With the development of critical medical emergency technology, the success rate of sepsis treatment has been significantly improved, and the improvement of the long-term quality of life of sepsis survivors has also attracted more and more attention. ICU-acquired weakness (ICU-AW) refers to a group of syndromes with systemic and symmetrical muscle weakness during the intensive care unit (ICU) hospitalization and cannot be explained by the patient's own disease, which often involve diaphragm and skeletal muscle, resulting in difficulty in weaning and nosocomial infection. The incidence of ICU-AW in sepsis patients is over 50%, making it an important factor affecting the prognosis of these patients. The occurrence of sepsis ICU-AW is related to many factors, which can be summarized into two categories, including sepsis-related factors such as sepsis-associated inflammatory response, sepsis-associated encephalopathy (SAE), and treatment-related factors such as physical immobilization and insufficient nutritional support. The current ICU-AW risk assessment tools are mainly on subjective assessment scales, but there are some limitations in clinical application, and objective assessment tools including predictive model and imaging assessment, which are still in the research stage. "ABCDEF bundle strategy" is an important measure to prevent ICU-AW, in which early rehabilitation is the core element. This review of the literature from the risk factors, risk assessment and early rehabilitation of ICU-AW, and focuses on the timing, content, method and safety assessment of early rehabilitation, aims to improve the understanding of ICU-AW, strengthen the prevention of sepsis with ICU-AW, and improve the prognosis of sepsis patients, not only survive, but also live better.
Humans ; Sepsis/complications* ; Muscle Weakness/etiology* ; Intensive Care Units ; Prognosis ; Quality of Life

Sepsis-acquired weakness (SAW) is a common complication in critically ill patients, yet significant gaps remain in both mechanistic understanding and therapeutic interventions for this condition. SAW not only prolongs the duration of mechanical ventilation and hospitalization but is also closely associated with increased mortality. Even if these SAW patients survive, they often experience long-term physical dysfunction after hospital discharge, leading to diminished quality of life. Emerging evidence suggests that sustained mitochondrial dysfunction may constitute a pivotal pathophysiological basis for the development and progression of SAW, primarily encompassing five key aspects: dysregulated mitochondrial quality control (MtQC), impaired oxidative phosphorylation (OXPHOS), exacerbated oxidative stress, disrupted Ca²⁺; homeostasis, and their mediation of diverse myofiber injuries. This article systematically elucidates the central role of mitochondrial dysfunction in the pathogenesis of SAW. Furthermore, we explore potential therapeutic strategies targeting mitochondrial function, including mitigating mitochondrial oxidative stress, optimizing nutritional support, and supplementing with muscle-derived mesenchymal stem cells. These insights provide a critical theoretical framework for understanding SAW mechanisms and developing clinical interventions, with particular emphasis on the translational value of mitochondrial-targeted therapies in improving outcomes for septic patients.
Humans ; Sepsis/metabolism* ; Mitochondria/metabolism* ; Muscle Weakness/etiology* ; Oxidative Stress ; Oxidative Phosphorylation

INTRODUCTION: Rehabilitation medicine in a tertiary care hospital involves attending to many patients affected by intensive care unit (ICU)-associated weakness (ICU-AW) and hospital-associated deconditioning (HAD). These conditions contribute to poor long-term functional outcomes and increased mortality. We explored the role of short-term adjunctive androgen therapy in this group of patients in improving the rehabilitative outcomes. METHODS: This was a retrospective analysis of five patients with either ICU-AW or HAD who were given testosterone replacement therapy (TRT) or oxandrolone for a total of 2 weeks during the period from April to November 2020 was undertaken. During the 2-week trial period, the subjects underwent standard rehabilitation therapy. RESULTS: Grip strength was used as the primary outcome measure, and the mean improvement was 4.2 kg (+24.9%), which is encouraging in a 2-week timeframe. This was matched with good functional recovery in terms of distance ambulated and less assistance needed for ambulation. Sex hormone analysis was also done before initiation of TRT, and it showed that four out of five of the subjects were biochemically hypogonadal. None of the subjects dropped out or experienced any significant adverse events over the 2-week trial period. All the subjects except one improved to full independence at 3 months post-discharge. CONCLUSION TRT has the potential to be used as a useful adjunct to standard rehabilitation in enhancing functional recovery in critically ill patients. A multidisciplinary approach would ensure that suitable patients benefit from optimal nutrition, optimal rehabilitation and synergistic testosterone therapy in a clinically sound and resource-efficient fashion.
Humans ; Testosterone/therapeutic use* ; Retrospective Studies ; Male ; Hormone Replacement Therapy/methods* ; Intensive Care Units ; Singapore ; Middle Aged ; Muscle Weakness/drug therapy* ; Aged ; Hospitals, General ; Hand Strength ; Androgens/therapeutic use* ; Treatment Outcome ; Critical Illness/rehabilitation* ; Female ; Recovery of Function

OBJECTIVE: To observe the clinical efficacy of vitamin B1 in patients with ICU-acquired weakness (ICU-AW). METHODS: A retrospective analysis was conducted to select ICU-AW patients treated with vitamin B1 in the intensive care unit (ICU) of Shanghai East Hospital, Tongji University from July 2022 to December 2023. Propensity score matching was used to match the control group and observation group at a 1 : 1 ratio, considering differences in patient age, gender, and acute physiology and chronic health evaluation II (APACHE II). The control group received conventional treatment, while the observation group received vitamin B1 in addition to conventional treatment. Comparisons were made between the two groups in terms of basic information, APACHE II scores, Medical Research Council muscle strength score (MRC), interleukin-6 (IL-6), C-reactive protein (CRP), and serum creatinine (SCr) levels before and after treatment, mechanical ventilation time, and length of ICU stay were collected and compared between the two groups. RESULTS: A total of 30 patients with ICU-AW were included, both 15 in each group. There were no significant differences in gender, age and APACHE II score between the two groups. There were no significant differences in APACHE II score, MRC score, IL-6, CRP, SCr levels, and the duration of ventilator-assisted ventilation and ICU stay between the two groups of patients before treatment. Both groups showed improvements in APACHE II scores and MRC scores after treatment, with the observation group showing significantly greater improvement compared to the control group (APACHE II score: 10.80±1.47 vs. 12.20±1.52, MRC score: 50.40±7.10 vs. 42.00±8.78, both P < 0.05). After treatment, both groups showed significant reductions in IL-6, CRP, and SCr levels, but there were no significant differences between the observation group and control group. The observation group had significantly shorter durations of mechanical ventilation (days: 7.67±1.54 vs. 9.67±2.44, P < 0.05) and ICU stay (days: 13.33±1.72 vs. 15.13±2.56, P < 0.05) compared to the control group. CONCLUSIONS The use of vitamin B1 during the treatment of ICU-AW significantly improves muscle strength and reduces the duration of mechanical ventilation and ICU stay.
Humans ; Intensive Care Units ; Retrospective Studies ; APACHE ; Muscle Weakness/drug therapy* ; Respiration, Artificial ; Thiamine/therapeutic use* ; C-Reactive Protein ; Interleukin-6/blood* ; Length of Stay ; Male ; Female ; Treatment Outcome ; Propensity Score ; Middle Aged

OBJECTIVE: To systematically assess the efficacy of traditional Chinese therapy in the treatment of ICU-acquired weakness (ICU-AW). METHODS: PubMed, Cochrane Library, Embase, Web of Science, CNKI, Wanfang, VIP were retrieved by computer and were used to collect a randomized controlled trials (RCT) of traditional Chinese therapy for ICU-AW. The retrieval time was from databases establishment to December 2021. After 2 researchers independently screened the literature, extracted data and evaluated the risk of bias included in the study, and RevMan 5.4 software was used for Meta-analysis. RESULTS: 334 articles were selected, totally 13 clinical studies and 982 patients were included, including 562 in the trial group and 420 in the control group. Meta-analysis results showed that traditional Chinese therapy could improve clinical efficacy of ICU-AW patients [relative risk (RR) = 1.35, 95% confidence interval (95%CI) was 1.20 to 1.52, P < 0.000 01], improve the muscle strength [Medical Research Council score (MRC score); standardized mean difference (SMD) = 1.00, 95%CI was 0.67 to 1.33, P < 0.000 01], improve daily life ability [modified Barthel index score (MBI score); SMD = 1.67, 95%CI was 1.20 to 2.14, P < 0.000 01], shorten mechanical ventilation time (SMD = -1.47, 95%CI was -1.84 to -1.09, P < 0.000 01), reduce the length of intensive care unit (ICU) stay [mean difference (MD) = -3.28, 95%CI was -3.89 to -2.68, P < 0.000 01], reduce the total hospitalization time (MD = -4.71, 95%CI was -5.90 to -3.53, P < 0.000 01), reduce tumor necrosis factor-α (TNF-α; MD = -4.55, 95%CI was -6.39 to -2.70, P < 0.000 01) and interleukin-6 (IL-6; MD = -5.07, 95%CI was -6.36 to -3.77, P < 0.000 01). There was no obvious advantage in reducing the severity of the disease [acute physiology and chronic health evaluation II (APACHE II; SMD = -0.45, 95%CI was -0.92 to 0.03, P = 0.07). CONCLUSIONS Based on the current research, traditional Chinese therapy can improve the clinical efficacy of ICU-AW, improve muscle strength and daily life ability, shorten mechanical ventilation, the length of ICU stay and total hospitalization time, reduce TNF-α and IL-6. But traditional Chinese therapy can not reduce the overall disease severity.
Humans ; APACHE ; East Asian People ; Intensive Care Units ; Interleukin-6 ; Tumor Necrosis Factor-alpha ; Medicine, Chinese Traditional ; Muscle Weakness/therapy*

Objective: To investigate the clinical features and gene variation characteristics of children with dynein cytoplasmic 1 heavy chain 1 (DYNC1H1) gene associated spinal muscular atrophy with lower extremity predominant (SMALED) 1. Methods: The clinical data of 4 SMALED1 children admitted to Peking University First Hospital from December 2018 to May 2021, who were found to have pathogenic variation of DYNC1H1 gene through genetic testing, except for other genes known to be related to motor retardation, were retrospectively summarized to analyze the phenotype and genotype characteristics. Results: There were 3 males and 1 female. The age of onset was 1 year, 1 day, 1 day and 4 months, respectively. The age of diagnosis was 4 years and 10 months, 9 months, 5 years and 9 months, and 3 years and 1 month, respectively. The clinical manifestations were muscle weakness and muscular atrophy of lower limbs, 2 cases with foot deformity, 1 case with early non progressive joint contracture, 1 case with hip dislocation and 1 case with mental retardation. De novo heterozygous missense variations in DYNC1H1 gene were found in all 4 children. According to the rating of American College of medical genetics and genomics, they were all possible pathogenic and pathogenic variations, with p.R598C, p.P776L, p.Y1109D variations had been reported, and p.I1086R variation had not been reported. Conclusions: For those with unexplained lower limb muscle weakness, muscle atrophy, joint contracture and foot deformity, upper limb motor ability related retention, with or without mental retardation, as well as the motor ability progresses slowly, it is necessary to consider the possibility of SMALED1 and the detection of DYNC1H1 gene when necessary.
Female ; Male ; Humans ; Intellectual Disability ; Retrospective Studies ; Muscular Atrophy, Spinal/genetics* ; Lower Extremity ; Muscle Weakness ; Muscular Atrophy ; Contracture ; Cytoplasmic Dyneins/genetics*

Objective: To summarize the genetic and clinical phenotypic characteristics of patients with early-onset myopathy, areflexia, respiratory distress and dysphagia (EMARDD) caused by multiple epidermal growth factor 10 (MEGF10) gene defect. Methods: The clinical data of 3 infants in 1 family with EMARDD caused by MEGF10 gene defect diagnosed in the Department of Neonatology, Xiamen Children's Hospital in April 2022 were analyzed retrospectively. Using "multiple epidermal growth factor 10" "myopathy" or "MEGF10" "myopathy" as the key words, and searching the relevant literature reports of CNKI, Wanfang Database and PubMed Database from the establishment of the database to September 2022. Combined with this family, the main clinical information and genotype characteristics of EMARDD patients caused by MEGF10 gene defect were summarized. Results: The proband, male, first infant of monozygotic twins, was admitted to hospital 7 days after birth "due to intermittent cyanosis with weak sucking". The infant had dysphagia accompanied with cyanosis of lips during feeding and crying after birth. Physical examination on admission revealed reduced muscle tone of the extremities, flexion of the second to fifth fingers of both hands with limited passive extension of proximal interphalangeal joints, and limited abduction of both hips. He was diagnosed as dysphagia of newborn, congenital dactyly. After admission, he was given limb and oral rehabilitation training, breathing gradually became stable and oral feeding fully allowed, and discharged along with improvement. The younger brother of the proband was admitted to the hospital at the same time, and his clinical manifestations, diagnosis and treatment process were the same as those of the proband. The elder brother of the proband died at the age of 8 months due to the delayed growth and development, severe malnutrition, hypotonia, single palmoclal crease and weak crying. A whole exon sequencing of the family was done, and found that the 3 children were all compound heterozygous variations at the same site of MEGF10 gene, with 2 splicing variants (c.218+1G>A, c.2362+1G>A), which came from the father and mother respectively, and the new variation was consistent with the autosomal recessive inheritance model. Three children were finally diagnosed as EMARDD caused by MEGF10 gene defect. There are 0 Chinese literature and 18 English literature that met the search conditions. Totally 17 families including 28 patients were reported. There were 31 EMARDD patients including 3 infants from this family. Among them, there were 13 males and 18 females. The reported age of onset ranged from 0 to 61 years. Except for 5 patients with incomplete clinical data, 26 patients were included in the analysis of phenotypic and genotypic characteristics. The clinical features were mainly dyspnea (25 cases), scoliosis (22 cases), feeding difficulties (21 cases), myasthenia (20 cases), and other features including areflexia (16 cases) and cleft palate or high palatal arch(15 cases). Muscle biopsy showed non-specific changes, with histological characteristics ranging from slight muscle fiber size variation to minicores change which was seen in all 5 patients with at least 1 missense mutation of allele. In addition, the adult onset was found in patients with at least 1 missense variant of MEGF10 gene. Conclusions: MEGF10 gene defect related EMARDD can occur in the neonatal period, and the main clinical features are muscle weakness, breathing and feeding difficulties. Patients with myopathy who have at least 1 missense mutation and muscle biopsy indicating minicores change may be relatively mild.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Young Adult ; Cyanosis ; Deglutition Disorders ; EGF Family of Proteins ; Muscle Hypotonia ; Muscle Weakness ; Muscular Diseases/genetics* ; Retrospective Studies

OBJECTIVE: To study the clinical features of children with Guillain-Barré syndrome (GBS) and the significance of Brighton criteria in childhood GBS. METHODS: A retrospective analysis was performed on the medical data of 72 children with GBS. Brighton criteria were used for the grading of diagnostic certainty (level 1 as the highest level, and level 4 as the lowest level). A Spearman's rank correlation analysis was used to evaluate the correlation of auxiliary examinations with the level of diagnostic certainty of Brighton criteria. RESULTS: A total of 72 children with GBS were enrolled, with a mean age of onset of (98±32) months. All children (100%, 72/72) had weakness of bilateral limbs and disappearance or reduction of tendon reflex, and limb weakness reached the highest level of severity within 4 weeks. Of all the 72 children, 68 (94%) had positive results of neural electrophysiological examination and 51 (71%) had positive results of cerebrospinal fluid (CSF) examination, and the positive rate of neural electrophysiological examination was significantly higher than that of CSF examination ( CONCLUSIONS Most of the children with GBS meet Brighton criteria level 1, and the positive results of CSF examination and neural electrophysiological examination play an important role in improving the level of diagnostic certainty of Brighton criteria. Neural electrophysiological examination has a higher positive rate than CSF examination in the early stage of the disease.
Child ; Child, Preschool ; Extremities ; Guillain-Barre Syndrome/diagnosis* ; Humans ; Muscle Weakness ; Physical Examination ; Retrospective Studies

OBJECTIVE: To compare balance performance and lower limb muscle strength between older adults with type 2 diabetes mellitus (DM), with and without sensory impairments and non-DM groups. Influence of a number of sensory impairments, and muscle strength on balance performance were explored. METHODS: Ninety-two older adults with and without type 2 DM, were examined relative to visual function with the Snellen chart, Melbourne Edge test, and Howard-Dolman test, vestibular function with the modified Romberg test, proprioception of the big toe, and diabetic peripheral neuropathy with the Michigan Neuropathy Screening Instrument. Balance performances were evaluated with the Romberg test, Functional Reach Test (FRT), and Timed Up and Go test (TUG). Strength of knee and ankle muscles was measured. RESULTS: FRT of type 2 DM groups with at least two sensory impairments, was lower than the non-DM group (p<0.05). TUG of all DM groups, was worse than the non-DM group (p<0.01). Lower limb muscle strength of type 2 DM groups with two and three sensory impairments, was weaker than non-DM group (p<0.05). Regression analysis showed that type 2 DM with three sensory impairments, ankle dorsiflexors strength, and age were influential predictors of TUG. CONCLUSION: There were significant differences, of muscle strength and balance performance among groups. Poorer balance and reduced lower limb strength were marked in older adults with type 2 DM, even ones without sensory impairment. Muscle weakness seemed to progress, from the distal part of lower limbs. A greater number of sensory impairments, weaker dorsiflexors, and advanced age influenced balance performance.
Adult ; Aged ; Ankle ; Diabetes Mellitus, Type 2 ; Hallux ; Humans ; Knee ; Lower Extremity ; Mass Screening ; Michigan ; Muscle Strength ; Muscle Weakness ; Muscles ; Peripheral Nervous System Diseases ; Proprioception ; Vestibular Function Tests

Myasthenia gravis (MG) is a neuromuscular autoimmune disorder which clinically presents as muscular weakness and fatigue due to autoantibody formation against acetylcholine receptors (AChR), leading to their subsequent destruction. Due to the neuromuscular implications of MG, certain considerations must be taken into account when providing anesthesia to MG patients. In the following case report, we have outlined procedural considerations for the anesthetic management of a patient with MG undergoing deep sedation for an elective oral surgery in an outpatient setting, as well as a discussion of relevant literature.
Anesthesia ; Deep Sedation ; Fatigue ; Humans ; Muscle Weakness ; Myasthenia Gravis ; Outpatients ; Receptors, Cholinergic ; Surgery, Oral