Muscle & Nerve最新文献

筛选
英文 中文
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}
引用次数: 0
Ultra high-frequency ultrasound of fascicles in the ulnar and radial nerves. 尺神经和桡神经束的超高频超声。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-05-06 DOI: 10.1002/mus.28097
James B Meiling, Rachana K Gandhi Mehta, Vanessa Baute Penry, Michael S Cartwright, Marisa Barclay McGhee
{"title":"Ultra high-frequency ultrasound of fascicles in the ulnar and radial nerves.","authors":"James B Meiling, Rachana K Gandhi Mehta, Vanessa Baute Penry, Michael S Cartwright, Marisa Barclay McGhee","doi":"10.1002/mus.28097","DOIUrl":"10.1002/mus.28097","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Ultra high-frequency ultrasound (UHFUS) has been demonstrated to allow easy visualization and quantification of median and digital nerve fascicles; however, there is a lack of normative data for other upper limb nerves. The purpose of this study was to use UHFUS to establish normative reference values and ranges for fascicle count and density within selected upper extremity nerves.</p><p><strong>Methods: </strong>Twenty-one healthy volunteers underwent sonographic examination of the ulnar, superficial branch of the radial, and radial nerves on one upper limb using UHFUS with a 48 MHz linear transducer. The number of fascicles in each peripheral nerve and fascicle density were assessed.</p><p><strong>Results: </strong>The mean fascicle number and fascicle density for each of the measured nerves was ulnar nerve at the wrist 11.7 and 2.0, ulnar nerve at the elbow 9.2 and 1.1, superficial branch of the radial nerve 7.3 and 2.5, and radial nerve at the spiral groove 4.2 and 0.8. A single significant association was observed between CSA and fascicle number in the ulnar nerve at the wrist (p = .023, r = 0.66). Neither fascicle number nor density could be predicted by age, sex, height, weight, or body mass index.</p><p><strong>Discussion: </strong>UHFUS may help to establish a baseline of normative data on upper limb nerves that are not frequently biopsied due to their mixed motor and sensory functions and has the potential for increased understanding of nerve fascicular anatomy to improve diagnostic accuracy of focal nerve lesions, particularly those with selective fascicular involvement.</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":"140857224","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
Safety of risdiplam in spinal muscular atrophy patients after short-term treatment with nusinersen. 短期使用纽西奈森治疗脊髓性肌萎缩症患者后,利斯地平的安全性。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-08-13 DOI: 10.1002/mus.28228
Yue Yan, Yijie Feng, Liya Jiang, Jianing Jin, Shanshan Mao
{"title":"Safety of risdiplam in spinal muscular atrophy patients after short-term treatment with nusinersen.","authors":"Yue Yan, Yijie Feng, Liya Jiang, Jianing Jin, Shanshan Mao","doi":"10.1002/mus.28228","DOIUrl":"10.1002/mus.28228","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Following the approval of risdiplam, there are more possibilities for disease-modifying therapy (DMT) in children with spinal muscular atrophy (SMA). Non-treatment-naïve subjects with SMA involved in the JEWELFISH study, designed to evaluate the safety and tolerability of risdiplam, were required to undergo a washout period before receiving risdiplam. This study aims to investigate the safety of administering risdiplam in patients within 90 days of receiving treatment with nusinersen.</p><p><strong>Methods: </strong>Data were collected on SMA patients who had undergone treatment with nusinersen, and who then received risdiplam within 90 days of their last dose of nusinersen, including demographic characteristics, information on treatment with nusinersen and risdiplam, adverse events, and laboratory assessments in a follow-up period of 90 days, presented as median (range).</p><p><strong>Results: </strong>A total of 15 children with SMA were reported, including 8 males and 7 females. The median number of doses of previous nusinersen treatment received was 8 (6-17) doses, and the median age at first risdiplam treatment was 4.3 (1.9-11.2) years. Specifically, 8 children received risdiplam 30 days or less after their most recent nusinersen treatment, 2 at 31-60 days after nusinersen, and 5 at 61-89 days post-nusinersen. Adverse events of pyrexia, pneumonia, vomiting and rash were reported in 4 patients.</p><p><strong>Discussion: </strong>Our study showed good safety data on patients who received risdiplam following nusinersen within the washout period of 90 days. This supplements the JEWELFISH study in the era of DMT, providing additional guidance for clinicians, but additional data from other centers is needed.</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":"141971485","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
Role of prazosin in patients with Guillain-Barré syndrome with sympathetic overactivity: A cohort study. 哌唑嗪在伴有交感神经过度活动的格林-巴利综合征患者中的作用:一项队列研究。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-08-22 DOI: 10.1002/mus.28236
Mritunjai Kumar, Abhishek Guin, Anu Singh, Rajni Singh, Ashutosh Tiwari
{"title":"Role of prazosin in patients with Guillain-Barré syndrome with sympathetic overactivity: A cohort study.","authors":"Mritunjai Kumar, Abhishek Guin, Anu Singh, Rajni Singh, Ashutosh Tiwari","doi":"10.1002/mus.28236","DOIUrl":"10.1002/mus.28236","url":null,"abstract":"<p><strong>Introduction/aims: </strong>In Guillain-Barré syndrome (GBS), patients with dysautonomia demonstrate sympathetic overactivity (SO). This study assessed the role of prazosin (α<sub>1</sub>-blocker) in the management of SO.</p><p><strong>Methods: </strong>This cohort study was conducted from January 2022 to September 2023. Thirty-two GBS patients with SO received prazosin (2.5-10 mg three times a day) (prazosin group). For comparison, we included historical controls that included 33 GBS patients having SO with similar baseline characteristics, including median age and disability, who did not receive prazosin, from a GBS registry of patients admitted during February 2018-December 2021. The primary endpoint was days to resolution of SO. Secondary endpoints were daily fluctuations in the systolic (SBP) and diastolic blood pressure (DBP), duration of hospital stay, in-hospital mortality, and disability at 3 months.</p><p><strong>Results: </strong>The median ages of both the treatment and the control groups were 36 (IQR 25-49) years and 43 (66.2%) were males. The demographic and clinical parameters were comparable. Prazosin resulted in significantly earlier normalization of SO compared to the control group (median 15 vs. 20 days; p = .01). The mean fluctuations in the SBP and DBP at 15 days were significantly lower in the prazosin group. However, the duration of hospital stay and good recovery at 3 months were comparable. Three patients developed hypotension, while two patients died (ventilator-associated pneumonia) in the prazosin group.</p><p><strong>Discussion: </strong>This study provides new evidence supporting the role of prazosin in SO, and needs randomized trials to confirm our findings.</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":"142036352","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 other face of facioscapulohumeral muscular dystrophy: Exploring orofacial weakness using muscle ultrasound. 面囊肱肌营养不良症的另一面:利用肌肉超声波探究口面部肌无力。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2024-11-01 Epub Date: 2024-09-19 DOI: 10.1002/mus.28254
Sanne C C Vincenten, Jeroen L M van Doorn, Sjan Teeselink, Nathaniel B Rasing, George W Padberg, Nicol C Voermans, Baziel G M van Engelen, Nens van Alfen, Karlien Mul
{"title":"The other face of facioscapulohumeral muscular dystrophy: Exploring orofacial weakness using muscle ultrasound.","authors":"Sanne C C Vincenten, Jeroen L M van Doorn, Sjan Teeselink, Nathaniel B Rasing, George W Padberg, Nicol C Voermans, Baziel G M van Engelen, Nens van Alfen, Karlien Mul","doi":"10.1002/mus.28254","DOIUrl":"10.1002/mus.28254","url":null,"abstract":"<p><strong>Introduction/aims: </strong>One of the most distinct clinical features of facioscapulohumeral muscular dystrophy (FSHD) is facial weakness. It leads to diminished facial expression and functional impairments. Despite its clinical relevance, little else is known about orofacial muscle involvement. We therefore evaluated orofacial muscle involvement in a sizeable cohort of FSHD participants with muscle ultrasound.</p><p><strong>Methods: </strong>Muscle ultrasound images of the following orofacial muscles were scored visually and quantitatively: depressor anguli oris (DAO), orbicularis oris (OO), buccinator, temporalis, masseter, digastric, zygomaticus major and minor bilaterally, and the geniohyoid. Reliability analyses of both visual and quantitative evaluations were performed. Ultrasound results were correlated with other measures: the FSHD clinical score, facial weakness score, and facial function scale.</p><p><strong>Results: </strong>We included 107 FSHD participants (male 54%; age 52 ± 14 years), of whom 92% showed signs of facial weakness. The reliability of visual ultrasound analysis varied widely (κ 0.0-1.0). Quantitative ultrasound reliability was high (intraclass correlation analysis ≥ 0.96). The DAO, buccinator, OO, temporalis, and zygomaticus minor muscles were affected most often (15%-39%). The digastric, geniohyoid, zygomaticus major, and masseter muscles were least often affected (<5%). The ultrasound compound score correlated weakly to moderately with other outcome measures used (ρ = 0.3-0.7).</p><p><strong>Discussion: </strong>This study adds to the understanding of orofacial weakness in FSHD, confirming the involvement of the muscles of facial expression in FSHD using ultrasound. We showed that orofacial muscle ultrasound is feasible and reliable when quantitatively assessed. Future studies should evaluate orofacial muscle ultrasound longitudinally, alongside clinical and patient-reported facial weakness outcome measures, to assess their potential as outcome measures.</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":"142291681","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
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信