Objective: To evaluate the care status of the amyotrophic lateral sclerosis (ALS) patients with long-term use of tracheostomy tube by caregivers of ALS patients.
Methods: A survey was conducted in the form of questionnaires to ALS patients and their caregivers. All measurements were performed by two visiting nurses. For statistical analysis, SPSS ver. 22.0 and Mann-Whitney U test on non-normal distribution were used.
Results: In total, 19 patients (15 males and 4 females) and their caregivers participated in the survey. In the case of patients, the average duration of care was 5.9±3.7 years, and the mean periods of illness and tracheostomy were 5.3±3.2 years and 3.0±2.6 years, respectively. Replacement intervals were 14 days in 11 patients, 7 days in 4 patients, 28 days in 2 patients, and 21 days in 1 patient. One patient was unable to provide an accurate replacement interval. Eighteen (99%) caregivers had experience of adding volume to a cuff without pressure measure in the following instances: due to patients' needs in 7 cases, air leakage in 7 cases, and no reason in 4 cases. Mean pressure of tracheostomy cuff was 40±9.4 cmH2O, and air volume of tracheostomy cuff was 6.7±3.2 mL, but real mean volume was 7.0±2.9 mL. The number of suctioning for airway clearance was a mean 27.5±18.2 times a day.
Conclusion: According to this survey, we notice that almost all the patients and caregivers had an erroneous idea about cuff volume and pressure. Moreover, education and long-term professional care of tracheostomy cannot be overemphasized in this manner.
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http://dx.doi.org/10.5535/arm.2015.39.6.964 | DOI Listing |
J Neurol Sci
December 2024
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea; Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea; Wide River Institute of Immunology, Seoul National University, Hongcheon, Republic of Korea; Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address:
Facial onset sensory and motor neuronopathy (FOSMN) syndrome is a rare neurodegenerative disorder initially characterized by facial sensory deficits, which later progress to motor deficits in a rostral-caudal distribution. This study investigated the prevalence, clinical features, and prognosis of FOSMN syndrome and compared these aspects with those of bulbar-onset amyotrophic lateral sclerosis (ALS) within a single institutional cohort of motor neuron diseases. We identified four patients with FOSMN syndrome who had been misclassified as having bulbar-onset ALS, representing approximately 2 % of such ALS cases.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
January 2025
Neurology Department, LR18SP03, Razi University Hospital, Tunis, Tunisia.
Amyotrophic Lateral Sclerosis(ALS) has traditionally been managed as a neuromuscular disorder. However, recent evidence suggests involvement of non-motor domains. This study aims to evaluate the impact of APOE and MAPT genotypes on the cognitive features of ALS.
View Article and Find Full Text PDFBackground: Inclusions of TAR DNA binding protein of 43kDa (TDP-43) constitute the main characteristic pathology in the majority (∼97%) of amyotrophic lateral sclerosis (ALS) cases and approximately 50% of patients with frontotemporal lobar degeneration (FTLD). TDP-43 is a nuclear RNA binding protein; however, in disease, it becomes hyperphosphorylated and/or insoluble, hindering its nuclear function in maintaining RNA homeostasis. Importantly, the incidence of TDP-43 proteinopathy extends to aging brains (LATE) and may be concomitant with Alzheimer's disease (AD) neuropathological changes (LATE/AD) in up to 70% of AD patients.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
Background: Glaucoma is characterized by progressive optic nerve degeneration that results in irreversible blindness, and it can be considered a neurodegenerative disorder of both the eye and the brain. Increasing evidence suggest that glaucoma shares some common neurodegenerative pathways with Frontotemporal Lobar Degeneration (FTLD), Amyotrophic Lateral Sclerosis (ALS), and Alzheimer's Disease (AD) among others. Interestingly, a recent study revealed the presence of abnormal TAR DNA-binding protein 43 (TDP-43) inclusions and aggregates in retinal ganglion cells and other retinal cell types in FTLD-TDP patients; however, the significance of this pathology and its impact on retinal function and optical nerve integrity is unknown.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Barrow Neurological Institute, Phoenix, AZ, USA; Arizona State University, Tempe, AZ, USA.
Background: TDP-43 is an RNA binding protein that is a pathological hallmark of multiple neurodegenerative diseases including Amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and Alzheimer's disease (AD). The frequency of observed TDP-43 pathology is estimated at 97% in ALS, 45% in FTD and 40-57% in AD and is characterized by a mislocalization of TDP-43 from the nucleus to the cytoplasm. Indeed, TDP-43 is the third most common proteinopathy in AD, behind only Amyloid beta and Tau.
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