a one-point improvement in the modified Rankin scale (mRS), or a four-point increase in the Medical Research Council sum score (MRCSS).</p><p><strong>Results: </strong>Twenty patients met criteria: 17 of the 18 (94%) patients with CSF protein >45 mg/dL, 6 of the 14 (43%) with MRI lumbosacral root or plexus enhancement, and 4 of the 6 (67%) with enlarged proximal nerves on US. Eighteen patients received intravenous immunoglobulin, 10 corticosteroids, one plasma exchange, and six other immunomodulatory therapies. Twelve patients had a positive treatment response on the MRCSS or mRS. The presence of MRI lumbosacral root or plexus enhancement was associated with a positive treatment response.</p><p><strong>Discussion: </strong>A trial of immunomodulating treatment should be considered for patients with clinical features of CIDP in the absence of NCS evidence of demyelination, particularly when there is MRI lumbosacral root or plexus enhancement.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492707","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}
引用次数: 0
Skeletal muscle symptoms and quantitative MRI in females with dystrophinopathy. 患有肌营养不良症的女性的骨骼肌症状和定量核磁共振成像。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-09-02 DOI: 10.1002/mus.28235
Breana M Jenkins, Lathan D Dixon, Kevin J Kokesh, Carla D Zingariello, Krista Vandenborne, Glenn A Walter, Alison M Barnard
{"title":"Skeletal muscle symptoms and quantitative MRI in females with dystrophinopathy.","authors":"Breana M Jenkins, Lathan D Dixon, Kevin J Kokesh, Carla D Zingariello, Krista Vandenborne, Glenn A Walter, Alison M Barnard","doi":"10.1002/mus.28235","DOIUrl":"10.1002/mus.28235","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The dystrophinopathies primarily affect males; however, female carriers of pathogenic dystrophin variants can develop skeletal muscle symptoms. This study aimed to evaluate muscle involvement and symptoms in females with dystrophinopathy using quantitative magnetic resonance imaging (MRI), functional assessments, and patient-reported outcomes.</p><p><strong>Methods: </strong>Controls and females with dystrophinopathy with muscle symptoms of pain, weakness, fatigue, or excessive tightness were enrolled in this cross-sectional study. Participants underwent lower extremity MRI to quantify muscle inflammation, replacement by fat, and disease asymmetry. Cardiac MRI, functional ability, muscle symptoms, and serum creatine kinase levels were also evaluated.</p><p><strong>Results: </strong>Six pediatric females with dystrophinopathy (mean age: 11.7 years), 11 adult females with dystrophinopathy (mean age: 41.3 years), and seven controls enrolled. The mean fat fraction was increased in females with dystrophinopathy compared to controls in the soleus (0.11 vs. 0.03, p = .0272) and vastus lateralis (0.16 vs. 0.03, p = .004). Magnetic resonance spectroscopy water T<sub>2</sub>, indicative of muscle inflammation, was elevated in the soleus and/or vastus lateralis in 11 of 17 individuals. North Star Ambulatory Assessment score was lower in the dystrophinopathy group compared to controls (29 vs. 34 points, p = .0428). From cardiac MRI, left ventricle T<sub>1</sub> relaxation times were elevated in females with dystrophinopathy compared to controls (1311 ± 55 vs. 1263 ± 25 ms, p < .05), but ejection fraction and circumferential strain did not differ.</p><p><strong>Discussion: </strong>Symptomatic females with dystrophinopathy quantitatively demonstrate muscle replacement by fat and inflammation, along with impairments in functional ability and cardiac function. Additional research is needed to evaluate how symptoms and muscle involvement change longitudinally.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11493146/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142109671","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}
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