Myopathies of endocrine origin: A review for physicians

Shah, Devarsh N.; Chorya, Harshal Prakash; Ramesh, N. Nishitha; Gnanasekaram, Sulochana; Patel, Neil; Sethi, Yashendra; Kaka, Nirja

doi:10.1016/j.disamonth.2023.101628

Cited by 3 publications

(1 citation statement)

References 89 publications

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“…The clinical manifestations of hypothyroidism also include symptoms involving the musculoskeletal system, such as muscle hypotonia or isolated pains in the muscles and the joints [4,8]; however, severe myopathies are not common in children. KDSS is a rare hypothyroid myopathy that is developed in childhood, characterised by the muscular pseudohypertrophy of the lower limbs.…”

Section: Discussionmentioning

confidence: 99%

Hypothyroid Myopathy—A Rare Case from Paediatric Practice

Elkina,

Stoyanova,

Halvadzhiyan

et al. 2024

Children

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Hypothyroid myopathy is uncommon in childhood. Severe hypothyroid myopathy observed in paediatric practice is a part of Kocher–Debré–Semelaigne syndrome (KDSS, OR-PHA:2349), a rare disorder characterised by muscular pseudohypertrophy and long-standing moderate-to-severe hypothyroidism. We present a pubertal girl with KDSS diagnosed with severe myopathy and significantly limited mobility and progressively increasing pains in the lumbar area, hip joints, and the lower limbs. Additionally, the patient presented metabolic syndrome with severe obesity, growth retardation, and educational difficulties. In this case, adequate hormone replacement therapy with Levothyroxine evoked full recovery of the myopathy and a significant reversal in the patient’s general condition. In conclusion, emphasizing the knowledge related to KDSS can improve the diagnosis and prognosis of the condition.

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Section: Discussionmentioning

confidence: 99%

Hypothyroid Myopathy—A Rare Case from Paediatric Practice

Elkina,

Stoyanova,

Halvadzhiyan

et al. 2024

Children

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Hospital admissions from the emergency department of adult patients affected by myopathies

Monforte,

Torchia,

Bortolani

et al. 2024

Euro J of Neurology

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Background and purposeMyopathies are associated with classic signs and symptoms, but also with possible life‐threatening complications that may require assistance in an emergency setting. This phenomenon is understudied in the literature. We aimed to assess the presentation, management, and outcomes of clinical manifestations potentially related to a muscle disorder requiring referral to the adult emergency department (ED) and hospitalization.MethodsAnonymized patient data retrieved using the International Classification of Diseases, Ninth Revision codes related to muscle disorders over 4 years were retrospectively analyzed. Medical reports were evaluated to extract demographic and clinical variables, along with outcomes. Two groups were defined based on the presence (known diagnosis [KD] group) or absence (unknown diagnosis [UD] group) of a diagnosed muscle disorder at arrival.ResultsA total of 244 patients were included, 51% of whom were affected by a known myopathy, predominantly limb‐girdle muscular dystrophies and myotonic dystrophies. The main reasons for ED visits in the KD group were respiratory issues, worsening of muscle weakness, and gastrointestinal problems. Heart complications were less prevalent. In the UD group, 27 patients received a new diagnosis of a specific primary muscle disorder after the ED access, mostly an inflammatory myopathy. Death during hospitalization was recorded in 26 patients, with a higher rate in the KD group and in patients affected by mitochondrial and inflammatory myopathies. Sepsis and dyspnea were associated with increased death risk.ConclusionsRespiratory complications are the most common reason for myopathic patients accessing the ED, followed by gastrointestinal issues. Infections are severe threats and, once hospitalized, these patients have relatively high mortality.

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Hydroxychloroquine Mitigates Cytokine Storm and Prevents Critical Illness Neuromyopathy in a Rat Sepsis Model

Eygi,

Kucuk,

Aydemir

et al. 2024

Medicina

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Background and Objectives: It is known that critical illness and associated neuromuscular problems begin to appear in patients hospitalized in the intensive care unit (ICU) for more than a week. The goal of this study was to research the role of hydroxychloroquine (HCQ) in the treatment of cytokine storm and critical illness neuromyopathy (CINM) in a rat sepsis model. Materials and Methods: Rats were assigned into three groups, and a feces intraperitoneal-injection group (FIP) procedure was carried out on 30 rats to induce a model of sepsis for critical illness polyneuromyopathy (CINM). The study groups were as follows: Group 1: control (nonoperative and orally fed control, n = 10), Group 2: FIP with 0.9% NaCl saline was given as 1 mL/kg/day by oral gavage (n = 10), and Group 3: FIP with 10 mg/kg/day of hydroxychloroquine (Plaquenil 200 mg) administered by oral gavage (n = 10). Electrophysiological recordings (EMG) were conducted six days after surgery. EMG was carried out three times on the right sciatic nerve, which was stimulated with supramaximal intensity utilizing a bipolar needle electrode at the sciatic notch. Tumor necrosis factor-alpha (TNF-α), malondialdehyde (MDA), lactic acid levels, and interleukin-6 (IL-6) were evaluated. Results: In terms of TNF-α, MDA, lactic acid levels, and IL-6, there was a statistically significant decrease in the CINM + 10 mg/kg HCQ group compared to the CINM and saline group (p < 0.0001, p < 0.05, p < 0.05, and p < 0.05, respectively). Compound muscle action potentials (CMAPs) latency and duration were decreased in the CINM + 10 mg/kg HCQ group compared to other groups (p < 0.01 and p < 0.001). However, CMAP amplitude was significantly higher in the CINM + 10 mg/kg HCQ group unlike the CINM and saline group (p < 0.001). Conclusions: This is the first study to demonstrate the effects of HCQ on CINM in a rat model of sepsis. The findings of our research suggest that hydroxychloroquine may be used as a potential therapeutic agent in the treatment of sepsis. Hydroxychloroquine may have an important effect in the pathogenesis of sepsis-associated CINM by reducing cytokine production and oxidative stress.

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Myopathies of endocrine origin: A review for physicians

Cited by 3 publications

References 89 publications

Hypothyroid Myopathy—A Rare Case from Paediatric Practice

Hypothyroid Myopathy—A Rare Case from Paediatric Practice

Hospital admissions from the emergency department of adult patients affected by myopathies

Hydroxychloroquine Mitigates Cytokine Storm and Prevents Critical Illness Neuromyopathy in a Rat Sepsis Model

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