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Topics in Spinal Cord Injury... 2024The World Health Organization (WHO) recommends that children be breastfed exclusively for the first 6 months of age. This recommendation may prove challenging for women...
The World Health Organization (WHO) recommends that children be breastfed exclusively for the first 6 months of age. This recommendation may prove challenging for women with spinal cord injury (SCI) who face unique challenges and barriers to breastfeeding due to the impact of SCI on mobility and physiology. Tailored provision of care from health care professionals (HCPs) is important in helping women navigate these potential barriers. Yet, HCPs often lack the confidence and SCI-specific knowledge to meet the needs of mothers with SCI. An international panel of clinicians, researchers, consultants, and women with lived experience was formed to create an accessible resource that can address this gap. A comprehensive survey on breastfeeding complications, challenges, resources, and quality of life of mothers with SCI was conducted, along with an environmental scan to evaluate existing postpartum guidelines and assess their relevance and usability as recommendations for breastfeeding after SCI. Building on this work, this article provides evidence-based recommendations for HCPs, including but not limited to general practitioners, obstetricians, pediatricians, physiatrists, lactation consultants, nurses, midwives, occupational therapists, and physiotherapists who work with prospective and current mothers with SCI.
Topics: Humans; Spinal Cord Injuries; Breast Feeding; Female; Autonomic Dysreflexia; Practice Guidelines as Topic; Mothers; Quality of Life; Adult
PubMed: 38799607
DOI: 10.46292/sci23-00079 -
Journal of Neurology May 2024Axial postural abnormalities (PA) are invalidating symptoms of Parkinson's disease (PD). Risk factors for PA are unknown.
BACKGROUND
Axial postural abnormalities (PA) are invalidating symptoms of Parkinson's disease (PD). Risk factors for PA are unknown.
OBJECTIVES
We sought to evaluate PA incidence and risk factors over the first 4-6 years of PD.
METHODS
We included 441 PD patients from the Parkinson's Progression Markers Initiative (PPMI) cohort with data at diagnosis and after 4-year follow-up. PA was defined according to a posture item ≥ 2 at the Movement Disorder Society-sponsored-revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) in Off therapeutic condition. The Kruskal-Wallis test was used to compare characteristics of patients without PA ('no-PA'), with PA at disease onset ('baseline-PA'), and PA developed during follow-up ('develop-PA'). To identify predictors of PA development, univariate and multivariate Cox regression analyses were performed considering demographic, clinical and therapeutic variables.
RESULTS
10.9% of patients showed PA at baseline and 23.7% developed PA within the first 4-6 years since diagnosis. Older age, malignant phenotype, higher MDS-UPDRS part III, Hoehn & Yahr, and dysautonomia (SCOPA-AUT) score, and lower levels of physical activity were predictors of PA development at the univariate analysis. Older age (Hazard ratio [HR] per year: 1.041) and higher MDS-UPDRS part III score (HR per point: 1.035) survived as PA development predictors in the multivariate analysis.
CONCLUSIONS
PPMI cohort data show that > 30% of PD patients present PA within the first 4-6 years of disease. Older age at onset and higher motor burden are associated with a higher risk for PA development. The protective role of physical activity merits to be further investigated.
PubMed: 38796527
DOI: 10.1007/s00415-024-12457-3 -
Medicina (Kaunas, Lithuania) May 2024: Cardiac autonomic neuropathy (CAN) is a severe complication of diabetes mellitus (DM) strongly linked to a nearly five-fold higher risk of cardiovascular mortality....
: Cardiac autonomic neuropathy (CAN) is a severe complication of diabetes mellitus (DM) strongly linked to a nearly five-fold higher risk of cardiovascular mortality. Patients with Type 2 Diabetes Mellitus (T2DM) are a significant cohort in which these assessments have particular relevance to the increased cardiovascular risk inherent in the condition. : This study aimed to explore the subtle correlation between the Ewing test, Sudoscan-cardiovascular autonomic neuropathy score, and cardiovascular risk calculated using SCORE 2 Diabetes in individuals with T2DM. The methodology involved detailed assessments including Sudoscan tests to evaluate sudomotor function and various cardiovascular reflex tests (CART). The cohort consisted of 211 patients diagnosed with T2DM with overweight or obesity without established ASCVD, aged between 40 to 69 years. : The prevalence of CAN in our group was 67.2%. In the study group, according SCORE2-Diabetes, four patients (1.9%) were classified with moderate cardiovascular risk, thirty-five (16.6%) with high risk, and one hundred seventy-two (81.5%) with very high cardiovascular risk. : On multiple linear regression, the SCORE2-Diabetes algorithm remained significantly associated with Sudoscan CAN-score and Sudoscan Nephro-score and Ewing test score. Testing for the diagnosis of CAN in very high-risk patients should be performed because approximately 70% of them associate CAN. Increased cardiovascular risk is associated with sudomotor damage and that Sudoscan is an effective and non-invasive measure of identifying such risk.
Topics: Humans; Middle Aged; Male; Female; Diabetes Mellitus, Type 2; Adult; Aged; Cardiovascular Diseases; Diabetic Neuropathies; Cohort Studies; Risk Assessment; Autonomic Nervous System Diseases; Heart Disease Risk Factors; Risk Factors
PubMed: 38793011
DOI: 10.3390/medicina60050828 -
Molecular Brain May 2024The aggregated alpha-synuclein (αsyn) in oligodendrocytes (OLGs) is one of the pathological hallmarks in multiple system atrophy (MSA). We have previously reported that...
The aggregated alpha-synuclein (αsyn) in oligodendrocytes (OLGs) is one of the pathological hallmarks in multiple system atrophy (MSA). We have previously reported that αsyn accumulates not only in neurons but also in OLGs long after the administration of αsyn preformed fibrils (PFFs) in mice. However, detailed spatial and temporal analysis of oligodendroglial αsyn aggregates was technically difficult due to the background neuronal αsyn aggregates. The aim of this study is to create a novel mouse that easily enables sensitive and specific detection of αsyn aggregates in OLGs and the comparable analysis of the cellular tropism of αsyn aggregates in MSA brains. To this end, we generated transgenic (Tg) mice expressing human αsyn-green fluorescent protein (GFP) fusion proteins in OLGs under the control of the 2', 3'-cyclic nucleotide 3'-phosphodiesterase (CNP) promoter (CNP-SNCAGFP Tg mice). Injection of αsyn PFFs in these mice induced distinct GFP-positive aggregates in the processes of OLGs as early as one month post-inoculation (mpi), and their number and size increased in a centripetal manner. Moreover, MSA-brain homogenates (BH) induced significantly more oligodendroglial αsyn aggregates than neuronal αsyn aggregates compared to DLB-BH in CNP-SNCAGFP Tg mice, suggestive of their potential tropism of αsyn seeds for OLGs. In conclusion, CNP-SNCAGFP Tg mice are useful for studying the development and tropism of αsyn aggregates in OLGs and could contribute to the development of therapeutics targeting αsyn aggregates in OLGs.
Topics: Animals; Humans; Mice; alpha-Synuclein; Brain; Cytoplasm; Disease Models, Animal; Green Fluorescent Proteins; Inclusion Bodies; Mice, Transgenic; Multiple System Atrophy; Oligodendroglia; Protein Aggregates; Protein Aggregation, Pathological
PubMed: 38790036
DOI: 10.1186/s13041-024-01104-7 -
Pediatric Neurology Jul 2024Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome... (Review)
Review
BACKGROUND
Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature.
METHODS
Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed.
RESULTS
All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care.
CONCLUSIONS
GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.
Topics: Humans; Guillain-Barre Syndrome; Male; Posterior Leukoencephalopathy Syndrome; Child; Adolescent; Child, Preschool
PubMed: 38788278
DOI: 10.1016/j.pediatrneurol.2024.04.018 -
Journal of Neuromuscular Diseases May 2024Motor neuron diseases and peripheral neuropathies are heterogeneous groups of neurodegenerative disorders that manifest with distinct symptoms due to progressive...
Motor neuron diseases and peripheral neuropathies are heterogeneous groups of neurodegenerative disorders that manifest with distinct symptoms due to progressive dysfunction or loss of specific neuronal subpopulations during different stages of development. A few monogenic, neurodegenerative diseases associated with primary metabolic disruptions of sphingolipid biosynthesis have been recently discovered. Sphingolipids are a subclass of lipids that form critical building blocks of all cellular and subcellular organelle membranes including the membrane components of the nervous system cells. They are especially abundant within the lipid portion of myelin. In this review, we will focus on our current understanding of disease phenotypes in three monogenic, neuromuscular diseases associated with pathogenic variants in components of serine palmitoyltransferase, the first step in sphingolipid biosynthesis. These include hereditary sensory and autonomic neuropathy type 1 (HSAN1), a sensory predominant peripheral neuropathy, and two neurodegenerative disorders: juvenile amyotrophic lateral sclerosis affecting the upper and lower motor neurons with sparing of sensory neurons, and a complicated form of hereditary spastic paraplegia with selective involvement of the upper motor neurons and more broad CNS neurodegeneration. We will also review our current understanding of disease pathomechanisms, therapeutic approaches, and the unanswered questions to explore in future studies.
PubMed: 38788085
DOI: 10.3233/JND-240014 -
Journal of Alzheimer's Disease : JAD 2024Higher blood pressure variability (BPV) predisposes to cognitive decline. To investigate underlying mechanisms, we measured 24-h ambulatory BPV, nocturnal dipping and...
Higher blood pressure variability (BPV) predisposes to cognitive decline. To investigate underlying mechanisms, we measured 24-h ambulatory BPV, nocturnal dipping and orthostatic hypotension in 518 participants with vascular cognitive impairment, carotid occlusive disease, heart failure, or reference participants. We determined cross-sectional associations between BPV indices and plasma biomarkers of neuronal injury (neurofilament light chain) and Alzheimer's disease (phosphorylated-tau-181 and Aβ42/Aβ40). None of the BPV indices were significantly associated with any of the biomarkers. Hence, in patients with diseases along the heart-brain axis, we found no evidence for an association between BPV and selected markers of neuronal injury or Alzheimer's disease.
Topics: Humans; Alzheimer Disease; Male; Female; Aged; Biomarkers; Blood Pressure; Amyloid beta-Peptides; Cross-Sectional Studies; tau Proteins; Middle Aged; Peptide Fragments; Neurofilament Proteins; Brain; Hypotension, Orthostatic; Heart Failure; Aged, 80 and over
PubMed: 38788076
DOI: 10.3233/JAD-240119 -
Frontiers in Neurology 2024Guillain-Barré syndrome (GBS) frequently leads to respiratory failure and autonomic dysfunction, resulting in approximately one-third of patients requiring mechanical...
BACKGROUND
Guillain-Barré syndrome (GBS) frequently leads to respiratory failure and autonomic dysfunction, resulting in approximately one-third of patients requiring mechanical ventilation.
OBJECTIVE
This study aimed to identify clinical predictors for mechanical ventilation in patients with GBS.
METHODS
This research was conducted from 2010 to 2021 using registries from a tertiary hospital in an upper middle-income Latin American country. Participants were categorized into two groups based on their ventilation status. Demographic data were collected, and independent predictors of the need for mechanical ventilation were determined through multivariate logistic regression analysis.
RESULTS
Dysautonomic events occurred in 36% of the patients, with 17% requiring mechanical ventilation; the average duration of intubation was 1.16 ± 3.18 days. The multivariate analysis indicated that bulbar dysfunction significantly increased the likelihood of requiring mechanical ventilation by 19-fold (OR 18.67, 95% CI 5.85-59.42), followed by ophthalmoplegia, which increased the likelihood by sixfold (OR 5.68, 95% CI 1.28-25.19).
CONCLUSION
Bulbar dysfunction, dysautonomia, and lower Medical Research Council (MRC) scores were significant predictors of the need for mechanical ventilation in hospitalized GBS patients. These findings support the need for close monitoring and early admission to the intensive care unit (ICU) admission for at-risk patients.
PubMed: 38784912
DOI: 10.3389/fneur.2024.1385945 -
Journal of Education and Health... 2024The pandemic, coronavirus disease 2019 (COVID-19) has led to a heavy toll on the human health. The aim of this study was to determine the influence of body fat...
BACKGROUND
The pandemic, coronavirus disease 2019 (COVID-19) has led to a heavy toll on the human health. The aim of this study was to determine the influence of body fat distribution, evolving long-term effect on autonomic function, and its correlation with Chalder Fatigue Severity Score in post-COVID-19-recovered individuals of Indian ethnicity.
MATERIALS AND METHOD
A case-control study was conducted in the Department of Physiology on 31 cases and 29 age- and gender-matched controls. Cardiovascular evaluation including heart rate variability (HRV), galvanic skin response (GSR), body fat analysis, and Chalder Fatigue Severity Score was performed on the study participants. The continuous variables of basal anthropometric parameters, GSR values, HRV indices, and body fat parameters are expressed as mean and standard deviation (SD).
RESULTS
Diastolic blood pressure (DBP) was significantly increased among cases ( = 0.04). GSR (average) for cases is higher when compared to controls and was borderline significant ( = 0.05). There were no statistically significant differences in the HRV parameters. Cases showed significantly higher body fat distribution as compared to the control group indicating increased susceptibility of the obese population to COVID-19. Chalder's post-COVID-19 Fatigue Severity Score of cases showed a negative correlation with LF:HF and RMSSD but it was not statistically significant.
CONCLUSION
In our study, we conclude that there was a significant increase in DBP and GSR (average) with significantly higher visceral fat percentage, body fat percentage, subcutaneous fat percentage, skeletal muscle percentage, and trunk fat percentage in cases as compared to the control group suggestive of higher propensity of obese individuals suffering from COVID-19 and resulting in dysautonomia as compared to the controls.
PubMed: 38784259
DOI: 10.4103/jehp.jehp_1081_23 -
Journal of Nanobiotechnology May 2024Spinal Cord Injury (SCI) is a condition characterized by complete or incomplete motor and sensory impairment, as well as dysfunction of the autonomic nervous system,... (Review)
Review
Spinal Cord Injury (SCI) is a condition characterized by complete or incomplete motor and sensory impairment, as well as dysfunction of the autonomic nervous system, caused by factors such as trauma, tumors, or inflammation. Current treatment methods primarily include traditional approaches like spinal canal decompression and internal fixation surgery, steroid pulse therapy, as well as newer techniques such as stem cell transplantation and brain-spinal cord interfaces. However, the above methods have limited efficacy in promoting axonal and neuronal regeneration. The challenge in medical research today lies in promoting spinal cord neuron regeneration and regulating the disrupted microenvironment of the spinal cord. Studies have shown that gas molecular therapy is increasingly used in medical research, with gasotransmitters such as hydrogen sulfide, nitric oxide, carbon monoxide, oxygen, and hydrogen exhibiting neuroprotective effects in central nervous system diseases. The gas molecular protect against neuronal death and reshape the microenvironment of spinal cord injuries by regulating oxidative, inflammatory and apoptotic processes. At present, gas therapy mainly relies on inhalation for systemic administration, which cannot effectively enrich and release gas in the spinal cord injury area, making it difficult to achieve the expected effects. With the rapid development of nanotechnology, the use of nanocarriers to achieve targeted enrichment and precise control release of gas at Sites of injury has become one of the emerging research directions in SCI. It has shown promising therapeutic effects in preclinical studies and is expected to bring new hope and opportunities for the treatment of SCI. In this review, we will briefly outline the therapeutic effects and research progress of gasotransmitters and nanogas in the treatment of SCI.
Topics: Spinal Cord Injuries; Humans; Animals; Gasotransmitters; Nitric Oxide; Neuroprotective Agents; Hydrogen Sulfide; Carbon Monoxide; Oxygen; Spinal Cord; Hydrogen
PubMed: 38783332
DOI: 10.1186/s12951-024-02523-3