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PloS One 2024
Topics: Muscular Dystrophy, Duchenne; Signal Transduction; Humans; Hippo Signaling Pathway; Protein Serine-Threonine Kinases
PubMed: 38935667
DOI: 10.1371/journal.pone.0306508 -
Journal of Clinical Neurophysiology :... Jul 2024Repetitive transcranial magnetic stimulation (rTMS) can enhance brain plasticity after stroke. At low frequencies, rTMS has an inhibitory effect, whereas at high... (Randomized Controlled Trial)
Randomized Controlled Trial Comparative Study
Comparison of Bilateral Versus Unilateral 5 Hz or 1 Hz Repetitive Transcranial Magnetic Stimulation in Subacute Stroke: Assessment of Motor Function in a Randomized Controlled Study.
PURPOSE
Repetitive transcranial magnetic stimulation (rTMS) can enhance brain plasticity after stroke. At low frequencies, rTMS has an inhibitory effect, whereas at high frequencies, it has an excitatory effect. Combining both frequencies in bilateral stimulation is a new rTMS protocol under investigation, especially in the subacute stage.
METHODS
Fifty-five patients with subacute stroke were divided into four groups according to the rTMS protocol delivered: bilateral, inhibitory, excitatory, and control groups. All groups received concomitant task-oriented physiotherapy. Pretreatment to posttreatment assessment was performed twice, immediately after sessions and 1 month later. Volitional motor control was evaluated by Fugl-Meyer and Wolf motor function tests, and for spasticity, the Ashworth scale was used.
RESULTS
All groups showed significant improvement. Bilateral, inhibitory, and excitatory groups showed same efficacy, but the bilateral protocol was superior in spasticity. No correlations were found between improvement and stroke duration and site except for spasticity.
CONCLUSIONS
Bilateral rTMS shows a comparable effect to inhibitory and excitatory rTMS in improving motor disability in subacute stroke. However, it is superior for spasticity.
Topics: Humans; Male; Female; Transcranial Magnetic Stimulation; Middle Aged; Stroke; Aged; Stroke Rehabilitation; Treatment Outcome; Recovery of Function; Adult; Muscle Spasticity
PubMed: 38935659
DOI: 10.1097/WNP.0000000000000987 -
Muscle & Nerve Jun 2024Point-of-care ultrasound of the diaphragm is highly sensitive and specific in the detection of neuromuscular diaphragmatic dysfunction. In some patients with...
INTRODUCTION/AIMS
Point-of-care ultrasound of the diaphragm is highly sensitive and specific in the detection of neuromuscular diaphragmatic dysfunction. In some patients with neuromuscular diaphragmatic dysfunction, paradoxical thinning of the diaphragm during inspiration is observed on ultrasound; however, its frequency, electrodiagnostic associations, and prognostic significance remain uncertain.
METHODS
Medical records of patients presenting to two electrodiagnostic laboratories (Mayo Clinic, Rochester, Minnesota and University of Alberta, Edmonton, Alberta) from January 1, 2022 to December 31, 2022, for evaluation of suspected neuromuscular respiratory failure, were reviewed.
RESULTS
214 patients were referred and 19 patients excluded due to incomplete information. Of 195 patients (384 hemidiaphragms), 104 had phrenic neuropathy, 12 had myopathy, and 79 had no evidence of neuromuscular disease affecting the diaphragm. Paradoxical thinning occurred in 31 (27%) patients with neuromuscular diaphragmatic dysfunction and was unilateral in 30, the majority (83%) having normal contralateral ultrasound. Phrenic nerve conduction studies and diaphragm electromyography results did not distinguish patients with paradoxical thinning versus without. Most patients (71%) with paradoxical thinning required non-invasive ventilation (NIV), including 16 with unilateral paradoxical thinning. Paradoxical thinning and BMI ≥30 kg/m were risk factors for requiring NIV in multivariable logistic regression analysis, with odds ratios of 2.887 (95% CI:1.166, 7.151) and 2.561 (95% CI: 1.186, 5.532), respectively.
DISCUSSION
Paradoxical thinning of the diaphragm occurs in patients with prominent neuromuscular diaphragmatic dysfunction, most commonly from phrenic neuropathy, and is a significant risk factor for requiring NIV. Unilateral paradoxical thinning is sufficient for needing NIV. BMI ≥30 kg/m additionally increases risk of requiring NIV in patients with neuromuscular diaphragmatic dysfunction.
PubMed: 38935447
DOI: 10.1002/mus.28194 -
Indian Journal of Dental Research :... Jan 2024Gout is a metabolic disorder that leads to elevated serum uric acid levels and deposition of urate crystals in the joints. The disease is usually confined to the joint... (Review)
Review
Gout is a metabolic disorder that leads to elevated serum uric acid levels and deposition of urate crystals in the joints. The disease is usually confined to the joint space and leads to pain and limitation of jaw opening. The case describes a 45-year-old female patient with a chief complaint of 'occasional pain in the left temporal muscle region'. The case disclosed a gout manifestation in the temporomandibular joint (TMJ) after physical and radiographic findings. Gout manifestation in the TMJ is an unusual presentation and a few reports in the English literature address the subject. Gout in the TMJ should be included as a differential diagnosis for joint disorders because of its rarity. A clinician may overlook gout involving the TMJ in the differential diagnosis of facial pain even when the patient has received a diagnosis of gout in other joints.
Topics: Humans; Female; Middle Aged; Temporomandibular Joint Disorders; Arthritis, Gouty; Diagnosis, Differential
PubMed: 38934761
DOI: 10.4103/ijdr.ijdr_15_22 -
Immunity, Inflammation and Disease Jun 2024To investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and...
OBJECTIVE
To investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and interstitial lung disease (ILD).
METHODS
A retrospective analysis was conducted on clinical data of 125 patients with anti-MDA5 + CADM-ILD collected from 10 branches in eastern China between December 2014 and December 2022. Prognostic factors were analyzed using χ test, Log-rank test, COX and logistic regression analysis.
RESULTS
In this cohort, 125 anti-MDA5 + CADM-ILD patients exhibited a rapidly progressive interstitial lung disease (RPILD) incidence of 37.6%, and an overall mortality rate of 24.8%. One patient was lost to follow-up. After diagnosis of RPILD, a mortality rate of 53.2% occurred in patients died within 3 months, and that of 5.6% appeared in those who survived for more than 3 months. Multiple factor analysis revealed that C-reactive protein (CRP) ≥ 10 mg/L (p = 0.01) and recombinant human tripartite motif containing 21 (Ro52) (+) (p = 0.003) were associated with a higher risk of RPILD in anti-MDA5 + CADM-ILD patients; CRP ≥ 10 mg/L (p = 0.018) and the presence of RPILD (p = 0.003) were identified as the factors influencing survival time in these patients, while arthritis was the protective factor (p = 0.016).
CONCLUSION
Patients with anti-MDA5 + CADM-ILD will have a higher mortality rate, and the initial 3 months after diagnosis of RPILD is considered the risk window for the dismal prognosis. Patients with CRP ≥ 10 mg/L, Ro52 (+) and RPILD may be related to a shorter survival time, while patients complicated with arthritis may present with relatively mild conditions.
Topics: Humans; Lung Diseases, Interstitial; Dermatomyositis; Interferon-Induced Helicase, IFIH1; Male; Female; Prognosis; Middle Aged; Retrospective Studies; Adult; Autoantibodies; China; Aged
PubMed: 38934403
DOI: 10.1002/iid3.1332 -
Acta Odontologica Scandinavica Jun 2024The Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) is part of the undergraduate dental curriculum. Online teaching has nowadays become common also in...
INTRODUCTION
The Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) is part of the undergraduate dental curriculum. Online teaching has nowadays become common also in dentistry.
OBJECTIVE
To compare undergraduate students' self-assessed ability and satisfaction with learning DC/TMD Axis I between traditional and online learning and to evaluate the possible gains of online teaching.
MATERIAL AND METHODS
Third-year undergraduate dental students in 2018 (traditional learning, Group 1, n = 43/50) and in 2019 (online learning, Group 2, n = 34/50) at the University of Oulu, Finland evaluated their self-assessed ability and satisfaction with learning DC/TMD clinical examination and diagnostics on a 10-point scale. Additionally, those participating in online courses answered to two open-ended questions; Group 2 (n = 50) and another group from the University of Eastern Finland in 2019 and 2020 (n = 75, Group 3). Total of 105/125 students (84%) responded. Content analysis was used to open-ended responses.
RESULTS
The online course reported significantly higher self-assessed ability in measurements (p = 0.004), identifying referred pain (p = 0.043) and statement for the diagnostics (p = 0.017) and also higher self-assessed satisfaction in measurements (p = 0.046). According to the content analysis, essential gains of online teaching were efficient learning, videos and exercises, and adjustability to own timetable.
CONCLUSION
The online learning course can be considered as a good option for traditional learning of the DC/TMD protocol.
Topics: Humans; Temporomandibular Joint Disorders; Education, Dental; Education, Distance; Finland; Male; Female; Curriculum; Surveys and Questionnaires
PubMed: 38934339
DOI: 10.2340/aos.v83.40984 -
Archives of Rheumatology Jun 2024This study aimed to analyze the risk factors for mortality of idiopathic inflammatory myopathy (IIM) patients admitted with interstitial lung disease (ILD) to guide...
OBJECTIVES
This study aimed to analyze the risk factors for mortality of idiopathic inflammatory myopathy (IIM) patients admitted with interstitial lung disease (ILD) to guide rapid and accurate judgment of clinical prognosis.
PATIENTS AND METHODS
This retrospective, single-center cohort study was conducted with 135 participants (37 males, 98 females; mean age: 54.8±11.1 years; range, 24 to 85 years) between June 1, 2016, and June 30, 2021. The participants were categorized into the survival group (n=111) and nonsurvivors (n=24) according to whether they survived during the one-year follow-up. The independent risk factors for mortality in one year after discharge were analyzed. Receiver operating characteristic curve analysis was used to determine the accuracy of oxygenation index at baseline combined with pulmonary infection (PI) at follow-up to indicate death in IIM-ILD patients.
RESULTS
Compared to the survival group, nonsurvivors were older (p=0.006) and had a higher proportion of anti-MDA5 (melanoma differentiation-associated protein 5) positivity (p<0.001). The ILD duration was shorter (p=0.006), the oxygenation index was lower (p<0.001), and the intensive care unit occupancy rate (p<0.001) and ventilator utilization rate (p<0.001) were elevated in nonsurvivors compared to the survival group. Oxygenation index at baseline (odds ratio [OR]=1.021, 95% confidence interval [CI]: 1.001-1.023, p=0.040) and PI (clinical judgment) at follow-up (OR=16.471, 95% CI: 1.565-173.365, p=0.020) were found as independent risk factors for death in the year after discharge in IIM inpatients with ILD. An oxygenation index ≤279 mmHg at baseline combined with PI at follow-up exhibited a promising predictive value for all-cause death in IIM-ILD patients within one year.
CONCLUSION
Oxygenation index at baseline and PI during follow-up were independent risk factors for death of IIM-ILD patients within one year after discharge. Patients with an oxygenation index ≤279 mmHg at baseline had an increased risk of death once they developed PI during the one-year follow-up.
PubMed: 38933718
DOI: 10.46497/ArchRheumatol.2024.10418 -
Cureus May 2024Pneumatosis cystoides intestinalis (PCI) is a rare disease wherein air accumulates in the intestinal subserosa and submucosa, causing multiple gaseous cysts within the...
Pneumatosis cystoides intestinalis (PCI) is a rare disease wherein air accumulates in the intestinal subserosa and submucosa, causing multiple gaseous cysts within the gastrointestinal wall. While PCI has various known risk factors, reports identifying muscular diseases as a factor are scarce. The aim of this study is to elucidate the clinical characteristics of PCI in muscle disease. We present a case series of five cases, including two cases of Duchenne muscular dystrophy (DMD) and three cases of rare congenital myopathies. All cases are of male patients, with poor intestinal peristalsis and constipation, who underwent tube feeding and mechanical ventilation via tracheostomy. They had no signs of severe complications, such as intestinal necrosis, and all of them improved with conservative treatment. Case 1 is a 23-year-old man with DMD who developed cardiopulmonary arrest at the age of 20 years. Pulmonary hemorrhage occurred three months before the incidental detection of PCI in the ascending colon, which resolved with conservative oxygen treatment. Case 2 is a 25-year-old man with DMD who progressed to immobility necessitating tracheostomy at the age of 20 years. He experienced persistent abdominal pain and nausea, and PCI was detected in the cecum and ascending colon. He showed near-complete resolution of PCI after three months of conservative treatment. Case 3 is a six-year-old boy with reducing body myopathy. Constipation was diagnosed at four years of age. He experienced intermittent bloody stools, leading to the incidental detection of PCI at six years of age. After two months of conservative treatment, the PCI resolved with no subsequent recurrence. Case 4 is a 33-year-old man with infantile severe myotubular myopathy. He required mechanical ventilation immediately after birth and later underwent tracheostomy and tube feeding due to complications. At the age of 27 years, PCI was incidentally detected on abdominal CT. He had episodes of remission and worsening for a few years; however, PCI completely resolved after three years. Case 5 is a 27-year-old man with nemaline myopathy. At the age of 14 years, he had persistent bloody stools. After lower gastrointestinal endoscopy, he was diagnosed with PCI with numerous rectal cysts. PCI required no specific therapeutic intervention. There was spontaneous resolution of PCI and bloody stools. Given that PCI lacks specific symptoms and cases with muscular diseases often experience abdominal issues, many cases are liable to be overlooked or misdiagnosed. Cases with muscular diseases complaining of persistent abdominal symptoms should undergo radiographic imaging to rule out PCI.
PubMed: 38933611
DOI: 10.7759/cureus.61188 -
Neurology. Clinical Practice Jun 2024Nusinersen has shown significant functional motor benefit in the milder types of spinal muscular atrophy (SMA). Less is known on the respiratory outcomes in patients...
BACKGROUND AND OBJECTIVES
Nusinersen has shown significant functional motor benefit in the milder types of spinal muscular atrophy (SMA). Less is known on the respiratory outcomes in patients with nusinersen-treated SMA. The aim of this study was to describe changes in respiratory function in pediatric patients with SMA type 2 and 3 on regular treatment with nusinersen within the iSMAc international cohort and to compare their trajectory with the natural history (NH) data published by the consortium in 2020.
METHODS
This is a 5-year retrospective observational study of pediatric SMA type 2 and nonambulant type 3 (age ≤18 years) treated with nusinersen. The primary objective was to compare the slopes of decline in forced vital capacity % predicted (FVC% pred.), FVC, and age when FVC dropped below 60% between the treated patients and a control group from the natural history cohort. Data on peak cough flow and the use of noninvasive ventilation (NIV) and cough assist were collected.
RESULTS
Data were available for 69 treated patients, 53 were SMA type 2 and 16 type 3. The mean (SD) age at first injection was 8.5 (3.2) and 9.7 (3.7) years, respectively. The median (interquartile range) treatment duration was 1 (0.7; 1.9) and 1.2 (0.9; 1.9) years, respectively. At the time of the first nusinersen injection, 24 of 52 (46%) patients with SMA type 2 and 2 of 16 (13%) patients with SMA type 3 were on NIV. Forty-three of 53 (81%) and 4 of 16 (25%) patients used cough device. FVC% pred. in treated patients with SMA type 2 declined annually by 2.3% vs 3.9% in NH ( = 0.08) and in treated patients with type 3 by 2.6% vs 3.4% NH ( = 0.59). Patients treated reached FVC <60% later than untreated (12.1 vs 10 years, = 0.05). A higher percentage of treated vs untreated patients maintained FVC% pred. equal/above their baseline after 12 (65% vs 36%) and 24 (50% vs 24%) months, respectively. NIV use among treated did not significantly change throughout 1-year follow-up.
DISCUSSION
This study included the largest real-world cohort of pediatric patients with milder SMA types. The results suggest a positive role of nusinersen in delaying the respiratory decline in patients treated longer than 1 year when compared with natural history. Larger cohorts and longer observation are planned.
CLASSIFICATION OF EVIDENCE
This study provided Class III evidence that nusinersen slows progression for patients with SMA types 2 and 3 compared with a natural history cohort.
PubMed: 38932995
DOI: 10.1212/CPJ.0000000000200298 -
Viruses Jun 2024This study analyzed the neurological manifestation profiles of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection across pandemic waves in pediatric...
This study analyzed the neurological manifestation profiles of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection across pandemic waves in pediatric patients. The study collected data on patients aged between 0 and 18 years, diagnosed with acute SARS-CoV-2 infection, admitted to a pediatric tertiary hospital between 1 March 2020 and 28 February 2023. This study included 1677 patients. Neurological manifestations were noted in 10% (n = 168) of patients with a median age of 3.2 years (interquartile range: 1-11.92). Neurological manifestations were significantly associated with the pandemic waves ( = 0.006) and age groups ( < 0.001). Seizures were noted in 4.2% of cases and reached an increasing frequency over time ( = 0.001), but were not associated with age groups. Febrile seizures accounted for the majority of seizures. Headache was reported in 2.6% of cases and had similar frequencies across the pandemic waves and age groups. Muscular involvement was noted in 2% of cases, reached a decreasing frequency over time ( < 0.001), and showed different frequencies among the age groups. Neurological manifestations of acute SARS-CoV-2 infection exhibit distinct patterns, depending on the pandemic wave and patient age group. The Wuhan and Omicron waves involved the nervous system more often than the other waves.
Topics: Humans; COVID-19; Child, Preschool; Child; Male; Female; Infant; SARS-CoV-2; Adolescent; Nervous System Diseases; Headache; Infant, Newborn; Seizures, Febrile; Seizures; Pandemics
PubMed: 38932259
DOI: 10.3390/v16060967