Background/objective: In this study, we demonstrated the effects of obstructive sleep apnea syndrome (OSAS) on the prognosis of amyotrophic lateral sclerosis (ALS). However, the effects of sleep-related disorders other than breathing problems are still waiting to be delineated.
Methods: In this longitudinal retrospective and prospective study, we investigated 73 patients with ALS compared to 20 healthy subjects, to determine sleep-related disorders and their impact on disease prognosis.
Results: In patients with ALS, the mean respiratory disturbance index (RDI) was 28.0/h, which was significantly higher than that in the controls (p < 0.001). OSAS was present in 67% of the patients, sleep-related hypoxemia was observed in 13.7% of the patients, and 5.4% had central sleep apnea syndrome. In patients with bulbar-onset disease, higher RDI was almost significantly associated with lower survival (p = 0.056). The mean index of periodic leg movements in sleep was significantly higher in patients with ALS (34.0 ± 19.9/h) than that in the controls (12.5 ± 15.5/h; p < 0.001). Periodic leg movements disorder (PLMD) was diagnosed in 23 patients with ALS (31.5%); fragmentary myoclonus was present in 13.7% of patients, and REM-sleep behavior disorder was diagnosed in 4 patients (5.4%). The presence of PLMD in addition to OSAS was significantly associated with worse prognosis and poorer survival (p = 0.040).
Conclusions: These findings emphasize that sleep-related disorders other than OSAS deserve attention in ALS. ALS is a catastrophic and frustrating disease for both patients and physicians; thus, the diagnosis and treatment of comorbid sleep disorders could improve the survival of patients with ALS.
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http://dx.doi.org/10.1159/000505575 | DOI Listing |
Br J Haematol
January 2025
Institut d'Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Barcelona, Spain.
VEXAS syndrome is a haemato-inflammatory disease caused by somatic UBA1 mutations and characterized by cytoplasmic vacuoles in myeloid and erythroid precursor cells. Although there is currently no standard treatment algorithm for VEXAS, patients are generally treated with anti-inflammatory therapies focused on symptom management, with only partial effectiveness. Hypomethylating agents (HMA) have shown promise in VEXAS patients with concomitant myelodysplastic syndrome (MDS), while the efficacy of HMA in VEXAS patients without MDS is largely unknown.
View Article and Find Full Text PDFJ Electromyogr Kinesiol
January 2025
Hacettepe University, Faculty of Medicine, Department of Neurology, Ankara, Turkey.
Objectives: Jitter analysis with concentric needle electrode of the thoracic 9 (T9) paraspinal muscle (PM), where the needle EMG examination at rest is difficult, was performed in both amyotrophic lateral sclerosis (ALS) patients and the controls.
Methods: For the T9 PM, both upper limit for mean and individual mean consecutive difference (MCD) values and spike numbers were calculated according to jitter values of pairs from controls. In addition to the descriptive statistics, differences between two groups and T9 PM needle EMG and jitter analysis findings of patients were compared (p = 0.
Neurogenetics
January 2025
Department of Biomedical Science, Faculty of Medicine, University of Malaya, Kuala Lumpur, 50603, Malaysia.
Intermediate CAG repeats from 29 to 33 in the ATXN2 gene contributes to the risk of amyotrophic lateral sclerosis (ALS) in European and Asian populations. In this study, 148 ALS patients of multiethnic descent: Chinese (56.1%), Malay (24.
View Article and Find Full Text PDFScand J Prim Health Care
January 2025
Center for General Practice, Aalborg University, Aalborg, Denmark.
Introduction: Polypharmacy is widespread. The demographic shift toward older patients receiving multiple medications increases risk and drug-related problems in these patients.
Objective: To investigate patient perspectives on polypharmacy and the experienced effects of medication reviews by pharmacists in general practice.
Ther Adv Neurol Disord
January 2025
Department of Neurology, The Second Hospital of Hebei Medical University, 215 Heping West Road, Shijiazhuang, Hebei 050000, P.R. China.
Background: Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and rare neurodegenerative disease. Therefore, evaluating the risk factors affecting the survival of patients with ALS is crucial. Constipation, a common but overlooked symptom of ALS, can be effectively managed.
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