Muscle Involvement Research Articles

Tubercular pyomyositis: A case series

Tuberculosis is one of the endemic diseases in India. Tuberculosis can involve almost every organ of the body, however isolated muscle involvement is considered rare. We hereby report a series of three cases of tubercular pyomyositis to highlight the importance of high clinical suspicion in endemic countries like India, in both immunocompetent and immunocompromised individuals, presenting with persistent fever and musculoskeletal symptoms. A timely diagnosis with effective drainage and antitubercular therapy helps in good functional recovery.

Subclinical Ocular Motility Dysfunction and Extraocular Muscle Changes in Inactive Graves' Orbitopathy.

This study aimed to investigate the presence of structural and functional changes in extraocular muscles (EMs) among patients with inactive Graves' orbitopathy (GO) classified according to the Clinical Activity Score (CAS). Sixty-seven patients with Graves' disease (GD) and inactive GO were included. The data collected included clinical parameters, thyroid function, autoantibody levels, EOM morphology via orbital ultrasound (US), and ocular motility. Patients were stratified into Red Filter Test (RFT)-positive or RFT-negative groups based on the presence or absence of latent diplopia during the RFT examination. Thirty-three patients (49.25%) exhibited latent diplopia on the RFT, despite not reporting double vision during standard ocular motility tests. Significant differences were observed between the two groups in terms of age, disease duration, intraocular pressure (IOP) elevation in up-gaze, and medial rectus muscle thickness (p < 0.05). No significant differences were found in thyroid status, TRAb and ATA levels, CASs, exophthalmos, or lateral rectus thickness between the two groups. This study revealed that in inactive GO, subclinical EM dysfunction and morphological changes may be present, which might not be apparent through routine ocular examinations. The RFT is effective in detecting latent diplopia, highlighting its utility in identifying subtle ocular motility issues and subclinical muscle involvement. Comprehensive evaluations combining functional tests like the RFT and imaging are essential for early detection of GO-related abnormalities, enabling tailored and prompt management and improving patient outcomes.

Peripheral nervous system immune-related adverse events due to checkpoint inhibition.

Immune checkpoint inhibitors have revolutionized cancer therapy and are increasingly used to treat a wide range of oncological conditions, with dramatic benefits for many patients. Unfortunately, the resulting increase in T cell effector function often results in immune-related adverse events (irAEs), which can involve any organ system, including the central nervous system (CNS) and peripheral nervous system (PNS). Neurological irAEs involve the PNS in two-thirds of affected patients. Muscle involvement (immune-related myopathy) is the most common PNS irAE and can be associated with neuromuscular junction involvement. Immune-related peripheral neuropathy most commonly takes the form of polyradiculoneuropathy or cranial neuropathies. Immune-related myopathy (with or without neuromuscular junction involvement) often occurs along with immune-related myocarditis, and this overlap syndrome is associated with substantially increased mortality. This Review focuses on PNS adverse events associated with immune checkpoint inhibition. Underlying pathophysiological mechanisms are discussed, including antigen homology between self and tumour, epitope spreading and activation of pre-existing autoreactive T cells. An overview of current approaches to clinical management is provided, including cytokine-directed therapies that aim to decouple anticancer immunity from autoimmunity and emerging treatments for patients with severe (life-threatening) presentations.

Neuromuscular conditions in post-stroke ankle-foot dysfunction reflected by surface electromyography

BackgroundRating scales and linear indices of surface electromyography (sEMG) cannot quantify all neuromuscular conditions associated with ankle-foot dysfunction in hemiplegic patients. This study aimed to reveal potential neuromuscular conditions of ankle-foot dysfunction in hemiplegic patients by nonlinear network indices of sEMG.MethodsFourteen male patients with hemiplegia and 10 age- and sex-matched healthy male adults were recruited and tested in static standing position. The characteristics of the root mean square (RMS), median frequency (MF), and three nonlinear indices, the clustering coefficient (C), the average shortest path length (L), and the degree centrality (DC), of eight groups of muscles in bilateral calves were observed.ResultsCompared to those of the control group, the RMS of the medial gastrocnemius (MG), flexor digitorum longus (FDL), and extensor digitorum longus (EDL) on the affected side were significantly lower (P < 0.05), and the RMS of the tibial anterior (TA) and EDL on the unaffected side were significantly higher (P < 0.05). The MF of the EDL on the affected side was significantly higher than that on the control side (P < 0.05). The C of the unaffected side was significantly higher than that of the control group, whereas the L was lower (P < 0.05). Compared to those of the control group, the DC of the TA, EDL, and soleus (SOL) on the unaffected sides were higher (P < 0.05), and the DC of the MG on the affected sides was lower (P < 0.05).ConclusionThe change trends and clinical significance of these three network indices, including C, L, and DC, are not in line with those of the traditional linear indices, the RMS and the MF. The C and L may reflect the degree of synchronous activation of muscles during a certain motor task. The DC might be able to quantitatively assess the degree of muscle involvement and reflect the degree of involvement of a single muscle. Linear and nonlinear indices may reveal more neuromuscular conditions in hemiplegic ankle-foot dysfunction from different aspects.Trial registrationChiCTR2100055090.

A head-to-head comparison of 18F-FDG PET/CT and 18F-FDG PET/MR in patients with nasopharyngeal carcinoma under different disease settings.

Positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) are complementary in staging of nasopharyngeal carcinoma (NPC). The combination of MRI and functional imaging from PET in PET/MR is promising in NPC management. Diagnostic performance of PET/CT and PET/MR was compared in 46 patients with histologically confirmed NPC under different disease scenarios, including primary non-metastatic cases, primary metastatic cases, recurrence and/or metastasis after treatment, and post-treatment follow-up cases. Forty-six patients underwent both PET/CT and PET/MR in the same day. Primary tumor extension into risk-stratified anatomic structures, retropharyngeal and cervical lymph node metastasis, distant metastasis and post-treatment follow-up results, were compared. For high-risk structures, PET/MR detected two more sides of tensor/levator veli palatine muscle involvement, one more case of clivus involvement, and ruled out 12 false-positive sides of prevertebral muscle involvement by PET/CT. For medium-risk structures, PET/MR detected four more sides of medial pterygoid muscle involvement. For low-risk structures, abnormal signal on massa lateralis atlantis was detected by PET/MR. PET/MR detected 14 more positive retropharyngeal lymph nodes and more liver micrometastases than PET/CT. Overall, PET/MR changed two patients' T staging. Positron emission tomography/MR outperforms PET/CT in delineating muscle, skull-base bone, and nodal involvement, and identifying liver micrometastases, may serve as a single-step staging modality for NPC.

Functional involvement of the sternohyoid muscle during breathing and swallowing in rats.

The sternohyoid muscle depresses the hyoid bone, but it is unclear whether the muscle contributes to respiratory and swallowing mechanisms. This study aimed to clarify whether the sternohyoid muscle participates in the respiration and swallowing reflex and how the activity is modulated in two conditions: with airway stenosis and with a fixed sternohyoid muscle length. Electromyographic activity in the sternohyoid, digastric, thyrohyoid, and diaphragm muscles was recorded in anesthetized rats. The sternohyoid muscle activity was observed in the inspiratory phase and during swallowing, and was well coordinated with digastric and thyrohyoid muscle activity. With airway stenosis, the respiratory activity per respiratory cycle was facilitated in all assessed muscles but the facilitation of activity per second occurred only in the digastric, thyrohyoid, and sternohyoid muscles. With airway stenosis, the swallowing activity was facilitated only in the digastric muscle but not in the thyrohyoid and sternohyoid muscles. Swallowing activity was not observed in the sternohyoid muscle in the condition with the sternohyoid muscle length fixed, although increased inspiratory activity remained. The current results suggest that 1) the sternohyoid muscle is slightly activated in the inspiratory phase, 2) the effect of airway stenosis on respiratory function may differ between the upper airway muscles and diaphragm, and 3) swallowing activity in the sternohyoid muscle is not dominantly controlled by the swallowing central pattern generator but instead occurs as a myotatic reflex.NEW & NOTEWORTHY We found that the sternohyoid muscle was activated in the inspiratory phase. However, increased airway resistance had different effects on the extrathoracic muscles than on the diaphragm. The swallowing activity of the sternohyoid disappeared when the muscle length was fixed. These findings suggest that the sternohyoid muscle may be activated not by the swallowing central pattern generator but as a myotatic reflex.

Diagnosis and management of a case of gestational trophoblastic neoplasia with lumbosacral metastases.

Gestational trophoblastic neoplasia (GTN) with spinal metastasis is rare with few documented cases worldwide. Few studies have explored chemotherapy combined with radiotherapy in the treatment of such cases. However, because of its rarity, there is still no standardized treatment regimen. A 34-year-old Gravida 1 Para 0 (0010) was diagnosed with GTN with metastasis to the lumbosacral spine, resulting in conus medullaris syndrome with lumbar radiculopathy. She presented with a 14-month history of amenorrhea, left lower extremity pain, and urinary and bowel retention. On examination, there was a 10.0 × 7.0 cm lumbosacral mass and atrophy of the left lower extremity. Transvaginal ultrasound showed a cul de sac mass, and diluted β-human chorionic gonadotropin (β-hCG) titer was markedly elevated at more than 1000 000 mIU/mL. Magnetic resonance imaging (MRI) of the lumbosacral spine showed an ill-defined sacral mass measuring 13.3 × 11.5 × 6.3 cm with spinal canal, bone, muscle, and nerve root involvement. She was treated with 10 cycles of EMACO and palliative radiotherapy with 10 sessions of 30 Gy of external beam radiation therapy directed toward the lumbosacral mass. Repeat MRI showed a decrease in size of the mass to 6.6 × 8.2 × 4.1 cm with concurrent decrease in β-hCG to 1.30 mIU/ml, and resolution of leg pain and urinary and bowel symptoms. She was declared to be in remission 3 months after the last cycle of EMACO.

ETV6 Molecular Heterogeneity in Salivary Secretory Carcinoma: A Case Series Report and Literature Review

BackgroundETV6 gene rearrangement is the molecular hallmark of secretory carcinoma (SC), however; the nature, frequency, and clinical implications of atypical ETV6 signal patterns by fluorescence in situ hybridization (FISH) has not yet been systematically evaluated in salivary gland neoplasms.MethodsThe clinical, histopathologic, immunohistochemical and molecular features of seven salivary SCs, including four cases with atypical ETV6 FISH patterns, were retrospectively analyzed along with a critical appraisal of the literature on unbalanced ETV6 break-apart in SCs.ResultsThe patients were four males and three females (31–70 years-old). Five presented with a painless neck mass and two patients with recurrent disease had a history of a previously diagnosed acinic cell carcinoma of the buccal mucosa. Histologically, there were varied combinations of microcystic, papillary, tubular, and solid patterns. All tumors were diffusely positive for S100 and/or SOX10, while 2 cases also showed luminal DOG1 staining. Rearrangement of the ETV6 locus was confirmed in 5/7 cases, of which 3 cases showed classic break-apart signals, 1 case further demonstrated duplication of the ETV6 5`end and the other loss of one copy of ETV6. Two cases harbored ETV6 deletion without rearrangement. Two of the 4 cases with atypical ETV6 FISH patterns represented recurrent tumors, one with widespread skeletal muscle involvement, bone and lymphovascular invasion. Surgical treatment resulted in gross-total resection in all 7 cases, with a median follow up of 9.5 months post-surgery for primary (n = 3) and recurrent disease (n = 1).ConclusionDuplication of the distal/telomeric ETV6 probe represented the most common (26/40; 65%) variant ETV6 break-apart FISH pattern in salivary SC reported in the literature and appears indicative of an aggressive clinical course.

Comparative analysis of elevated and floor push-up exercises for activation of the pectoralis major muscle

This study aimed to measure muscle activation by comparing electromyography (EMG) of two types of pushups i.e., ground-based and elevated. 20 male active sportspersons (age 20.12±3.57years, weight 65.38±9.32kg, height 175.5±10.5cm) were recruited in the study. The upper body muscle pectoralis major (PM) was chosen as target muscle for the study. Root mean square (RMS) of EMG activities were analysed for the PM muscle to determine maximum muscle involvement. In the elevated pushups, the samples performed the pushups on an elevated pushup bar of 18-centimeter height, in which they gripped the bar as per their shoulder width. Also, their feet were ground-based on a similar height platform. The paired t-test was used as the statistical tool to compare the EMG activation of PM between both variations of pushups. Further, mean and standard deviation values were considered for descriptive statistics. The level of significance was set at 0.05. The result of the study showed that there was no statistically significant difference in EMG activities of PM muscle between the two pushup variations with a t-value of -0.89. However, the descriptive statistics showed that the mean value of elevated pushups (RMS, 350.63) was higher than the grounded pushups (RMS, 311.44). These findings suggest elevated pushups may induce greater PM muscle activation, potentially due to increased instability due to the influence of elveated exercise variations on muscle engagement. Key words: pushups, muscle activation, electromyography, pectoralis major, exercise variations, root mean square

Enhancing Radiological Reporting in Head and Neck Cancer: Converting Free-Text CT Scan Reports to Structured Reports Using Large Language Models

Objective The aim of this study was to assess efficacy of large language models (LLMs) for converting free-text computed tomography (CT) scan reports of head and neck cancer (HNCa) patients into a structured format using a predefined template. Materials and Methods A retrospective study was conducted using 150 CT reports of HNCa patients. A comprehensive structured reporting template for HNCa CT scans was developed, and the Generative Pre-trained Transformer 4 (GPT-4) was initially used to convert 50 CT reports into a structured format using this template. The generated structured reports were then evaluated by a radiologist for instances of missing or misinterpreted information and any erroneous additional details added by GPT-4. Following this assessment, the template was refined for improved accuracy. This revised template was then used for conversion of 100 other HNCa CT reports into structured format using GPT-4. These reports were then reevaluated in the same manner. Results Initially, GPT-4 successfully converted all 50 free-text reports into structured reports. However, there were 10 places with missing information: tracheostomy tube (n = 3), noninclusion of involvement of sternocleidomastoid muscle (n = 2), extranodal tumor extension (n = 3), and contiguous involvement of the neck structures by nodal mass rather than the primary (n = 2). Few instances of nonsuspicious lung nodules were misinterpreted as metastases (n = 2). GPT-4 did not indicate any erroneous additional findings. Using the revised reporting template, GPT-4 converted all the 100 CT reports into a structured format with no repeated or additional mistakes. Conclusion LLMs can be used for structuring free-text radiology reports using plain language prompts and a simple yet comprehensive reporting template. Summary Statement Large language models can successfully and accurately convert conventional radiology reports for oncology scans into a structured format using a comprehensive predefined template and thus can enhance the utility and integration of these reports in routine clinical practice. Key Points

Neuromyelitis optica spectrum disorder: Exploring the diverse clinical manifestations and the need for further exploration.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder characterized by inflammatory assaults on the central nervous system (CNS), particularly on the optic nerves and spinal cord. In recent years, a wider range of clinical manifestations of this complex disease have been observed, emphasizing the importance of gaining a more profound understanding beyond optic neuritis (ON) and transverse myelitis (TM). This study explores the many clinical symptoms of NMOSD, including common and uncommon presentations. Distinctive aspects of ON, TM, and diencephalic/brainstem syndromes are examined, highlighting their unique characteristics in contrast to conditions such as multiple sclerosis. We also discuss extra-CNS involvement, such as unusual signs, including muscle involvement, retinal injury, auditory impairment, and rhinological symptoms. Our study intends to highlight the wide range and complexity of NMOSD presentations, emphasizing the significance of identifying unusual symptoms for precise diagnosis and prompt management. The specific processes that contribute to the varied clinical presentation of NMOSD are not well understood despite existing information. This emphasizes the necessity for more study to clarify the mechanisms that cause different symptoms and discover new treatment targets for this complex autoimmune disorder. It is essential to acknowledge the complex and varied clinical manifestations of NMOSD to enhance diagnosis, treatment, and patient results. By enhancing our comprehension of the fundamental processes and investigating innovative therapeutic approaches, we may aim to enhance the quality of life for persons impacted by this illness.

Current status of pre- and retropectoral breast reconstructions worldwide: a narrative review.

Advances in breast cancer research and technology contribute to conservative ablative surgical approach with emphasis on reconstruction. The introduction of biologic membranes in breast surgery facilitates a one-stage implant reconstruction while the importance of the pectoralis major muscle involvement in the procedure becomes debatable. A subsequent increase in prepectoral implant placement procedures seems to close a cycle of innovations in implant-based breast reconstructions. This sparks a debate that calls for a critical review of existing literature considering that new challenges tend to arise along with new perspectives. The authors seek to scope the present status of prepectoral and subpectoral implant reconstruction worldwide, and answer recurring questions, including the novelty of presented innovations in the context of existing literature. The article is based on a literature search in PubMed with the keywords "prepectoral" or "subpectoral" and "breast reconstruction", in addition to the authors' experience with a large number of patients. Recent studies focus on the comparative safety of prepectoral vs. subpectoral placement debating the use of biologic vs. non-biologic sheets and implant texture. There seems to be more emphasis on early post-operative safety of the procedures, rather than any long-term prospects of their comparison, up to this point. Skin and nipple sparing mastectomy (SSM/NSM) together with biological membranes have played a key role in current practice and cannot be overlooked. After reading this paper, the reader should have a firm understanding of the key elements of implant-based breast reconstruction in historical context with emphasis on muscle planes and their pros and cons.

Assessment of clinical and histopathological characteristics in COVID-19-associated mucormycosis (CAM) patients correlating with outcome: A hospital-based cross-sectional study.

The second wave of the COVID-19 pandemic led to a very dreaded complication of mucormycosis. Immunosuppressive action of the COVID-19 virus, co-morbidities, for example, diabetes mellitus (DM), hypertension, use of steroids, and humidified oxygen, are among the important factors that make the patients susceptible to developing mucormycosis. The present study was conducted to identify and understand all the significant histological changes including the type and extent of tissue involvement, the pattern of inflammation, the volume of fungal hyphae, hemorrhage, etc., in patients with COVID-19 associated mucormycosis (CAM) and correlate with clinical outcome. It was a retrospective cross-sectional observational study involving all the patients of CAM, who underwent debridement or biopsy over a period of 5 months, from April 01, 2021, to August 31, 2021. CAM was classified based on the radiological evaluation, clinical features, and organs involved. Different demographic, clinical, laboratory, and histologic parameters were recorded. The variables were assessed for their association with poor clinical outcomes using multiple logistic regression. P < 0.05 was considered statistically significant. A total of 146 patients were included in the study with a mean age of 49.4 years and 71.2% were male. Sino-naso-palatal was the most common type of CAM (32.9%), while sino-naso-cerebral was the least common (14.3%). DM was present in 54.1% of patients, out of which 26.6% were recently diagnosed. The death occurred in 21.9% of patients. Maximum mortality was observed in CAM of sino-naso-cerebral involvement (42.9%). Total leucocyte count (TLC) [OR = 0.87; 95%CI: 0.76-0.97; P = 0.02] and C-reactive protein (CRP) [OR = 0.97; 95%CI: 0.96-0.99; P = 0.008] were significantly associated with poor outcomes. Other factors, that is, high prothrombin time, DM, ferritin, and the involvement of muscle, skin, and cartilage, were also associated with poor clinical outcomes but were not statistically significant. Similarly, high fungal volume and the presence of thrombosis were also associated with poor outcomes but were not statistically significant. CAM more commonly affects males with co-morbidities. TLC and CRP were significantly associated with poor outcomes. Histologically, the involvement of skin, muscle, and cartilage and the presence of excessive fungal hyphae and thrombosis were also associated with poor outcomes.

Vitamin D and Muscle Status in Inflammatory and Autoimmune Rheumatic Diseases: An Update.

Background and Objectives: Vitamin D is a secosteroid hormone essential for calcium homeostasis and skeletal health, but established evidence highlights its significant roles also in muscle health and in the modulation of immune response. This review aims to explore the impact of impaired vitamin D status on outcomes of muscle function and involvement in inflammatory and autoimmune rheumatic diseases damaging the skeletal muscle efficiency both with direct immune-mediated mechanisms and indirect processes such as sarcopenia. Methods: A comprehensive literature search was conducted on PubMed and Medline using Medical Subject Headings (MeSH) terms: "vitamin D, muscle, rheumatic diseases." Additionally, conference abstracts from The European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR) (2020-2023) were reviewed, and reference lists of included papers were scanned. The review emphasizes the evidence published in the last five years, while also incorporating significant studies from earlier years, structured by the extent of evidence linking vitamin D to muscle health in the most commonly inflammatory and autoimmune rheumatic diseases encountered in clinical practice. Results: Observational studies indicate a high prevalence of vitamin D serum deficiency (mean serum concentrations < 10 ng/mL) or insufficiency (<30 ng/mL) in patients with idiopathic inflammatory myopathies (IIMs) and polymyalgia rheumatica, as well as other autoimmune connective tissue diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Of note, vitamin D insufficiency may be associated with reduced muscle strength (2 studies on RA, 2 in SLE and 1 in SSc), increased pain (1 study on SLE), fatigue (2 studies on SLE), and higher disease activity (3 studies on IIMs and 1 on SLE) although there is much heterogeneity in the quality of evidence and different associations for the different investigated diseases. Therefore, linked to the multilevel biological intervention exerted by vitamin D, several translational and clinical studies suggest that active metabolites of this secosteroid hormone, play a role both in reducing inflammation, but also in enhancing muscle regeneration, intra-cellular metabolism and mitochondrial function, although interventional studies are limited. Conclusions: Altered serum vitamin D status is commonly observed in inflammatory and autoimmune rheumatic diseases and seems to be associated with adverse muscle health outcomes. While maintaining adequate serum vitamin D concentrations may confer muscle-protective effects, further research is needed to confirm these findings and establish optimal supplementation strategies to obtain a safe and efficient serum threshold.

Exercise selection plays an important role in inducing non-uniform acute responses on elbow flexors muscle thickness

The muscle thickness (MT) acutely increases in response to the redirection of blood flow during exercise, with this muscle swelling being a proxy to quantify the degree of muscle stimuli and involvement in an exercise. The present study aims to compare the MT acute variations between the biceps brachii (BB) and brachialis (BRA) muscles in two different unilateral exercises, the dumbbell curl (DC) and dumbbell row (DR). Sixteen well-trained young males performed a 10-repetition maximum (10RM) load test and retest for each exercise. Four sets were performed with the 10RM load for each exercise until the concentric failure. The BB and BRA muscles MT were measured pre and post-intervention with a B-mode ultrasound images. The main finding of this study was that, when DC is performed, the BB presented greater MT changes (18.67% ± 6.23) compared to BRA (12.27% ± 6.20) (p = 0.025), and compared to BB when performed in DR (12.23% ± 6.03) (p = 0.024). It was also found that BB greatest increase from BRA was not observed in DR and the BRA results were similar in both exercises. These results indicate that the structural acute responses can be muscle-specific among the elbow flexors muscles for the single-joint exercise and also exercise-specific. Therefore, either one of these two exercises can be chosen to stimulate BRA, but it would be interesting to use DC to offer greater stimuli for BB.

Skeletal muscle involvement in systemic amyloidosis is often overlooked.

Systemic amyloidosis is a condition in which misfolded amyloid fibrils are deposited within tissues. Amyloid myopathy is a rare manifestation of systemic amyloidosis. However, whether skeletal muscle involvement is underestimated and whether such deposition guarantees clinical and pathological myopathic features remain to be investigated. We retrospectively reviewed patients with systemic amyloidosis, in whom skeletal muscle biopsies were performed at our centre between January 2018 and June 2023. In total, 28 patients with suspected systemic amyloidosis were included. Among these, 21 presented with cardiomyopathy but lacked myopathic symptoms. The clinical and pathological data of these patients were further analysed. The amyloid type was confirmed by immunohistochemistry. Twenty-eight patients with suspected systemic amyloidosis underwent muscle biopsy. Amyloid deposition in the skeletal muscle was confirmed in 24 patients, including 22 with light-chain amyloidosis (AL) and two with transthyretin amyloidosis (ATTR). Among the 24 patients, seven presented with muscle weakness and decreased muscle strength (Group 1, symptomatic myopathy), whereas the remaining 17 exhibited normal muscle strength (Group 2, asymptomatic myopathy). Group 1 included four patients with AL-λ, one with AL-κ and two with ATTR. Group 2 included 15 patients with AL-λ and two patients with AL-κ. In Group 1, six patients exhibited neuropathy, whereas only one patient in Group 2 presented with subclinical neuropathy on nerve conduction studies. Amyloid deposition in the interstitium was the most obvious change, observed in all 24 patients. Neuropathic changes, including denervation atrophy and muscle fibre grouping, were also common. Except for type 2 fibre atrophy, the other myopathic changes were mild and nonspecific. No sarcolemmal disruption was observed. Immunohistochemical analysis revealed marked positivity for MAC and MHC1 expression in the regions with amyloid deposits. Clinicopathological analysis revealed no significant differences in the extent of muscular amyloid deposition between the two groups. Nevertheless, patients in Group 1 displayed more pronounced neurogenic atrophy on skeletal muscle biopsies. Our study indicates that amyloid deposition in skeletal muscle is commonly observed but rarely causes symptomatic myopathy in systemic amyloidosis.

A protocol for scoping reviews on the role of whole-body and dedicated body-part magnetic resonance imaging for assessment of adult and juvenile idiopathic inflammatory myopathies.

Currently, standardized magnetic resonance imaging (MRI) scoring systems and protocols for assessment of idiopathic inflammatory myopathies (IIMs) in children and adults are lacking. Therefore, we will perform a scoping review of the literature to collate and evaluate the existing semi-quantitative and quantitative MRI scoring systems and protocols for the assessment and monitoring of skeletal muscle involvement in patients with IIMs. The aim is to compile evidence-based information that will facilitate the future development of a universal standardized MRI scoring system for both research and clinical applications in IIM. A systematic search of electronic databases (PubMed, EMBASE, and Cochrane) will be undertaken to identify relevant articles published between January 2000 and October 2023. Data will be synthesized narratively. This scoping review seeks to comprehensively summarize and evaluate the evidence on the scanning protocols and scoring systems used in the assessment of diagnosis, disease activity, and damage using skeletal muscle MRI in IIMs. The results will allow the development of consensus recommendations for clinical practice and enable the standardization of research methods for the MRI assessment of skeletal muscle changes in patients with IIMs.

Muscle Ultrasonography in Relation to Motor Function in Children with Duchenne Muscular Dystrophy

Abstract Background and Aim Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy of childhood and affects 1 per 3600 male births. Electromyography and muscle biopsy were a commonly ordered invasive diagnostic procedure, used to evaluate children with weakness suspected to be caused by muscle disease. Recently, neuromuscular imaging is used as non-invasive diagnostic tool for various muscle diseases. Muscle ultrasonography is increasingly used for the diagnosis and follow up of children with DMD. Aim of the Work The aim of this study was to describe the pattern of ultrasonographic thigh muscles involvement, and to correlate the US findings with motor functional status. Patients and Methods Functional motor assessment was the functional performance protocol (GSGC score) Gait (G), Climbing stairs (S), Gower's maneuver (G), and Arising from a chair (C). Sonographic assessment of quadriceps rectus femoris muscles. Results Twenty-four genetically confirmed ambulatory DMD children were enrolled in this cross- sectional study. Their mean age of 7.46 ± 1.82 (4.5–11 years). The Rt and Lt Quadriceps muscles positively correlated with the age. The Rt. and Lt. Quadriceps muscle assessment significantly related to the scores for gait, stairs, chair arising, GSGC total score, time to walk 10 m, Time to rise from the floor. Conclusion Ultrasonography could be used as a non-invasive method to assess progressive muscle hypertrophy in boys with Duchenne muscular dystrophy. Furthermore, Quadriceps muscle US correlates with the motor functions in ambulant DMD boys below the age of 12 years.

Amyopathic Dermatomyositis Presenting with Digital Ulcerations: Case Report and Literature Review.

Clinically amyopathic dermatomyositis (CADM) is a rare subtype of dermatomyositis that presents with cutaneous features and no muscle involvement. This case report describes a 26-year-old woman with recurrent and multiple digital ulcerations coinciding with the start of winter each year. There was no evidence of myopathy, and antibody testing yielded negative results. A diagnosis of CADM was ultimately made based on clinicopathologic correlation. The patient's ulcers demonstrated excellent response to a combination therapy of hydroxychloroquine and potent topical and systemic steroids. Herein, the authors discuss the pathologic and immunologic characteristics of CADM.

Clinical Profile of Thyroid Eye Disease and Factors Predictive of Disease Severity in Chennai District of Tamil Nadu

Abstract Background: Thyroid eye disease (TED) is a possibly sightthreatening ocular disease generally occurring in the patients diagnosed with hyperthyroidism. TED, also known as Graves orbitopathy, is typically self-limiting auto-immune disease linked with a dysthyroid status. Based on the ocular symptoms and signs, the current grading systems of TED are the VISA classification and the EUGOGO classification; each parameter of VISA score is separately graded and scored. Worsening in any of the VISA parameters is graded as an active disease in the geographic region of South India; there are no studies on clinical profiles of TED; hence, we planned to do this study to analyse the demographic profile and clinical manifestations of TED in patients. Material and Methodology: This study was done as an observational study for a period of 12 months. All patients with systemic thyroid disorder are included in the study. If lid retraction is seen, the presence of laboratory evidence of thyroid dysfunction, exophthalmos, optic nerve dysfunction, or extra ocular muscle involvement will be considered as TED. In case of no lid retraction in a patient with laboratory evidence of thyroid dysfunction, the presence of exophthalmos, optic nerve involvement, or restrictive myopathy will be considered as TED. Patients with diagnosis of TED were further evaluated. TED was graded according to VISA and EUGOGO classification. Results: This study was done as an observational study in 50 patients diagnosed with TED. In our study population, 32 (64%) were hyperthyroid, 11 (22%) were hypothyroidism, and the rest 7 (14%) were euthyroid. The most common presenting complaints were dryness of eye in 46% of patients, followed by blurred vision in 38% of patients. We evaluated severity of disease using EUGOGO score, and 42 patients had mild disease, while six had moderate disease and two patients had severe disease. Based on VISA score, in our study population, only two patients had active disease and the rest 48 had inactive disease. Conclusion: Thyroid status has a direct and positive correlation with active stage of disease and severity. Hyperthyroidism and active disease were risk factors for severe and sight-threatening disease, emphasising the need for close follow-up and prompt detection and management of severe and sight-threatening disease, especially in newly diagnosed hyperthyroid patients. It is important to diagnose TED at the earliest to prevent vision-threatening complications. Early identification and management will help in reducing the severity of disease.