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1.
World J Clin Cases ; 12(21): 4455-4459, 2024 Jul 26.
Article in English | MEDLINE | ID: mdl-39070840

ABSTRACT

This editorial explores the significant challenge of intensive care unit-acquired weakness (ICU-AW), a prevalent condition affecting critically ill patients, characterized by profound muscle weakness and complicating patient recovery. Highlighting the paradox of modern medical advances, it emphasizes the urgent need for early identification and intervention to mitigate ICU-AW's impact. Innovatively, the study by Wang et al is showcased for employing a multilayer perceptron neural network model, achieving high accuracy in predicting ICU-AW risk. This advancement underscores the potential of neural network models in enhancing patient care but also calls for continued research to address limitations and improve model applicability. The editorial advocates for the development and validation of sophisticated predictive tools, aiming for personalized care strategies to reduce ICU-AW incidence and severity, ultimately improving patient outcomes in critical care settings.

2.
World J Clin Cases ; 12(21): 4853-4855, 2024 Jul 26.
Article in English | MEDLINE | ID: mdl-39070851

ABSTRACT

Wang et al reported 1063 cases from the initial 14 d of intensive care unit (ICU) stay, and analyzed relevant data such as age, comorbidities, recent dosages, vapor pressure dosages, duration of mechanical ventilation, length of ICU stay, and rehabilitation therapy, which are closely related to ICU-acquired weakness (ICU-AW). It is suggested that the length of ICU stay and the duration of mechanical ventilation are the main factors. ICU-AW is the most common neuromuscular injury in the ICU, which affects clinical progression and outcomes of patients. This manuscript helps to improve the early recognition of ICU-AW, thereby reducing mortality and improving prognosis.

3.
SAGE Open Med Case Rep ; 12: 2050313X241267073, 2024.
Article in English | MEDLINE | ID: mdl-39071194

ABSTRACT

Functional neurological symptom disorder (FNSD) is a complex condition with multifactorial etiology in which psychological factors may play a significant role in some patients, and they are not universally present or necessary for the diagnosis. FNSD can occur in the absence of psychological distress and each patient's presentation should be evaluated individually, considering a wide range of potential contributing factors. This is the first report related to prevalence data from Rwanda. This report presents a case study of a 15-year-old female patient who presented hemibody weakness without a structural neurological explanation on the background of the direct exposure to parental conflict. she was ultimately diagnosed with an FNSD and responded effectively to five sessions of cognitive behavioral therapy. Additionally, follow-up appointments were conducted every 4 months over the course of 1 year. During this period, the patient successfully resumed her studies and demonstrated normal functioning in all essential areas of daily life. This is the first reported case in Rwanda among similar cases. Addressing these specific stressors played a crucial role in the patient's overall outcome, leading to improved quality of care and prevention of unnecessary medical costs and interventions.

4.
J Med Life ; 17(4): 426-431, 2024 Apr.
Article in English | MEDLINE | ID: mdl-39071514

ABSTRACT

This multicenter retrospective cohort study aimed to evaluate the effectiveness of the retrosigmoid surgical approach in decompressing vestibular schwannomas, focusing on tumor decompression, neurological function preservation, and postoperative complications. A cohort of 60 patients, operated between 2016 and 2019, was analyzed for age, sex, symptoms, tumor size, surgery duration, complications, mortality, and facial/auditory functions using established criteria. Hearing loss was observed in 80% of patients, mainly progressive, with tumor size emerging as a critical prognostic factor. Facial weakness affected 10% of patients preoperatively; postoperatively, 35% of patients had affected facial nerve function, with 10% exhibiting poor or no facial nerve function, linked to resection extent rather than tumor size. Tinnitus was more prevalent with larger tumors, whereas headaches were common irrespective of size. Balance disorders improved after surgery, especially in case of larger tumors. Functional recovery varied, with 41.67% of patients returning to their previous activity within 4 months, 25% within 4-12 months, and 33.33% remaining inactive. The mortality rate was low at 3.3 %, with two deaths out of 60 patients after surgery. This analysis highlights surgery risks for vestibular schwannomas (e.g., facial nerve decline, tinnitus, headaches), but also emphasizes benefits like improved balance and low mortality. Many patients regain professional activity, stressing the importance of informed treatment decisions for this condition.


Subject(s)
Decompression, Surgical , Neuroma, Acoustic , Humans , Retrospective Studies , Male , Female , Middle Aged , Neuroma, Acoustic/surgery , Neuroma, Acoustic/pathology , Decompression, Surgical/methods , Adult , Aged , Treatment Outcome , Postoperative Complications/epidemiology , Cohort Studies
5.
Rheumatol Ther ; 2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39073510

ABSTRACT

INTRODUCTION: Juvenile dermatomyositis (JDM) is characterized by persistent non-purulent inflammation in the muscle and skin. The underlying mechanisms still remain uncertain. This study aims to elucidate the mechanism of interleukin-6 (IL-6) activation of Janus kinase/signal transducer and activator of transcription 3 pathway (JAK/STAT3), contributing to the pathogenesis of JDM. METHODS: Serum IL-6 levels were compared between 72 newly diagnosed patients with JDM and the same patient cohort in treatment remission. Single-cell RNA sequencing (scRNA-seq) was employed to identify differential signaling pathway expression in muscle biopsy samples from two patients with JDM and healthy controls. Immunohistochemistry was used to examine differences in STAT3 phosphorylation between JDM and control muscle tissues. In vitro, skeletal muscle cell lines were stimulated with IL-6, and the transcription levels of genes related to mitochondrial calcium channels were quantified via reverse transcription-polymerase chain reaction (RT-PCR). Reactive oxygen species (ROS) production was measured in both IL-6 treated and untreated groups. ROS levels were then compared between IL-6 receptor antagonist pre-treated skeletal muscle cells and untreated cells. RESULTS: IL-6 levels in newly onset patients with JDM were significantly higher compared to the same patient cohort in remission states (p < 0.0001). Serum IL-6 was significantly increased in patients with negative myositis specific antibody (MSA), positive melanoma differentiation associated protein 5 (MDA5) and positive nuclear matrix protein 2 (NXP2), yet not for JDM with positive transcriptional intermediary factor γ (TIF1γ), based on subgroup analysis. ScRNA-seq analysis of muscle biopsies from patients with MDA5-positive JDM and patients with MSA negative JDM revealed abnormal activation of the JAK/STAT3 pathway in skeletal myocytes, macrophages, and vascular endothelial cells. The phosphorylation levels of STAT3 were elevated in active JDM cases. Transcription of the calcium channel modulation gene sarcolipin (SLN) was significantly higher in JDM primary skeletal muscle cells compared to normal cells. In vitro, IL-6 enhanced SLN transcription and induced ROS production, and blocking the IL-6 receptor resulted in decreased ROS generation in skeletal muscle cells. CONCLUSIONS: IL-6/JAK/STAT3 signaling pathway was abnormally activated in patients with JDM. IL-6 may be involved in the pathogenesis of muscle damage by triggering the development of calcium overload and production of ROS. Blockade of the IL-6/JAK/STAT3 pathway can be a potential treatment option for JDM, especially MDA5-positive patients and those who are negative for MSA.

6.
Int J Mol Sci ; 25(14)2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39063061

ABSTRACT

(1) Heart transplantation (HTX) improves the overall survival and functional status of end-stage heart failure patients with cardiomyopathies (CMPs). The majority of CMPs have genetic causes, and the overlap between CMPs and inherited myopathies is well documented. However, the long-term outcome in skeletal muscle function and possibility of an undiagnosed underlying genetic cause of both a cardiac and skeletal pathology remain unknown. (2) Thirty-nine patients were assessed using open and standardized interviews on muscle function, a quality-of-life (EuroQol EQ-5D-3L) questionnaire, and a physical examination (Medical Research Council Muscle scale). Whole-exome sequencing was completed in three stages for those with skeletal muscle weakness. (3) Seven patients (17.9%) reported new-onset muscle weakness and motor limitations. Objective muscle weakness in the upper and lower extremities was seen in four patients. In three of them, exome sequencing revealed pathogenic/likely pathogenic variants in the genes encoding nexilin, myosin heavy chain, titin, and SPG7. (4) Our findings support a positive long-term outcome of skeletal muscle function in HTX patients. However, 10% of patients showed clinical signs of myopathy due to a possible genetic cause. The integration of genetic testing and standardized neurological assessment of motor function during the peri-HTX period should be considered.


Subject(s)
Heart Transplantation , Neuromuscular Diseases , Humans , Heart Transplantation/adverse effects , Male , Female , Middle Aged , Neuromuscular Diseases/genetics , Adult , Quality of Life , Exome Sequencing , Muscle, Skeletal/pathology , Muscle, Skeletal/physiopathology , Aged , Heart Failure/genetics , Heart Failure/surgery , Heart Failure/etiology , Cardiomyopathies/genetics , Cardiomyopathies/etiology , Muscle Weakness/etiology , Muscle Weakness/genetics , Connectin/genetics
7.
Nutrients ; 16(14)2024 Jul 12.
Article in English | MEDLINE | ID: mdl-39064689

ABSTRACT

Phase angle (PhA), a marker of nutritional status obtained by bioelectrical impedance analysis (BIA), is associated with the integrity of cell membranes. Damage to muscle fiber membranes can impact muscle strength, which is related to adverse outcomes in adults with advanced chronic kidney disease (CKD). The main objective of this study was to determine the usefulness of the PhA in identifying muscle weakness in candidates for kidney transplants (KTs). Secondly, it aimed to examine the associations of PhA with other parameters of body composition, exercise performance, and muscle structure. Sensitivity, specificity, and area under the receiver operating characteristics curve were used to evaluate the PhA (index test) as a biomarker of muscle weakness. Muscle strength was estimated with maximal voluntary isometric contraction of the quadriceps (MVCI-Q) of the dominant side. Muscle weakness was defined as MVIC-Q < 40% of body weight. A total of 119 patients were evaluated (mean age 63.7 years, 75.6% men). A phase angle cut-off of 5.1° was identified to classify men with a higher likelihood of having low muscle strength in upper limbs (MVIC-Q 40% of their body weight). Male KT candidates with PhA < 5.1° had poorer exercise capacity, lower muscle strength, less muscle mass, and smaller muscle size. A PhA < 5.1° was significantly associated with an eight-fold higher muscle weakness risk (OR = 8.2, 95%CI 2.3-29.2) in a binary regression model adjusted by age, frailty, and hydration status. Remarkably, PhA is an easily obtainable objective parameter in CKD patients, requiring no volitional effort from the individual. The associations of PhA with aerobic capacity, physical activity, muscle mass, and muscle size underscore its clinical relevance and potential utility in the comprehensive evaluation of these patients.


Subject(s)
Electric Impedance , Kidney Transplantation , Muscle Strength , Muscle Weakness , Humans , Male , Kidney Transplantation/adverse effects , Middle Aged , Muscle Weakness/etiology , Female , Aged , Nutritional Status , Biomarkers , Renal Insufficiency, Chronic/physiopathology , Renal Insufficiency, Chronic/complications , Body Composition , Preoperative Exercise , Muscle, Skeletal/physiopathology , Isometric Contraction , ROC Curve , Cross-Sectional Studies
8.
Pediatr Surg Int ; 40(1): 171, 2024 Jul 03.
Article in English | MEDLINE | ID: mdl-38958763

ABSTRACT

PURPOSE: Split abdominal wall muscle flap (SAWMF) is a technique to repair large defects in congenital diaphragmatic hernia (CDH). A possible objection to this intervention could be any associated abdominal muscle weakness. Our aim is to analyze the evolution of this abdominal muscle wall weakness. METHODS: Retrospective review of CDH repair by SAWMF (internal oblique muscle and transverse) from 2004 to 2023 focusing on the evolution of muscle wall weakness. RESULTS: Eighteen neonates of 148 CDH patients (12,1%) were repaired using SAWMF. Mean gestational age and birth weight were 35.7 ± 3.5 weeks and 2587 ± 816 g. Mean lung-to-head ratio was 1.49 ± 0.28 and 78% liver-up. Seven patients (38%) were prenatally treated by tracheal occlusion. Ninety-four percent of the flaps were used for primary repair and one to repair a recurrence. One patient (5.6%) experienced recurrence. Abdominal muscle wall weakness was present in the form of a bulge. Resolution of weakness at 1, 2 and 3 years was 67%, 89% and 94%, respectively. No patient required treatment for weakness or died. CONCLUSIONS: Abdominal muscular weakness after a split abdominal wall muscle flap repair is not a limitation for its realization since it is asymptomatic and presents a prompt spontaneous resolution. LEVEL OF EVIDENCE: IV.


Subject(s)
Abdominal Muscles , Abdominal Wall , Hernias, Diaphragmatic, Congenital , Muscle Weakness , Surgical Flaps , Humans , Hernias, Diaphragmatic, Congenital/surgery , Hernias, Diaphragmatic, Congenital/complications , Infant, Newborn , Retrospective Studies , Male , Female , Abdominal Wall/surgery , Muscle Weakness/etiology , Muscle Weakness/surgery , Abdominal Muscles/surgery , Herniorrhaphy/methods , Postoperative Complications/surgery , Treatment Outcome
9.
PeerJ ; 12: e17630, 2024.
Article in English | MEDLINE | ID: mdl-38948217

ABSTRACT

Background: Type 2 diabetes mellitus (T2DM) is a prevalent metabolic disorder with systemic implications, potentially affecting musculoskeletal health. This study aimed to assess shoulder muscle strength and joint repositioning accuracy in individuals with T2DM, exploring potential correlations and shedding light on the musculoskeletal consequences of the condition. The objectives were two-fold: (1) to assess and compare shoulder strength and joint repositioning accuracy between individuals with T2DM and asymptomatic counterparts, and (2) to examine the correlation between shoulder strength and joint repositioning accuracy in individuals with T2DM. Methods: A cross-sectional study enrolled 172 participants using the convenience sampling method, including 86 individuals with T2DM and an age-matched asymptomatic group (n = 86). Shoulder strength was assessed using a handheld dynamometer, while joint repositioning accuracy was evaluated with an electronic digital inclinometer. Results: Individuals with T2DM exhibited reduced shoulder muscle strength compared to asymptomatic individuals (p < 0.001). Additionally, joint repositioning accuracy was significantly lower in the T2DM group (p < 0.001). Negative correlations were observed between shoulder strength and joint repositioning accuracy in various directions (ranging from -0.29 to -0.46, p < 0.001), indicating that higher muscle strength was associated with improved joint repositioning accuracy in individuals with T2DM. Conclusion: This study highlights the significant impact of T2DM on shoulder muscle strength and joint repositioning accuracy. Reduced strength and impaired accuracy are evident in individuals with T2DM, emphasizing the importance of addressing musculoskeletal aspects in diabetes management. The negative correlations suggest that enhancing shoulder muscle strength may lead to improved joint repositioning accuracy, potentially contributing to enhanced physical functioning in this population.


Subject(s)
Diabetes Mellitus, Type 2 , Muscle Strength , Muscle Weakness , Humans , Diabetes Mellitus, Type 2/complications , Diabetes Mellitus, Type 2/physiopathology , Diabetes Mellitus, Type 2/drug therapy , Male , Cross-Sectional Studies , Female , Middle Aged , Muscle Weakness/diagnosis , Muscle Weakness/physiopathology , Muscle Weakness/etiology , Shoulder/physiopathology , Proprioception/physiology , Shoulder Joint/physiopathology , Aged , Adult , Range of Motion, Articular
10.
Med Clin (Barc) ; 2024 Jul 02.
Article in English, Spanish | MEDLINE | ID: mdl-38960793

ABSTRACT

BACKGROUND: Acquired critical illness weakness (AWCIP) is the most frequent neuromuscular disease in intensive care medicine departments. Its importance is given by the prolongation of hospital stay and the delayed recovery it causes to patients after hospitalization. The main objective of this study was to investigate the association between neuromuscular blocking agents and the development of acquired weakness in critically ill patients. MATERIAL AND METHODS: We conducted a prospective study of 103 critically ill patients who were periodically monitored with electromyography. RESULTS: The development of AWCIP was observed in 63 patients. The group of patients who developed AWCIP had a significantly higher utilization of neuromuscular blocking agents than the group who did not develop AWCIP [79.4% vs 50%, OR:3.85 (1.63-9.39), p <0.02]; likewise, this group of patients had a longer ICU stay [32 days vs 14 days, OR: 1.11 (1.06-1.17), p <0. 001] and a longer mechanical ventilation time [24 days vs 9 days, OR:1.2 (1.11-1.32), p <0.001]. CONCLUSION: Neuromuscular blocking agents are a factor associated with the occurrence of AWCIP.

12.
J Phys Ther Sci ; 36(7): 396-401, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38952457

ABSTRACT

[Purpose] Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease characterized by decreased transmitter secretion from neuromuscular junctions and nerve terminals. Such cases require physical therapy for independently performing daily activities; however, care must be taken to avoid overwork weakness. This study aimed to investigate the effects of aerobic exercise-based physical therapy in patients with LEMS. [Participants and Methods] We report a case of LEMS with decreased muscle endurance due to inactivity. The participant was subjected to physical therapy with an exercise modality-improved muscle endurance with low-intensity repetitions, while monitoring subjective exercise intensity over time. [Results] The participant achieved independence activities of daily living without developing overwork weakness. [Conclusion] Appropriate physical therapy is an important aspect in treating LEMS.

13.
Eur J Med Res ; 29(1): 379, 2024 Jul 20.
Article in English | MEDLINE | ID: mdl-39033122

ABSTRACT

BACKGROUND: The diagnosis of ICU-acquired weakness (ICUAW) may be delayed due to the complexity of critically ill patients. This study aimed to investigate the value of ultrasound measurements of rectus femoris cross-sectional area (RFCSA) in predicting ICUAW in patients undergoing invasive mechanical ventilation. METHODS: This was a prospective cohort study of patients undergoing mechanical ventilation for at least 48 h. RFCSA was measured using ultrasound in patients upon ICU admission and followed until discharge. Using the Medical Research Council score as the gold standard, we evaluated the diagnostic value of ultrasound measurements in predicting ICUAW. Kaplan-Meier curves were constructed to evaluate and compare the length of ICU stay and duration of invasive mechanical ventilation between patients with and without ICUAW. RESULTS: Among the 76 patients, 34 (44.7%) were diagnosed with ICUAW using the Medical Research Council score as the gold standard. The RFCSA atrophy rate between day 1 and day 3 was significantly higher in the ICUAW group (7.9 ± 2.8% vs. 4.3 ± 2.1%, p < 0.001). By utilizing a cutoff point of 6.9%, we discovered that the RFCSA atrophy rate exhibited excellent diagnostic accuracy in predicting ICUAW, with a sensitivity of 76.5% and specificity of 92.9%. In ICUAW patients diagnosed based on an RFCSA atrophy rate, the proportion of patients with an ICU stay longer than 14 days was 42.9%, which was significantly higher compared to 22.9% in the non-ICUAW group (HR: 1.768; 95% CI 1.128-2.772; p = 0.006). Similarly, the proportion of patients continuing mechanical ventilation at 14 days was 28.6% versus 4.2% between the two groups (HR: 1.988; 95% CI 1.266-3.120; p < 0.001). CONCLUSION: Ultrasound measurements of RFCSA provide a reliable method for diagnosing ICUAW and indicating prognosis in patients undergoing invasive mechanical ventilation.


Subject(s)
Intensive Care Units , Muscle Weakness , Quadriceps Muscle , Respiration, Artificial , Ultrasonography , Humans , Male , Female , Prospective Studies , Middle Aged , Muscle Weakness/etiology , Muscle Weakness/diagnostic imaging , Aged , Quadriceps Muscle/diagnostic imaging , Quadriceps Muscle/pathology , Ultrasonography/methods , Predictive Value of Tests , Critical Illness , Length of Stay
14.
World J Clin Cases ; 12(18): 3288-3290, 2024 Jun 26.
Article in English | MEDLINE | ID: mdl-38983419

ABSTRACT

In this editorial, we discuss an article titled, "Significant risk factors for intensive care unit-acquired weakness: A processing strategy based on repeated machine learning," published in a recent issue of the World Journal of Clinical Cases. Intensive care unit-acquired weakness (ICU-AW) is a debilitating condition that affects critically ill patients, with significant implications for patient outcomes and their quality of life. This study explored the use of artificial intelligence and machine learning techniques to predict ICU-AW occurrence and identify key risk factors. Data from a cohort of 1063 adult intensive care unit (ICU) patients were analyzed, with a particular emphasis on variables such as duration of ICU stay, duration of mechanical ventilation, doses of sedatives and vasopressors, and underlying comorbidities. A multilayer perceptron neural network model was developed, which exhibited a remarkable impressive prediction accuracy of 86.2% on the training set and 85.5% on the test set. The study highlights the importance of early prediction and intervention in mitigating ICU-AW risk and improving patient outcomes.

15.
World J Clin Cases ; 12(18): 3285-3287, 2024 Jun 26.
Article in English | MEDLINE | ID: mdl-38983426

ABSTRACT

Intensive care unit-acquired weakness (ICU-AW) significantly hampers patient recovery and increases morbidity. With the absence of established preventive strategies, this study utilizes advanced machine learning methodologies to unearth key predictors of ICU-AW. Employing a sophisticated multilayer perceptron neural network, the research methodically assesses the predictive power for ICU-AW, pinpointing the length of ICU stay and duration of mechanical ventilation as pivotal risk factors. The findings advocate for minimizing these elements as a preventive approach, offering a novel perspective on combating ICU-AW. This research illuminates critical risk factors and lays the groundwork for future explorations into effective prevention and intervention strategies.

16.
Neurocrit Care ; 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38982001

ABSTRACT

BACKGROUND: The diagnosis of intensive care unit (ICU)-acquired weakness (ICUAW) and critical illness neuromyopathy (CINM) is frequently hampered in the clinical routine. We evaluated a novel panel of blood-based inflammatory, neuromuscular, and neurovascular biomarkers as an alternative diagnostic approach for ICUAW and CINM. METHODS: Patients admitted to the ICU with a Sequential Organ Failure Assessment score of ≥ 8 on 3 consecutive days within the first 5 days as well as healthy controls were enrolled. The Medical Research Council Sum Score (MRCSS) was calculated, and motor and sensory electroneurography (ENG) for assessment of peripheral nerve function were performed at days 3 and 10. ICUAW was defined by an MRCSS < 48 and CINM by pathological ENG alterations, both at day 10. Blood samples were taken at days 3, 10, and 17 for quantitative analysis of 18 different biomarkers (white blood cell count, C-reactive protein, procalcitonin, C-terminal agrin filament, fatty-acid-binding protein 3, growth and differentiation factor 15, syndecan 1, troponin I, interferon-γ, tumor necrosis factor-α, interleukin-1α [IL-1α], IL-1ß, IL-4, IL-6, IL-8, IL-10, IL-13, and monocyte chemoattractant protein 1). Results of the biomarker analysis were categorized according to the ICUAW and CINM status. Clinical outcome was assessed after 3 months. RESULTS: Between October 2016 and December 2018, 38 critically ill patients, grouped into ICUAW (18 with and 20 without) and CINM (18 with and 17 without), as well as ten healthy volunteers were included. Biomarkers were significantly elevated in critically ill patients compared to healthy controls and correlated with disease severity and 3-month outcome parameters. However, none of the biomarkers enabled discrimination of patients with and without neuromuscular impairment, irrespective of applied classification. CONCLUSIONS: Blood-based biomarkers are generally elevated in ICU patients but do not identify patients with ICUAW or CINM. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT02706314.

17.
World J Clin Cases ; 12(19): 3665-3670, 2024 Jul 06.
Article in English | MEDLINE | ID: mdl-38994273

ABSTRACT

In this editorial, comments are made on an interesting article in the recent issue of the World Journal of Clinical Cases by Wang and Long. The authors describe the use of neural network model to identify risk factors for the development of intensive care unit (ICU)-acquired weakness. This condition has now become common with an increasing number of patients treated in ICUs and continues to be a source of morbidity and mortality. Despite identification of certain risk factors and corrective measures thereof, lacunae still exist in our understanding of this clinical entity. Numerous possible pathogenetic mechanisms at a molecular level have been described and these continue to be increasing. The amount of retrievable data for analysis from the ICU patients for study can be huge and enormous. Machine learning techniques to identify patterns in vast amounts of data are well known and may well provide pointers to bridge the knowledge gap in this condition. This editorial discusses the current knowledge of the condition including pathogenesis, diagnosis, risk factors, preventive measures, and therapy. Furthermore, it looks specifically at ICU acquired weakness in recipients of lung transplantation, because - unlike other solid organ transplants- muscular strength plays a vital role in the preservation and survival of the transplanted lung. Lungs differ from other solid organ transplants in that the proper function of the allograft is dependent on muscle function. Muscular weakness especially diaphragmatic weakness may lead to prolonged ventilation which has deleterious effects on the transplanted lung - ranging from ventilator associated pneumonia to bronchial anastomotic complications due to prolonged positive pressure on the anastomosis.

18.
J Neuromuscul Dis ; 2024 Jul 04.
Article in English | MEDLINE | ID: mdl-38995797

ABSTRACT

Background: Congenital myasthenic syndromes (CMS) are a group of rare but often treatable inherited disorders of neuromuscular transmission characterized by fatigable skeletal muscle weakness. In this paper we present the largest phenotypic analysis to date of a cohort of patients carrying the pathogenic variant c.1327delG in the CHRNE gene, leading to CHRNE-CMS. Objective: This study aims to identify the phenotypic variability in CMS associated with c.1327delG mutation in the CHRNE gene. Methods: Disease specific symptoms were assessed using specific standardized tests for autoimmune myasthenia (Quantitative Myasthenia Gravis score) as well as patient-reported scales for symptom severity. Evaluated clinical manifestations included ocular symptoms (ophthalmoparesis and ptosis), bulbar weakness, axial muscle weakness, proximal and distal muscle weakness, and respiratory function. Patients were allocated into three groups according to clinical impression of disease severity: mild, moderate, and severe. Results: We studied 91 Bulgarian Roma patients, carrying the same causative homozygous CHRNE c.1327delG mutation. Bulbar weakness was present in patients throughout all levels of severity of CHRNE-CMS in this study. However, difficulties in eating and swallowing are more prominent characteristics in the moderate and severe clinical phenotypes. Diplopia and ptosis resulting from fatigue of the extraocular muscles were permanent features regardless of disease severity or age. Levels of axial, proximal and distal muscle weakness were variable between disease groups. The statistical analysis showed significant differences between the patients in the three groups, emphasizing a possible variation in symptom manifestation in the evaluated patient population despite the disease originating from the same genetic mutation. Impairment of respiratory function was more prominent in severely affected patients, which might result from loss of compensatory muscle function in those individuals. Conclusion: Results from our study indicate significant phenotypic heterogeneity leading to mild, moderate, or severe clinical manifestation in CHRNE-CMS, despite the genotypic homogeneity.

19.
Iran J Otorhinolaryngol ; 36(4): 527-535, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39015690

ABSTRACT

Introduction: Bell's palsy is one of the most concerning complications of the COVID vaccine that has impacted vaccine acceptance among the general population. These vaccines were introduced to provide immunity against the SARS-CoV-2 coronavirus and have been found to be quite effective. Little did we know that Bell's palsy could be one of its serious complications. Materials and Methods: We used various search engines to gather data in the form of a case series and case reports related to patients who were affected by the vaccine and had developed Bell's palsy. Results: A total of eleven case reports and 4 case series were included in the analysis. The vaccines mentioned in the case reports were Pfizer, Moderna, Sinovac, AstraZeneca, and Janssen, while the case series included Pfizer and Sinovac. The majority of patients were female and aged between 31-40 years. Right-sided laterality was observed in 45.45% of patients, left-sided laterality in 45.45% of patients, and bilateral laterality in 9.1% of patients. Three patients had a history of Bell's palsy or stroke. After treatment, three patients showed partial improvement, six patients fully recovered, and the status of two patients was unknown. Conclusions: Bell's palsy is a rare complication that can occur after receiving the COVID-19 vaccine. This review aims to increase awareness about this rare adverse event of the vaccine so that it can be properly addressed and managed. Additionally, it will serve as a foundation for future research on the administration of the COVID-19 vaccine.

20.
BMC Neurol ; 24(1): 243, 2024 Jul 15.
Article in English | MEDLINE | ID: mdl-39009960

ABSTRACT

Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.


Subject(s)
Arthritis, Rheumatoid , Meningitis , Humans , Female , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/diagnosis , Aged, 80 and over , Meningitis/diagnosis , Meningitis/complications , Aged , Male , Magnetic Resonance Imaging
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