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Brachio-cervical inflammatory myopathy associated with systemic sclerosis. Case series and review of literature
This study was aimed at describing a case series of brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc), due to its rarity and limited coverage in published data. Another aim was to provide a literature review. We reported four cases of BCIM-SSc from our tertiary center. In addition, we researched the literature and found six articles featuring 17 patients who fit this phenotype. We pooled all cases and reported their features. Most patients were female and had limited SSc, and the median time of BCIM presentation was three years after SSc diagnosis. Asymmetric muscle involvement, scapula...
Source: Reumatismo - August 3, 2021 Category: Rheumatology Authors: C S R Araujo R Miossi F H C De Souza M D Costa A M S Da Silva E D Campos E Zanoteli S K Shinjo Source Type: research
A Muscle Biosignature Differentiating Between Limb-Girdle Muscular Dystrophy and Idiopathic Inflammatory Myopathy on Magnetic Resonance Imaging
Conclusions: MRI could be a valuable tool to differentiate LGMD from IIM based on the discrepancy in muscle fat substitution, and the adductor magnus muscle could provide a biosignature to categorizing LGMD.
Source: Frontiers in Neurology - December 20, 2021 Category: Neurology Source Type: research
The Resting-State Brain Network Functional Connectivity Changes in Patients With Acute Thyrotoxic Myopathy Based on Independent Component Analysis
ConclusionThe bulbar paralysis (such as dysphagia, dysarthria) in ATM patients may be related to the functional connectivity changes of resting-state SMN and LFPN. The fMRI is expected to be one of the objective imaging indicators for the early clinical intervention of ATM patients.
Source: Frontiers in Endocrinology - March 24, 2022 Category: Endocrinology Source Type: research
Anti-signal Recognition Particle Antibody-positive Immune-mediated Myopathy after mRNA-1273 SARS-CoV-2 Vaccination
Intern Med. 2022 Sep 13. doi: 10.2169/internalmedicine.0404-22. Online ahead of print.ABSTRACTA 26-year-old Japanese woman developed a fever, myalgia and gait disturbance one day after receiving the second dose of the mRNA-1273 severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. A neurological examination revealed symmetrical weakness and myalgia in proximal lower limbs, and a blood examination showed prominent elevation of creatinine kinase. Magnetic resonance imaging (MRI) revealed a high signal intensity in the thigh muscles on short-tau inversion recovery images, and antibody testing revealed positive...
Source: Internal Medicine - September 14, 2022 Category: Internal Medicine Authors: Masanobu Tanemoto Ryosuke Oda Yuichiro Toyama Hirohiko Shizukawa Kazuki Yokokawa Taro Saito Syuuichirou Suzuki Akihiro Matsumura Shin Hisahara Source Type: research
Anti-SRP immune-mediated necrotizing myopathy: A critical review of current concepts
Purpose of reviewThis review aims to describe clinical and histological features, treatment, and prognosis in patients with anti-signal recognition particle (SRP) autoantibodies positive immune-mediated necrotizing myopathy (SRP-IMNM) based on previous findings.Previous findingsAnti-SRP autoantibodies are specific in IMNM. Humoral autoimmune and inflammatory responses are the main autoimmune characteristics of SRP-IMNM. SRP-IMNM is clinically characterized by acute or subacute, moderately severe, symmetrical proximal weakness. Younger patients with SRP-IMNM tend to have more severe clinical symptoms. Patients with SRP-IMNM...
Source: Frontiers in Immunology - October 13, 2022 Category: Allergy & Immunology Source Type: research
Assessing atrial myopathy with cardiac magnetic resonance imaging in embolic stroke of undetermined source
Left atrial myopathy has been implicated in atrial fibrillation (AF)-related stroke and embolic stroke of undetermined source (ESUS).
Source: International Journal of Cardiology - July 25, 2023 Category: Cardiology Authors: Stavroula Papapostolou, John Kearns, Benedict T. Costello, Jessica O'Brien, Murray Rudman, Mark C. Thompson, Geoffrey Cloud, Dion Stub, Andrew J. Taylor Source Type: research
PD-1 Inhibitor-associated Myopathies: Emerging Immune-mediated Myopathies
Programmed death-1 (PD-1) inhibitors are increasingly used in cancer immunotherapy. Various immune-related adverse events are reported, including infrequent individual case reports of myositis or rhabdomyolysis. The frequency and diagnostic spectrum of immune-related adverse events affecting skeletal muscle in PD-1 inhibitor-treated patients are unknown. We searched the Mayo Clinic Pharmacy database (2014–2016) to identify patients who developed myopathies during or after PD-1 inhibitor therapy. Among 654 cancer patients received PD-1 inhibitors (pembrolizumab=389; nivolumab=264; both=1), we identified 5 patients (pembro...
Source: Journal of Immunotherapy - April 21, 2018 Category: Allergy & Immunology Tags: Clinical Studies Source Type: research
Limb Girdle Muscular Dystrophy due to Digenic Inheritance of < b > < i > DES < /i > < /b > and < b > < i > CAPN3 < /i > < /b > Mutations
We report the clinical and genetic analysis of a 63-year-old man with progressive weakness developing over more than 20 years. Prior to his initial visit, he underwent multiple neurological and rheumatological evaluations and was treated for possible inflammatory myopathy. He did not respond to any treatment that was prescribed and was referred to our center for another opinion. He underwent a neurological evaluation, electromyography, magnetic resonance imaging of his legs, and a muscle biopsy. All testing indicated a chronic myopathy without inflammatory features suggesting a genetic myopathy. Whole-exome sequencing test...
Source: Case Reports in Neurology - September 18, 2018 Category: Neurology Source Type: research
Dynamic derangement in amino acid profile during and after a stroke-like episode in adult-onset mitochondrial disease: a case report
ConclusionsGrowth differentiation factor-15 can be used not only for the diagnosis of mitochondrial disease, but as an indicator of its acute exacerbation. A stroke-like episode of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes reflects a drastic derangement of multiple amino acids. The involvement of aspartic acid in the episodes should be explored in future studies.
Source: Journal of Medical Case Reports - October 20, 2019 Category: General Medicine Source Type: research
The spectrum of muscle histopathologic findings in 42 weak scleroderma patients
Conclusion: Non‐specific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histological evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of myopathy in scleroderma. This article is protected by copyright. All rights reserved.
Source: Arthritis Care and Research - May 18, 2015 Category: Rheumatology Authors: Julie J. Paik, Fredrick M. Wigley, Thomas E. Lloyd, Andrea M. Corse, Livia Casciola‐Rosen, Ami A. Shah, Francesco Boin, Laura K. Hummers, Andrew L. Mammen Tags: Original Article Source Type: research
Spectrum of Muscle Histopathologic Findings in Forty‐Two Scleroderma Patients With Weakness
ConclusionNonspecific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histologic evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of myopathy in scleroderma.
Source: Arthritis Care and Research - September 22, 2015 Category: Rheumatology Authors: Julie J. Paik, Fredrick M. Wigley, Thomas E. Lloyd, Andrea M. Corse, Livia Casciola‐Rosen, Ami A. Shah, Francesco Boin, Laura K. Hummers, Andrew L. Mammen Tags: Systemic Sclerosis Source Type: research
