Long-term Health-related Quality of Life and Physical Function of COVID-19 Survivors with ICU-acquired Weakness.

Objectives: This study examined the long-term health-related quality of life (HRQOL) and physical function of coronavirus 2019 (COVID-19) survivors diagnosed with intensive care unit-acquired weakness (ICU-AW). The correlation between muscle weakness at ICU discharge and HRQOL was assessed. Methods: A retrospective study was conducted on COVID-19 patients admitted to the ICU at Hyogo Medical University Hospital between January 2021 and November 2021. The HRQOL was evaluated using the SF-36 questionnaire, and physical function, including muscle strength assessed by the Medical Research Council Sum Score (MRC-SS), grip strength, and the 6-min walk distance (6MWD), were assessed 18 months after the onset. ICU-AW was diagnosed in patients with an MRC-SS of less than 48 at ICU discharge. We investigated the correlations between the MRC-SS at ICU discharge and the long-term clinical outcomes. Results: We included 26 patients, with 13 having ICU-AW. In the long-term follow-up, the ICU-AW group had significantly lower scores than the no ICU-AW group in the SF-36 subscales such as Physical Functioning (PF), Role Limitation-Physical (RP), Bodily Pain (BP), Vitality (VT), Social Functioning (SF), and Role Limitation-Emotional (RE), as well as in the Physical Component Summary Score (PCS). The muscle strength was also decreased in the ICU-AW group. The MRC-SS at ICU discharge was positively correlated with PF, RP, BP, SF, RE, and PCS in SF-36 at the 18-month follow-up. Conclusions: COVID-19 survivors with ICU-AW experienced a long-term decline in HRQOL, and muscle weakness at ICU discharge was correlated with the long-term HRQOL.

Qualitative interviews of patients with COPD and muscle weakness enrolled in a clinical trial evaluating a new anabolic treatment: patient perspectives of disease experience, trial participation and outcome assessments.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) and muscle weakness can cause impaired physical function, significantly impacting patients' health-related quality of life (HRQoL). Loss of muscle strength is usually assessed through clinical and performance outcome (PerfO) assessments, which consists of tasks performed in a standardized manner, providing evidence of a patient's functional ability. However, evidence documenting the patient experience of COPD and muscle weakness is limited. METHODS: This two-stage qualitative study used semi-structured interviews in patients aged 45-80 years with COPD (post-bronchodilator forced expiratory volume in 1s [FEV1]/forced vital capacity ratio < 0.70, and FEV1% predicted of 30-80%) and muscle weakness. In Stage 1, 30-minute concept elicitation interviews were conducted with participants recruited across three US sites to explore impacts on physical functioning and activities of daily living. In Stage 2, interviews were performed with participants exiting a Phase IIa trial investigating the efficacy of a selective androgen receptor modulator (GSK2881078) on leg strength, whereby PerfOs were used to evaluate strength and physical functioning endpoints. These participants completed either 60-minute in-depth (n = 32) or 15-minute confirmatory (n = 35) interviews exploring trial experience, completion of outcome measures, disease experience and treatment satisfaction. RESULTS: In Stage 1 (n = 20), most participants described their muscles as weak (83.3%). Difficulties with walking (100%) and lifting heavy objects (90%) were reported. In Stage 2, 60-minute interviews, all participants (n = 32) reported a positive trial experience. Most participants reported that the home exercise program was easy to fit into daily life (77.8%), the PROactive daily diary was easy to complete (100%) and wearable sensors were easy to use (65.6%). However, technical issues were reported (71%), and few participants (19.4%) found physical assessments easy to complete. Improvements in muscle strength and functional limitations were reported by most participants. The shorter 15-minute confirmatory interviews (n = 35) supported the in-depth interview results. CONCLUSION: The qualitative interviews generated in-depth evidence of key concepts relevant to patients with COPD and muscle weakness and support the assessments of patient strength and physical function as outcome measures in this population in future studies. TRIAL NUMBER: GSK Stage 1: 206869; Stage 2: 200182, NCT03359473; Registered December 2, 2017, https://clinicaltrials.gov/ct2/show/NCT03359473 .

ß-hydroxybutyrate exposure restores mitochondrial function in skeletal muscle satellite cells of critically ill patients.

BACKGROUND & AIM: Dysfunction of skeletal muscle satellite cells might impair muscle regeneration and prolong ICU-acquired weakness, a condition associated with disability and delayed death. This study aimed to elucidate the distinct metabolic effects of critical illness and ß-OH-butyrate on satellite cells isolated from these patients. METHODS: Satellite cells were extracted from vastus lateralis muscle biopsies of patients with ICU-acquired weakness (n = 10) and control group of healthy volunteers or patients undergoing elective hip replacement surgery (n = 10). The cells were exposed to standard culture media supplemented with ß-OH-butyrate to assess its influence on cell proliferation by ELISA, mitochondrial functions by extracellular flux analysis, electron transport chain complexes by high resolution respirometry, and ROS production by confocal microscopy. RESULTS: Critical illness led to a decline in maximal respiratory capacity, ATP production and glycolytic capacity and increased ROS production in ICU patients' cells. Notably, the function of complex II was impaired due to critical illness but restored to normal levels upon exposure to ß-OH-butyrate. While ß-OH-butyrate significantly reduced ROS production in both control and ICU groups, it had no significant impact on global mitochondrial functions. CONCLUSION: Critical illness induces measurable bioenergetic dysfunction of skeletal muscle satellite cells. ß-OH-butyrate displayed a potential in rectifying complex II dysfunction caused by critical illness and this warrants further exploration.

Unusual cause of muscle weakness, type II respiratory failure and pulmonary hypertension: a case report of ryanodine receptor type 1(RYR1)-related myopathy.

BACKGROUND: Patients with congenital myopathies may experience respiratory involvement, resulting in restrictive ventilatory dysfunction and respiratory failure. Pulmonary hypertension (PH) associated with this condition has never been reported in congenital ryanodine receptor type 1(RYR1)-related myopathy. CASE PRESENTATION: A 47-year-old woman was admitted with progressively exacerbated chest tightness and difficulty in neck flexion. She was born prematurely at week 28. Her bilateral lower extremities were edematous and muscle strength was grade IV-. Arterial blood gas analysis revealed hypoventilation syndrome and type II respiratory failure, while lung function test showed restrictive ventilation dysfunction, which were both worse in the supine position. PH was confirmed by right heart catheterization (RHC), without evidence of left heart disease, congenital heart disease, or pulmonary artery obstruction. Polysomnography indicated nocturnal hypoventilation. The ultrasound revealed reduced mobility of bilateral diaphragm. The level of creatine kinase was mildly elevated. Magnetic resonance imaging showed myositis of bilateral thigh muscle. Muscle biopsy of the left biceps brachii suggested muscle malnutrition and congenital muscle disease. Gene testing revealed a missense mutation in the RYR1 gene (exon33 c.C4816T). Finally, she was diagnosed with RYR1-related myopathy and received long-term non-invasive ventilation (NIV) treatment. Her symptoms and cardiopulmonary function have been greatly improved after 10 months. CONCLUSIONS: We report a case of RYR1-related myopathy exhibiting hypoventilation syndrome, type II respiratory failure and PH associated with restrictive ventilator dysfunction. Pulmonologists should keep congenital myopathies in mind in the differential diagnosis of type II respiratory failure, especially in patients with short stature and muscle weakness.

Thyrotoxic Periodic Paralysis, an Unusual Presentation of Paralysis After Spinal Surgery: A Case Report.

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism, seen predominantly in men of Asian origin. We report an unusual presentation of paralysis post-lumbar laminectomy surgery, associated with shortness of breath and arrhythmia. The patient was initially thought to have nerve compression as a complication of surgery but was found to have severe hypokalemia, which responded to intravenous potassium supplements. Additional tests identified suppressed thyroid stimulating hormone (TSH). The patient was diagnosed with thyrotoxic periodic paralysis (TPP), which was treated with oral potassium supplements and antithyroid drugs, followed by a total thyroidectomy. The report discusses the epidemiology, presentation, treatment, and complications of this rare condition.

Neuroprotective treatment with the nitrone compound OKN-007 mitigates age-related muscle weakness in aging mice.

Despite the universal impact of sarcopenia on compromised health and quality of life in the elderly, promising pharmaceutical approaches that can effectively mitigate loss of muscle and function during aging have been limited. Our group and others have reported impairments in peripheral motor neurons and loss of muscle innervation as initiating factors in sarcopenia, contributing to mitochondrial dysfunction and elevated oxidative stress in muscle. We recently reported a reduction in α motor neuron loss in aging mice in response to the compound OKN-007, a proposed antioxidant and anti-inflammatory agent. In the current study, we asked whether OKN-007 treatment in wildtype male mice for 8-9 months beginning at 16 months of age can also protect muscle mass and function. At 25 months of age, we observed a reduction in the loss of whole-body lean mass, a reduced loss of innervation at the neuromuscular junction and well-preserved neuromuscular junction morphology in OKN-007 treated mice versus age matched wildtype untreated mice. The loss in muscle force generation in aging mice (~ 25%) is significantly improved with OKN-007 treatment. In contrast, OKN-007 treatment provided no protection in loss of muscle mass in aging mice. Mitochondrial function was improved by OKN-007 treatment, consistent with its potential antioxidative properties. Together, these exciting findings are the first to demonstrate that interventions through neuroprotection can be an effective therapy to counter aging-related muscle dysfunction.

Balloon Pressure and Clinical Effectiveness of Percutaneous Microballoon Compression in the Treatment of Primary Trigeminal Neuralgia.

BACKGROUND: Primary trigeminal neuralgia (PTN) is a type of chronic neuropathic pain disorder caused by neurovascular compression. Percutaneous balloon compression (PBC) is a widely used method for the treatment of PTN. OBJECTIVES: To examine the correlation of balloon pressure (BP) during percutaneous microballoon compression (PBC) with postoperative pain relief and complications in the treatment of primary trigeminal neuralgia (PTN). STUDY DESIGN: Forty-five patients diagnosed with PTN and treated with PBC were recruited. The BP was recorded at 2 time points: when the balloon achieved the ideal pear shape (initial BP [IBP]) and when the pressure was maintained for 2 min (final BP [FBP]). SETTING: This study was conducted at the Department of Pain and Rehabilitation of the Second Affiliated Hospital at the University of South China in Hunan, China. METHODS: The patients' Barrow Neurological Institute (BNI) pain intensity score, BNI facial numbness score, masticatory muscle weakness score, and recurrence were recorded before and after surgery. The receiver operating characteristic (ROC) curves were generated for the IBP to predict treatment effectiveness, severe facial numbness, and severe masticatory muscle weakness. RESULTS: The BNI pain intensity score, BNI facial numbness score, and masticatory muscle weakness score were significantly decreased after surgery (all P < 0.001). IBP was positively correlated with the difference between IBP and FBP (P < 0.01). Both IBP and the difference between IBP and FBP were negatively correlated with the BNI pain intensity score and positively correlated with the BNI facial numbness score and masticatory muscle weakness score (P < 0.01). The IBP and the difference between the IBP and FBP were significantly lower in patients experiencing recurrence than in the nonrecurrent group (P < 0.05). The areas under the ROC curves of the IBP for predicting effective pain relief, severe facial numbness, and severe masticatory muscle weakness were 0.875, 0.980, and 0.988, respectively. LIMITATIONS: The sample size was relatively small, and the follow-up time was short. The correlations between the BP and other factors, such as filling amount, Meckel's cavity, and the size of the foramen ovale, were not investigated. The impact of the BP on long-term postoperative outcomes was not explored. CONCLUSIONS: An intraoperative BP of 138.65-153.90 KPa can be maintained for effective PBC treatment without causing serious complications.

Bioenergetic impairment in Gulf War illness assessed via 31P-MRS.

Time for post-exercise phosphocreatine-recovery (PCr-R), deemed a robust index of mitochondrial function in vivo, was previously reported to be elevated (signifying impaired ATP production) in veterans with Gulf War illness (GWI). Here we sought to replicate the finding and assess the impact of contravening previous eligibility requirements. The replication sample comprised white males. Cases reported ≥ moderate muscle-weakness to match the organ assessed to an organ affected; controls lacked recent headache or multiple symptoms. The expansion sample added cases without muscle-weakness, controls with recent headache, females, nonwhites. PCr-R, following pedal-depression-exercise, was compared in veterans with GWI versus controls (sample N = 38). In the replication sample, PCr-R results closely matched the prior report: PCr-R veterans with GWI mean(SD) = 47.7(16.5); control mean(SD) = 30.3(9.2), p = 0.017. (Prior-study PCr-R veterans with GWI mean(SD) = 46.1(17.9), control mean(SD) = 29.0(8.7), p = 0.023. Combined replication + prior samples: p = 0.001.) No case-control difference was observed in the expansion sample. In cases, PCr-R related to muscle-weakness: PCr-R = 29.9(7.1), 38.2(8.9), 47.8(15.2) for muscle-weakness rated none/low, intermediate, and high respectively (p for trend = 0.02), validating desirability of matching tissue assessed to tissue affected. In controls, headache/multiple symptoms, sex, and ethnicity each mattered (affecting PCr-R significantly). This study affirms mitochondrial/bioenergetic impairment in veterans with GWI. The importance of careful case/control selection is underscored.

Inflammatory Myopathy: When Electromyography and Autoantibodies Don't Help the Diagnosis.

Inflammatory myopathies (IM) are the most treatable myopathies. Necrotizing autoimmune myositis is a distinct clinicopathologic entity that starts either acutely or subacutely. Autoimmunity is essencial in the pathogenesis of myositis and autoantibodies may be present in more than 50% of patients. We present the case of a 73-year-old man with elevated levels of CK and aldolase, and proximal symmetric muscle weakness and weight loss. The etiological investigation revealed, via muscle biopsy, a necrotizing autoimmune myositis, even though the majority of usual autoantibodies and electromyography were negative. The case demonstrates the importance of combining the patient's symptoms, neurological examination, and analytical changes to corroborate the suspicion of myopathy.

Alcohol use disorder and muscle weakness: Original study of the effect of vitamin D supplementation in ambulatory participants with alcohol use disorder.

INTRODUCTION: Chronic alcohol-related myopathy presents with proximal muscle weakness. We studied the effect of vitamin D supplementation on muscle weakness in adults with alcohol use disorder. METHOD: Randomized controlled trial. Participants were community-dwelling adults with alcohol use disorder. Participants allocated to VIDIO, vitamin D intensive outreach, received bimonthly oral doses of 50,000‒100,000 IU cholecalciferol for 12 months. Participants allocated to CAU, care as usual, received prescriptions of once-a-day tablets containing 800 IU cholecalciferol and 500 mg calcium carbonate. Data included demographic variables, laboratory tests, alcohol use, and rating scales of help-seeking and support. Main outcomes were the participants' quadriceps maximum voluntary contractions (qMVC) and serum-25(OH)vitamin D concentrations, 25(OH)D. RESULTS: In 66 participants, sex ratio 50/16, mean age 51 year, alcohol use was median 52 [IQR 24‒95] drinks per week. Baseline qMVC values were 77% (SD 29%) of reference values. Laboratory tests were available in 44/66 participants: baseline 25(OH)D concentrations were 39.4 (SD 23.7) nmol/L. Thirty-one participants with 25(OH)D concentrations <50 nmol/L received either VIDIO or CAU and improved in qMVC, respectively with mean 51 (P<0.05) and 62 Newton (no P-value because of loss of follow-up) after one year of treatment. Vitamin D status increased with mean +56.1 and +37.4 nmol/L, respectively in VIDIO and CAU. CONCLUSION: The qMVC values improved during vitamin supplementation in adults with vitamin D deficiency and alcohol use disorder. Despite higher 25(OH)D concentrations in VIDIO, in terms of muscle health no advise could be given in favor of one vitamin strategy over the other. TRIAL REGISTRATION: Netherlands Trial Register (NTR) identifier: NTR4114.

COLQ-Congenital myasthenic syndrome in an Iranian cohort: the clinical and genetics spectrum.

BACKGROUND: Congenital myasthenic syndrome (CMS) is a group of neuromuscular disorders caused by abnormal signal transmission at the motor endplate. Mutations in the collagen-like tail subunit gene (COLQ) of acetylcholinesterase are responsible for recessive forms of synaptic congenital myasthenic syndromes with end plate acetylcholinesterase deficiency. Clinical presentation includes ptosis, ophthalmoparesis, and progressive weakness with onset at birth or early infancy. METHODS: We followed 26 patients with COLQ-CMS over a mean period of 9 years (ranging from 3 to 213 months) and reported their clinical features, electrophysiologic findings, genetic characteristics, and therapeutic management. RESULTS: In our population, the onset of symptoms ranged from birth to 15 years. Delayed developmental motor milestones were detected in 13 patients (∼ 52%), and the most common presenting signs were ptosis, ophthalmoparesis, and limb weakness. Sluggish pupils were seen in 8 (∼ 30%) patients. All patients who underwent electrophysiologic study showed a significant decremental response (> 10%) following low-frequency repetitive nerve stimulation. Moreover, double compound muscle action potential was evident in 18 patients (∼ 75%). We detected 14 variants (eight novel variants), including six missense, three frameshift, three nonsense, one synonymous and one copy number variation (CNV), in the COLQ gene. There was no benefit from esterase inhibitor treatment, while treatment with ephedrine and salbutamol was objectively efficient in all cases. CONCLUSION: Despite the rarity of the disease, our findings provide valuable information for understanding the clinical and electrophysiological features as well as the genetic characterization and response to the treatment of COLQ-CMS.

Ambulatory Status at Discharge Predicts Six-Month Mortality in Patients with COVID-19: A Retrospective Cohort Study.

This retrospective cohort study aimed to evaluate the association between ambulatory status at discharge and six-month post-discharge mortality among adults with coronavirus disease (COVID-19). We analyzed data from 398 patients aged over 18 admitted to a tertiary hospital in South Korea between December 2019 and June 2022. Patients were classified into two groups based on their ambulatory status at discharge: ambulatory (able to walk independently, n = 286) and non-ambulatory (unable to walk independently, requiring wheelchair or bed-bound, n = 112). Our analysis revealed that six-month survival rates were significantly higher in the ambulatory group (94.2%) compared to the non-ambulatory group (84.4%). Multivariate analysis identified ambulatory status at discharge (p = 0.047) and pre-existing malignancy (p = 0.007) as significant prognostic factors for post-discharge survival. This study highlights that the ability to walk independently at discharge is a crucial predictor of six-month survival in COVID-19 patients. These findings emphasize the need for interventions to improve the physical performance of non-ambulatory patients, potentially enhancing their survival prospects. This underscores the importance of targeted rehabilitation and physical therapy for the comprehensive care of COVID-19 survivors.

Role of recovery of acetylcholine release in compromised neuromuscular junction function.

Everyday physical activities, such as walking, are enabled by repeated skeletal muscle contractions and require a well-functioning neuromuscular transmission. In myasthenic disorders, activities of daily living are debilitated by a compromised neuromuscular transmission leading to muscle weakness and fatiguability in patients. To enable physical activity, acetylcholine (ACh) is released repeatedly from the motor nerve, however, the role of the nerve terminals' capacity to sustain ACh release to support repetitive contractions under compromised neuromuscular transmission remains unclear. To explore this, we studied synaptic and contractile function during repeated contractions in healthy rat skeletal muscles under conditions of pharmacological induced compromised neuromuscular transmission. Using recordings of endplate potentials, compound muscle action potential (CMAP) and force production in isolated skeletal muscles and living, anesthetized animals, we found that force and CMAP were markedly reduced by even very light activity performed up to 5 s prior to contraction showing that recovery of ACh release was insufficient to maintain synaptic transmission strength. Our results suggest that the timing of depletion and restoration of ACh release may impact clinical signs of weakness and fatigability in patients with impaired neuromuscular transmission and affect the sensitivity of electromyographic recordings in the clinic.

Semi-Immersive Virtual Reality Exercise Therapy for Upper Limb Rehabilitation in Patients With Spinal Cord Injury Using the Leap Motion Controller.

In this article, we present a case study involving a patient with spinal cord injury (SCI), resulting in tetraplegia and subsequent loss of upper limb function. The subject of our study was a 23-year-old woman with incomplete tetraplegia stemming from a cervical spinal cord injury. Our primary objective was to enhance hand function and grip strength. Throughout the intervention, we observed substantial enhancements in hand function, range of motion, and muscle power. Notably, the patient exhibited a favorable response to the therapy, demonstrating commendable adherence and active participation. To create an optimal training environment tailored to the patient's needs, we employed the Unity 3D game engine in conjunction with a Leap Motion controller sensor. This combination facilitated the development of a semi-immersive virtual training environment. The utilization of this technology aimed to simulate a conducive training atmosphere for the rehabilitation of hand function. Based on our study outcomes, we advocate for the incorporation of leap motion-related exercises in the treatment of hand functional loss and weakness. The promising results observed in this case study prompt the recommendation for further large-scale studies to validate and substantiate our findings. Such investigations would contribute to the establishment of evidence-based practices and enhance the understanding of the efficacy of Leap Motion technology in addressing upper limb impairments associated with spinal cord injuries.

Passive exercise is an effective alternative to HRT for restoring OVX induced mitochondrial dysfunction in skeletal muscle.

Background: Postmenopausal women are more prone to develop muscle weakness, which is strongly associated with impairment of mitochondrial function in skeletal muscle. This study aimed to examine the impact of a passive exercise modality, whole-body vibration training (WBVT), on muscle mitochondrial function in ovariectomized (OVX) mice, in comparison with 17ß-estradiol (E2) replacement. Methods: Female C57BL/6J mice were assigned to four groups: sham operation control group (Sham), ovariectomized group (OVX), OVX with E2 supplement group (OVX+E), and OVX with WBVT group (OVX+W). The estrous cycle, body weight, body composition, and muscle strength of the mice were monitored after the operation. Serum E2 level was assessed by enzyme-linked immunosorbent assay (ELISA). The ATP levels were determined using a luciferase-catalyzed bioluminescence assay. The activity of mitochondrial respiration chain complexes was evaluated using high-resolution respirometry (O2K). Expression levels of oxidative phosphorylation (OXPHOS), peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1α), and mitochondrial transcription factor A (TFAM) were detected using western blotting. Results: We observed decreased muscle strength and impaired mitochondrial function in the skeletal muscle of OVX mice. The vibration training alleviated these impairments as much as the E2 supplement. In addition, the vibration training was superior to the ovariectomy and the estradiol replacement regarding the protein expression of PGC-1α and TFAM. Conclusion: WBVT improves the OVX-induced decline in muscle strength and impairment of mitochondrial function in the skeletal muscle. This passive exercise strategy may be useful as an alternative to E2 replacement for preventing menopausal muscular weakness. Further studies are needed to understand the effects of WBVT on various physiological systems, and precautions should be taken when implementing it in patient treatment.

Hypokalemic Periodic Paralysis Type 2 Due to SCN4A Val1105Met Mutation: A Case Study.

Hypokalemic Periodic Paralysis Type 2 (HOKPP2) is a rare autosomal dominant disorder characterized by recurrent episodes of muscle weakness, paralysis, and hypokalemia. In this case report, we present the clinical details of a 49-year-old female diagnosed with HOKPP2. Genetic testing revealed a heterozygous mutation in the Sodium Voltage-Gated Channel Alpha Subunit 4 (SCN4A) gene, confirming the diagnosis of HOKPP2. Management strategies, including potassium supplementation and lifestyle modifications, were implemented, resulting in a significant decrease in the frequency of symptomatic episodes. This case highlights the importance of considering HOKPP2 in patients with recurrent muscle weakness, particularly those with a familial history of similar symptoms. Furthermore, it underscores the crucial role of genetic testing in guiding patient management and facilitating genetic counseling.

Risk factors for respiratory failure among hospitalized patients with Guillain–Barré syndrome / Factores de riesgo de insuficiencia respiratoria en pacientes hospitalizados con síndrome de Guillain-Barré

Background Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy that can lead to respiratory failure. In this study, we evaluate early clinical risk factors for respiratory failure at the time of hospital admission.Methods We studied a retrospective cohort of patients with GBS admitted to a tertiary care center. The potential risk factors studied were sociodemographic characteristics, GBS symptoms, overall and cervical muscle weakness (Medical Research Council [MRC] scores), electromyography findings, and cerebrospinal fluid analysis findings. Unadjusted odds ratios (OR) were calculated and exact logistic regression analysis (adjusted OR) performed to assess the association between baseline risk factors and respiratory failure. Results Overall, 13 of 113 (12%) patients included in the study developed respiratory failure. Unadjusted analyses showed that involvement of any cranial nerve (OR: 14.7; 95% CI, 1.8–117.1), facial palsy (OR: 17.3; 95% CI, 2.2–138.0), and bulbar weakness (OR: 10.7; 95% CI, 2.3–50.0) were associated with increased risk of respiratory failure. Lower MRC sum scores (for scores <30, OR: 14.0; 95% CI, 1.54–127.2) and neck MRC scores (for scores ≤3, OR: 21.0; 95% CI, 3.5–125.2) were associated with higher likelihood of respiratory failure. Adjusted analyses showed that presence of bulbar weakness (OR: 7.6; 95% CI, 1.3–43.0) and low neck MRC scores (scores ≤3, OR: 9.2; 95% CI, 3.5–125.2, vs scores >3) were independently associated with respiratory failure. Conclusions Bulbar and neck muscle weakness at admission are clinical predictors of increased risk of respiratory failure in patients with GBS. These findings could guide the adequate management of high-risk patients. (AU)

Introducción El síndrome de Guillain-Barré es una polineuropatía inflamatoria aguda que puede causar insuficiencia respiratoria. Evaluamos los factores de riesgo clínicos en el momento de la hospitalización. Métodos Realizamos un estudio de una cohorte retrospectiva de pacientes con síndrome de Guillain-Barré hospitalizados en un centro de tercer nivel. Analizamos las características sociodemográficas, síntomas de la enfermedad, fuerza muscular general y cervical (escala del Medical Research Council [MRC]), hallazgos electromiográficos, y resultados del análisis del líquido cefalorraquídeo. Calculamos el odds ratio (OR) sin ajustar y realizamos una regresión logística exacta (OR ajustada) para evaluar la asociación entre los factores de riesgo y la insuficiencia respiratoria. Resultados Trece de los 113 pacientes incluidos (12%) presentó insuficiencia respiratoria. Los análisis no ajustados mostraron una asociación entre mayor riesgo de insuficiencia respiratoria y la afectación de cualquier par craneal (OR: 14,7; IC 95%, 1,8-117,1), parálisis facial (OR: 17,3; IC 95%, 2,2-138,0) y debilidad bulbar (OR: 10,7; IC 95%, 2,3-50,0). Unas puntuaciones más bajas en la MRC-total (puntuaciones <30, OR: 14,0; IC 95%, 1,54-127,2) y en la MRC-cervical (puntuaciones <3, OR: 21,0; IC 95%, 3,5-125,2) se asociaron con una mayor probabilidad de presentar insuficiencia respiratoria. En los análisis ajustados, la presencia de debilidad bulbar (OR: 7,6; IC 95%, 1,3-43,0) y una puntuación baja en la MRC-cervical (puntuaciones ≤3, OR: 9,2; IC 95%, 3,5-125,2, frente a puntuaciones >3) se asociaron de forma independiente con la insuficiencia respiratoria. Conclusiones La presencia de debilidad bulbar y cervical en el momento de la hospitalización es un factor de riesgo de insuficiencia respiratoria en pacientes con síndrome de Guillain-Barré. Estos hallazgos pueden servir de guía para el manejo de los pacientes con mayor riesgo de presentar dicha complicación. (AU)

Physiotherapy Strategies in Hypokalemic Periodic Paralysis: A Case Report.

The rare neuromuscular disease known as hypokalemic periodic paralysis (hypoKPP), which results in severe muscle weakness in the extremities, is brought on by abnormalities in potassium transport within cells. Laboratory testing is confirmatory, which reveals notably low potassium levels, causing paralysis, which improves once the low potassium is restored. The patient generally complains of muscle weakness with difficulty in performing activities of daily living and impaired participation in functional tasks, with few suffering from coexisting sensory impairments. Physiotherapy generally plays a symptomatic role with motion exercises for the affected muscle groups. There is no standardized physiotherapy protocol for disease-specific impairments. A 46-year-old man complained of bilateral upper and lower limb muscular weakness and was admitted to the neurology ward. The patient also complained of having tingling numbness throughout their entire limbs and had experienced similar episodes of symptoms six months prior. During laboratory evaluation, a significantly low potassium level was found, leading to a diagnosis of hypoKPP. Following medical management, neurophysiotherapy was initiated. Physiotherapy strategy shows significant improvement in muscular strength and functional activities. Thus, this case report concludes that physiotherapy plays a vital role in managing hypoKPP by enhancing muscular strength, functional activities, and quality of life.

The Relationship between Structure of the Corticoreticular Tract and Walking Capacity in Children with Cerebral Palsy.

Background: Disruption in the descending pathways may lead to gait impairments in Cerebral Palsy (CP) children. Though, the mechanisms behind walking problems have not been completely understood. Objective: We aimed to define the relationship between the structure of the corticoreticular tract (CRT) and walking capacity in children with CP. Material and Methods: This is a retrospective, observational, and cross-sectional study. Twenty-six children with CP between 4 to 15 years old participated. Also, we used existed data of healthy children aged 4 to 15 years old. CRT structure was characterized using diffusion tensor imaging (DTI). The DTI parameters extracted to quantify CRT structure included: fractional anisotropy (FA), mean (MD), axial (AD), and radial (RD) diffusivity. Balance and walking capacity was evaluated using popular clinical measures, including the Berg balance scale (BBS), Timed-Up-and-Go (TUG; balance and mobility), six-minute walk test (6 MWT; gait endurance), and 10-meter walk Test (10 MWT; gait speed). Results: There are significant differences between MD, AD, and RD in CP and healthy groups. Brain injury leads to various patterns of the CRT structure in children with CP. In the CP group with abnormal CRT patterns, DTI parameters of the more affected CRT are significantly correlated with walking balance, speed, and endurance measures. Conclusion: Considering the high inter-subject variability, the variability of CRT patterns is vital for determining the nature of changes in CRT structure, their relationship with gait impairment, and understanding the underlying mechanisms of movement disorders. This information is also important for the development or prescription of an effective rehabilitation target for individualizing treatment.

Management of a 25-Year-Old Female Patient With Limb-Girdle Muscular Dystrophy With Physiotherapy: A Case Report.

Limb-girdle muscular dystrophy (LGMD) is a collection of neuromuscular diseases that develop gradually and are rare, genetically, and clinically diverse. The weakness in muscles affecting the shoulder and pelvic girdles is a defining feature of LGMD. Calpainopathy is another name for limb-girdle muscular dystrophy type 2A (LGMD2A). Limb-girdle muscular dystrophy type 2A results from alterations in the calpain-3 (CAPN3) gene, which results in a CAPN3 protein shortage. Gower's sign is most commonly found in LGMD2A. The prevalence ranges from one person in every 14,500 to one in every 123,000. We present a case of a 25-year-old hypotensive female patient who complained of weakness in all four limbs and easy fatigue with a positive Gower's sign. For subsequent management, the neurologist referred the patient to the physical therapy department. The physical therapy goals included enhanced muscle strength, increased joint mobility, reduced fatigue, normalizing gait, and building dynamic balance and postural stability. Diagnosing LGMD clinical variability is important, emphasizing the importance of precise subtype identification and tailoring therapy. Tackling specific muscular deficits and functional restrictions emerges as a critical component in the holistic care of LGMD by physiotherapists. Continuous monitoring and evaluation using appropriate scales and measurements are essential for tracking performance and tailoring treatment strategies. Regular follow-up consultations with the physiotherapist are needed to identify changes in an individual's health and alter the treatment plan accordingly.