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2.
A A Pract ; 18(7): e01812, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38958292

ABSTRACT

A 53-year-old woman underwent a thoracic epidural placement for a scheduled laparotomy. Postoperatively the patient had no appreciable epidural level after multiple epidural boluses and was noted to be severely hypotensive with right upper extremity weakness and numbness. She subsequently developed right-sided Horner's syndrome with worsening right upper extremity weakness and decreased sensation from C6 to T1. She regained full motor and sensory function in her right upper extremity with epidural removal. This unusual case raises awareness of the variability in the presentation of subdural spread and provides an example of an epidural complication that can mimic a cerebrovascular accident (CVA).


Subject(s)
Analgesia, Epidural , Anesthetics, Local , Horner Syndrome , Muscle Weakness , Paresthesia , Stroke , Humans , Female , Middle Aged , Horner Syndrome/etiology , Horner Syndrome/chemically induced , Analgesia, Epidural/adverse effects , Paresthesia/etiology , Muscle Weakness/etiology , Anesthetics, Local/adverse effects , Anesthetics, Local/administration & dosage , Upper Extremity/surgery , Diagnosis, Differential
3.
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
4.
Neurology ; 103(3): e209708, 2024 Aug 13.
Article in English | MEDLINE | ID: mdl-38991193

ABSTRACT

The clinical assessment of patients with proximal muscle weakness represents a frequent yet intricate challenge. We present the case of a 60-year-old woman who experienced a progressive, symmetrical weakness in proximal limbs and bulbar muscles over 6 months. Notable clinical findings included bilateral ophthalmoparesis; widespread muscle atrophy; and pronounced weakness in craniobulbar, cervical, and proximal muscles, more severe than in distal ones. We elucidate a methodical diagnostic approach, focusing on constructing a comprehensive differential diagnosis and identifying potential causes of proximal muscle weakness. Special emphasis is placed on exploring the etiologies in cases presenting with both progressive muscle weakness and ophthalmoparesis. We further describe the role of muscle biopsy results and their integration with genetic testing outcomes.


Subject(s)
Muscle Weakness , Ophthalmoplegia , Humans , Female , Middle Aged , Muscle Weakness/etiology , Muscle Weakness/diagnosis , Ophthalmoplegia/diagnosis , Clinical Reasoning , Diagnosis, Differential , Disease Progression
5.
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
6.
Front Immunol ; 15: 1378130, 2024.
Article in English | MEDLINE | ID: mdl-39021570

ABSTRACT

Brachio-cervical inflammatory myopathy (BCIM) is a rare inflammatory myopathy characterized by dysphagia, bilateral upper limb atrophy, limb-girdle muscle weakness, and myositis-specific antibody (MSA) negativity. BCIM has a low incidence and is commonly associated with autoimmune diseases. We present a case report of a 55-year-old man with progressive upper limb weakness and atrophy, diagnosed with flail arm syndrome (FAS). The initial electromyography revealed extensive spontaneous muscle activity and increased duration of motor unit potentials (MUPs). During follow-up, evidence of myogenic damage was observed, as indicated by a decreased duration of MUPs in the right biceps muscle. Laboratory and genetic testing ruled out hereditary or acquired diseases. Negative serological antibodies for myasthenia gravis. Hereditary or acquired diseases were ruled out through laboratory and genetic testing. Whole-body muscle magnetic resonance imaging (MRI) showed extensive edema and fat replacement in the bilateral upper limbs, scapular, and central axis muscles, while the lower extremities were relatively mildly affected. Muscle biopsy revealed numerous foci of inflammatory cells distributed throughout the muscle bundle, with predominant CD20, CD138, and CD68 expression, accompanied by a light infiltration of CD3 and CD4 expression. The muscle weakness improved with the combination of oral prednisone (initially 60 mg/day, tapered) and methotrexate (5 mg/week) treatment.


Subject(s)
Diagnostic Errors , Myositis , Humans , Middle Aged , Male , Myositis/diagnosis , Myositis/immunology , Arm , Muscle, Skeletal/pathology , Muscle, Skeletal/immunology , Muscle Weakness/diagnosis , Muscle Weakness/etiology , Muscular Atrophy/diagnosis , Electromyography , Magnetic Resonance Imaging
7.
Pediatr Rheumatol Online J ; 22(1): 66, 2024 Jul 22.
Article in English | MEDLINE | ID: mdl-39039532

ABSTRACT

BACKGROUND: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. METHODS: We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. RESULTS: One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. CONCLUSIONS: We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.


Subject(s)
Dermatomyositis , Registries , Humans , Dermatomyositis/epidemiology , Dermatomyositis/diagnosis , Female , Child , Male , Spain/epidemiology , Prognosis , Retrospective Studies , Child, Preschool , Autoantibodies/blood , Muscle Weakness/etiology , Muscle Weakness/epidemiology , Deglutition Disorders/etiology , Deglutition Disorders/epidemiology
8.
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
9.
BMC Pediatr ; 24(1): 415, 2024 Jun 26.
Article in English | MEDLINE | ID: mdl-38926640

ABSTRACT

BACKGROUND: Kawasaki disease (KD) is an acute systemic immune vasculitis affecting multiple organs and systems in children, and is prevalent in children under 5 years of age. Muscular weakness is a rare manifestation of KD, and only 11 pediatric patients with KD combined with muscular weakness have been reported, of which evidence of myositis was found in 2/3 of the patients, and 1/3 could not be explained by myositis, the mechanism of which is still unclear. Cases of KD combined with bladder retention are even more rare, and there has been only 1 case report of KD combined with bladder retention in a child with no previous underlying disease. CASE PRESENTATION: We report a 22-month-old Asian child with incomplete Kawasaki disease (IKD) who initially presented with fever and progressive muscular weakness in the lower extremities, followed by the bladder and bowel retention abnormalities and rapid onset of heart failure, respiratory failure and shock. The child developed coronary artery ectasia (CAA) without the main clinical features of KD such as rash, conjunctival congestion, desquamation of the extremity endings, orofacial changes and enlarged lymph nodes in the neck. Creatine kinase and electromyography were normal. Temperature gradually normalized and muscle strength recovered slightly after intravenous immunoglobulin. The child could be helped to walk after 1 week of aspirin combined with steroid therapy. CONCLUSIONS: We present the case of a 22-month-old child with IKD. The child began with progressive muscular weakness in the extremities, followed by the bladder and bowel retention abnormalities, and rapidly developed heart failure, respiratory failure, and shock. Despite early failure to detect the disease, the child recovered rapidly and had a favorable prognosis. KD comorbidities with muscular weakness as the main manifestation are uncommon. This is the first case report of IKD combined with both muscular weakness and bladder and bowel retention, which may provide clinicians with diagnostic and therapeutic ideas, as well as a basis for future exploration of the mechanisms of KD combined with muscular weakness or bladder and bowel retention abnormalities.


Subject(s)
Mucocutaneous Lymph Node Syndrome , Muscle Weakness , Urinary Retention , Humans , Infant , Immunoglobulins, Intravenous/therapeutic use , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Muscle Weakness/etiology , Urinary Retention/etiology
11.
J Neurol Sci ; 462: 123098, 2024 Jul 15.
Article in English | MEDLINE | ID: mdl-38879961

ABSTRACT

BACKGROUND: Several studies have reported disproportionate wasting of the flexor muscles of the lower limbs (LL) compared to the extensors in patients with amyotrophic lateral sclerosis (ALS). However, these studies have involved small sample sizes (n ã€ˆ100), and their findings have been inconsistent. Thus, it remains uncertain whether a distinct pattern of LL muscle weakness is specific to ALS. AIMS: To investigate the muscle weakness pattern in the LL at the knee, ankle, and toes in a large cohort of ALS patients and evaluate the relationship between the pattern of muscle strength and the extent of upper (UMN) and lower (LMN) motoneuron impairment. MATERIAL AND METHODS: The strength of flexor and extensor muscle was evaluated in 1250 legs of newly diagnosed ALS patients at the knee, ankle, and foot toes. UMN and LMN burden were assessed using validated scores. Within-subjects ANOVA considering the type of muscle (flexor/extensor) and anatomical sites (knee/ankle/toes) and mixed-factorial ANOVA were conducted to explore the impact of UMN and LMN impairments on the muscle weakness pattern. RESULTS: Muscle strength showed a significant decline from proximal to distal regions. Indeed both flexor and extensor muscles at the knee outperformed those at the ankle and toes. Within each site, extensor muscles exhibited less strength than flexor, except at the knee. Patients with heightened UMN impairment showed a more marked difference between flexors and extensors within each site, with extensor muscles being more compromised at the ankle and toes. Higher LMN impairment corresponded to a more pronounced weakness in flexor muscles at the ankle and toes compared to those at the knee. CONCLUSIONS: The extensor muscle at the knee and the flexors at the foot and toes displayed relative resistance to ALS disease. UMN impairment amplified the differences between flexor and extensor muscles within each site, while LMN impairment demonstrated a clear distal-to-proximal vulnerability.


Subject(s)
Amyotrophic Lateral Sclerosis , Lower Extremity , Motor Neurons , Muscle Strength , Muscle, Skeletal , Humans , Amyotrophic Lateral Sclerosis/physiopathology , Male , Female , Middle Aged , Muscle, Skeletal/physiopathology , Motor Neurons/physiology , Aged , Muscle Strength/physiology , Lower Extremity/physiopathology , Muscle Weakness/physiopathology , Muscle Weakness/etiology , Adult , Cohort Studies
12.
Neurology ; 103(1): e209561, 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38833636

ABSTRACT

Hand weakness is a frequent chief concern in neurology practice. We report a case of a 55-year-old woman presenting with a chronic, gradually worsening right hand weakness and atrophy, selectively affecting the thenar muscles, without any sensory symptoms. She had a history of carpal tunnel syndrome and previously underwent surgical carpal tunnel release. This case delves into the differential diagnosis of hand weakness and atrophy, emphasizing the significance of myotomal innervation in intrinsic hand muscles. Furthermore, it outlines a systematic approach to diagnosing an uncommon cause for a common clinical presentation, offering a comprehensive differential diagnosis, and exploring various possible causes.


Subject(s)
Hand , Muscle Weakness , Humans , Female , Middle Aged , Muscle Weakness/etiology , Muscle Weakness/diagnosis , Clinical Reasoning , Diagnosis, Differential , Muscular Atrophy/etiology , Muscular Atrophy/diagnosis , Atrophy , Carpal Tunnel Syndrome/diagnosis
13.
J Bodyw Mov Ther ; 39: 214-217, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38876628

ABSTRACT

BACKGROUND: Motor impairments are common consequences of traumatic brain injury (TBI). It affects the individuals' participation in activities of daily living (ADLs). Dry needling treatment (DNT) uses a specialized needle to alter cortical activity. This case study aims to examine the effects of DNT on spasticity, balance, gait, and self-independence in a single patient with TBI. CASE DESCRIPTION: A twenty-six-year-old male with a history of TBI, resulting in muscle weakness on the right side of the body, spasticity, distributed balance, and difficulties with independent gait participated in this study. The Berg balance scale (BBS), 6-min walk test (6MWT), Modified Ashworth Scale (MAS), and Functional Independence Measure (FIM) were used to evaluate balance, gait, spasticity, and functional performance, respectively. OUTCOME: After 36 DNT sessions extended over 12 weeks, the patient demonstrated improvements in spasticity, balance, gait, and functional capacity both immediately after the intervention and at the 4-week follow-up. CONCLUSION: This case study demonstrates that DNT is considered a novel intervention for treating spasticity and improving balance, gait, and functional capacity post-TBI. Further research is recommended to verify these findings.


Subject(s)
Brain Injuries, Traumatic , Dry Needling , Muscle Spasticity , Postural Balance , Humans , Male , Brain Injuries, Traumatic/complications , Brain Injuries, Traumatic/rehabilitation , Muscle Spasticity/therapy , Muscle Spasticity/rehabilitation , Muscle Spasticity/etiology , Postural Balance/physiology , Dry Needling/methods , Adult , Gait/physiology , Activities of Daily Living , Muscle Weakness/rehabilitation , Muscle Weakness/etiology , Muscle Weakness/therapy
14.
Harefuah ; 163(6): 376-381, 2024 Jun.
Article in Hebrew | MEDLINE | ID: mdl-38884292

ABSTRACT

INTRODUCTION: Fibromyalgia syndrome (FMS) is a chronic pain syndrome, prevalent in women more than men. The main symptoms are widespread musculoskeletal pain, fatigue, and weakness. To date, the pathophysiological mechanisms are unclear, and there are several pathogenic theories elucidating this condition. In this review, we summarized articles published in the past few years, regarding the effect of musculoskeletal dysfunction on FMS. We focused on the musculoskeletal system and central nervous system (CNS) disarrays.


Subject(s)
Fibromyalgia , Fibromyalgia/physiopathology , Humans , Female , Male , Fatigue/physiopathology , Fatigue/etiology , Chronic Pain/physiopathology , Chronic Pain/etiology , Central Nervous System/physiopathology , Musculoskeletal Pain/physiopathology , Musculoskeletal Pain/etiology , Muscle Weakness/physiopathology , Muscle Weakness/etiology
15.
Mol Metab ; 86: 101976, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38925248

ABSTRACT

OBJECTIVES: A high proportion of women with advanced epithelial ovarian cancer (EOC) experience weakness and cachexia. This relationship is associated with increased morbidity and mortality. EOC is the most lethal gynecological cancer, yet no preclinical cachexia model has demonstrated the combined hallmark features of metastasis, ascites development, muscle loss and weakness in adult immunocompetent mice. METHODS: Here, we evaluated a new model of ovarian cancer-induced cachexia with the advantages of inducing cancer in adult immunocompetent C57BL/6J mice through orthotopic injections of EOC cells in the ovarian bursa. We characterized the development of metastasis, ascites, muscle atrophy, muscle weakness, markers of inflammation, and mitochondrial stress in the tibialis anterior (TA) and diaphragm ∼45, ∼75 and ∼90 days after EOC injection. RESULTS: Primary ovarian tumour sizes were progressively larger at each time point while severe metastasis, ascites development, and reductions in body, fat and muscle weights occurred by 90 Days. There were no changes in certain inflammatory (TNFα), atrogene (MURF1 and Atrogin) or GDF15 markers within both muscles whereas IL-6 was increased at 45 and 90 Day groups in the diaphragm. TA weakness in 45 Day preceded atrophy and metastasis that were observed later (75 and 90 Day, respectively). The diaphragm demonstrated both weakness and atrophy in 45 Day. In both muscles, this pre-severe-metastatic muscle weakness corresponded with considerable reprogramming of gene pathways related to mitochondrial bioenergetics as well as reduced functional measures of mitochondrial pyruvate oxidation and creatine-dependent ADP/ATP cycling as well as increased reactive oxygen species emission (hydrogen peroxide). Remarkably, muscle force per unit mass at 90 days was partially restored in the TA despite the presence of atrophy and severe metastasis. In contrast, the diaphragm demonstrated progressive weakness. At this advanced stage, mitochondrial pyruvate oxidation in both muscles exceeded control mice suggesting an apparent metabolic super-compensation corresponding with restored indices of creatine-dependent adenylate cycling. CONCLUSIONS: This mouse model demonstrates the concurrent development of cachexia and metastasis that occurs in women with EOC. The model provides physiologically relevant advantages of inducing tumour development within the ovarian bursa in immunocompetent adult mice. Moreover, the model reveals that muscle weakness in both TA and diaphragm precedes severe metastasis while weakness also precedes atrophy in the TA. An underlying mitochondrial bioenergetic stress corresponded with this early weakness. Collectively, these discoveries can direct new research towards the development of therapies that target pre-atrophy and pre-severe-metastatic weakness during EOC in addition to therapies targeting cachexia.


Subject(s)
Cachexia , Disease Models, Animal , Mice, Inbred C57BL , Mitochondria , Muscle Weakness , Ovarian Neoplasms , Animals , Cachexia/metabolism , Cachexia/etiology , Cachexia/pathology , Female , Mice , Ovarian Neoplasms/metabolism , Ovarian Neoplasms/pathology , Ovarian Neoplasms/complications , Muscle Weakness/metabolism , Muscle Weakness/etiology , Mitochondria/metabolism , Mitochondria/pathology , Muscle, Skeletal/metabolism , Muscle, Skeletal/pathology , Muscular Atrophy/metabolism , Muscular Atrophy/etiology , Muscular Atrophy/pathology , Neoplasm Metastasis , Carcinoma, Ovarian Epithelial/metabolism , Carcinoma, Ovarian Epithelial/pathology , Cell Line, Tumor
16.
BMJ Case Rep ; 17(5)2024 May 09.
Article in English | MEDLINE | ID: mdl-38724210

ABSTRACT

Hyperkalaemia is one of the common electrolyte imbalances dealt with in the emergency department and is caused by extracellular accumulation of potassium ions above normal limits usually greater than 5.0-5.5 mmol/L. It is found in a total of 1-10% of hospitalised patients usually associated with chronic kidney disease and heart failure. The presentation can range from being asymptomatic to deadly arrhythmias. The appearance of symptoms depends on the rate of change rather than just the numerical values. The rare presentation includes periodic paralysis characterised by the sudden onset of short-term muscle weakness, stiffness or paralysis. Management goals are directed towards reducing potassium levels in emergency settings and later on avoiding the triggers for future attacks. In this case, we present a man in his 50s with the generalised weakness later on diagnosed as hyperkalaemic periodic paralysis secondary to tumour lysis syndrome. Emergency physicians dealing with common electrolyte imbalances should keep a sharp eye on their rare presentation and their precipitating factors and should act accordingly.


Subject(s)
Emergency Service, Hospital , Hyperkalemia , Humans , Male , Hyperkalemia/etiology , Hyperkalemia/diagnosis , Hyperkalemia/therapy , Middle Aged , Paralysis, Hyperkalemic Periodic/diagnosis , Paralysis, Hyperkalemic Periodic/complications , Potassium/blood , Potassium/therapeutic use , Diagnosis, Differential , Muscle Weakness/etiology
17.
J Pediatr Orthop ; 44(7): e657-e661, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-38689487

ABSTRACT

BACKGROUND: We report changes in the natural history of hip instability with nusinersen treatment among patients with spinal muscular atrophy (SMA) type II after onset of weakness, historically wheelchair-bound but now potentially ambulatory in the era of disease-modifying therapy. METHODS: Patients with genetically confirmed diagnoses of SMA type II who received intrathecal nusinersen from January 1, 2018, to June 30, 2022, were screened for inclusion. Patients with less than 6 months of follow-up, or prior hip surgeries were excluded. Primary clinical outcome measures included scores from Hammersmith motor functional scale expanded (HMFSE), revised upper limb module (RULM), 6-minute walk test (6MWT), and ambulatory status. Radiographic outcomes, including Reimer migration index, the presence of scoliosis, and pelvic obliquity, were also assessed. Secondary outcomes involved comparisons with a historical cohort of SMA type II patients treated at our institution who never received nusinersen. RESULTS: Twenty hips from 5 boys and 5 girls were included in the analysis, with a mean follow-up of 3 years and 8 months. The median age at time of nusinersen initiation was 6.8 years old, ranging between 2.5 and 10.3 years. All patients developed lower limb motor weakness before nusinersen initiation. After treatment with nusinersen, 1 previously stable hip (5%) developed subluxation, 15 hips (75%) remain subluxated, 3 hips (15%) remain dislocated, and 1 hip (5%) remained stable, with a statistically significant difference between the pretreatment and posttreatment groups ( P <0.01). Six patients (60%) were ambulatory at latest follow-up. Six patients (60%) had improved ambulatory ability; 2 had static ambulatory ability (20%); and 2 had deterioration in their walking ability. The median HFMSE score improved from 18.5 (range 0 to 46) to 22 (range 0 to 49) ( P =0.813), whereas the median RULM score improved from 17 (range 2 to 28) to 21.5 (range 5 to 37), which was statistically significant ( P =0.007). CONCLUSIONS: Hip instability persists despite treatment with nusinersen among patients with SMA type II who received nusinersen after onset of lower limb weakness. LEVEL OF EVIDENCE: Therapeutic Level IV.


Subject(s)
Disease Progression , Joint Instability , Muscle Weakness , Oligonucleotides , Spinal Muscular Atrophies of Childhood , Humans , Male , Female , Child , Oligonucleotides/administration & dosage , Oligonucleotides/therapeutic use , Child, Preschool , Muscle Weakness/etiology , Spinal Muscular Atrophies of Childhood/drug therapy , Spinal Muscular Atrophies of Childhood/physiopathology , Joint Instability/drug therapy , Retrospective Studies , Hip Joint/physiopathology , Follow-Up Studies
18.
J Clin Neurosci ; 125: 7-11, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38733901

ABSTRACT

This study measures the impact of preoperative motor weakness (MW) on Patient-Reported Outcome Measures (PROMs) in lateral lumbar interbody fusion (LLIF) patients. Retrospectively-sourced data from a prospectively-maintained, single-surgeon database created two cohorts of LLIF patients: patients with/without documented MW. Demographics/perioperative characteristics/PROMs were collected preoperatively and at six-weeks/final follow-up (FF). Studied outcomes were Patient-Reported Outcomes Measurement Information System Physical Function (PROMIS-PF), 12-Item Short Form (SF-12) Physical/Mental Component Score (PCS/MCS), Patient Health Questionnaire (PHQ-9), Visual Analog Scale Back/Leg Pain (VAS-BP/LP), and Oswestry Disability Index (ODI). Multivariable linear/logistic regression calculated/compared intercohort minimum clinically important difference (MCID). Mean postoperative follow-up time was 11.5 ± 7.52 months. In total, 214 LLIF patients from December 2010 to May 2023 were included, with 149 having documented MW. In Table 1, self-reported gender was significant between cohorts (p < 0.025). Other significant demographic characteristics were smoker status (p < 0.002), diabetes (p < 0.016), and CCI score (p < 0.011). Table 2 shows notably significant perioperative characteristics: spinal pathology (degenerative spondylolisthesis/foraminal stenosis/herniated nucleus pulposus) (p < 0.005, all), estimated blood loss/length of stay/postoperative day (POD)-zero narcotic consumption (p < 0.001, all). Table 3 outcomes/MCID achievement percentages demonstrated insignificant intercohort differences besides a weakly significant FF ODI score (p < 0.036). MW, a frequently reported symptom in spine surgery, is poorly studied in LLIF patients. Thus, this study evaluates MW impact on PROMs and notes no significant differences. However, one exception regarding FF disability scores was recorded. MW did not affect MCID achievement for our patient population. Therefore, the preliminary findings suggest preoperative MW imparts minimal influence on PROMs/MCID in LLIF patients.


Subject(s)
Lumbar Vertebrae , Muscle Weakness , Patient Reported Outcome Measures , Spinal Fusion , Humans , Spinal Fusion/adverse effects , Male , Female , Middle Aged , Lumbar Vertebrae/surgery , Muscle Weakness/etiology , Aged , Retrospective Studies , Treatment Outcome , Disability Evaluation
19.
Neurophysiol Clin ; 54(4): 102984, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38759365

ABSTRACT

Botulinum neurotoxin serotype A (BoNT-A) has several therapeutic indications such as spasticity and dystonia. Although its use is generally considered safe, a systemic diffusion can lead to systemic complications, and a botulism-like syndrome can occur after intramuscular injections. Herein, two adult cases who developed general muscle weakness after a BoNT-A intramuscular injection are reported. Both presented with a progressive decrement on low-frequency (LF) repetitive nerve stimulation (RNS). It is suggested that a progressive decrement on LF-RNS in muscles distant from the injection site strongly supports the diagnosis of iatrogenic botulism.


Subject(s)
Botulinum Toxins, Type A , Botulism , Adult , Humans , Botulinum Toxins, Type A/adverse effects , Botulism/diagnosis , Injections, Intramuscular/adverse effects , Muscle Weakness/etiology , Neuromuscular Agents/adverse effects , Neuromuscular Junction/drug effects , Neuromuscular Junction/physiopathology , Synaptic Transmission/drug effects
20.
JBJS Case Connect ; 14(2)2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38788051

ABSTRACT

CASE: An 8-year-old girl with a history of acute flaccid paralysis presented with chronic valgus drop foot causing tripping and falling. Traditionally surgical correction of this deformity is accomplished by transferring the posterior tibialis tendon to enhance dorsiflexion. The authors describe a new technique which transfers the peroneus longus tendon to the dorsum of the foot in a patient with weakness of the posterior tibialis muscle. The patient's drop foot and gait were improved at the 22-month follow-up. CONCLUSION: Successful transfer of the peroneus longus was accomplished with improved limb clearance during gait and coronal alignment in stance.


Subject(s)
Gait Disorders, Neurologic , Muscle Weakness , Tendon Transfer , Humans , Female , Child , Tendon Transfer/methods , Gait Disorders, Neurologic/surgery , Gait Disorders, Neurologic/etiology , Muscle Weakness/surgery , Muscle Weakness/etiology
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