Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Spreading pattern and time interval of spreading are getting more and more attention. The aim of present study was to investigate spreading pattern in bulbar onset ALS patients and to explore the relationship between time interval of spreading and survival.
Methods: ALS patients with bulbar onset diagnosed at Chinese PLA General Hospital from January 2015 to December 2018 were recruited. Clinical features including gender, onset age, diagnostic delay, the second involved region (SIR), time of symptoms beyond the bulbar region, forced vital capacity (FVC), ALSFRS-R score, electromyography results, and survival time were retrospectively collected.
Results: A total of 96 bulbar onset ALS patients were collected. Overall patients showed female predominance. Median age at onset was 56 years. Median diagnostic delay was 8.5 months. Median time of symptoms beyond the bulbar region (TBBR) was 7 months. Median ALSFRS-R score at baseline was 40. Fifty-six (58.3%) patients' SIR were upper limb, 6 (6.3%) patients' SIR were lower limb, 3 (3.1%) patients' SIR were upper and lower limbs, and 5 (5.2%) patients' SIR were thoracic region. Twenty-six (27.1%) patients did not report SIR. The median survival time of patients with TBBR ≥ 7 months was significantly longer than that with TBBR < 7 month. Multivariate Cox regression showed that onset age and TBBR were prognostic factors.
Conclusions: In bulbar onset ALS patients, cervical region is the second most common SIR. TBBR is an independent prognostic factor.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s10072-021-05556-w | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients.
Ther Adv Neurol Disord
January 2025
Department of Neurology, Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Beijing 100053, China.
Background: Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes.
Objectives: To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years.
Interaction between riluzole treatment and dietary glycemic index in the disease progression of amyotrophic lateral sclerosis.
Ann Clin Transl Neurol
January 2025
Department of Epidemiology, Columbia University Mailman School of Public Health, New York, USA.
Objective: We examined whether riluzole treatment modifies the associations between the dietary glycemic index (GI) and load (GL) and disease progression in amyotrophic lateral sclerosis (ALS).
Methods: Sporadic ALS patients in the Multicenter Cohort Study of Oxidative Stress who completed a baseline food frequency questionnaire were included (n = 304). Interactions between baseline riluzole treatment and GI/GL on functional decline and tracheostomy-free survival were examined using linear regression and Cox proportional hazard models adjusted for covariates.
Lesion Topography and Clinical Features Associated With Respiratory Failure in Patients With Medullary Infarction.
Brain Behav
January 2025
Department of Neurology, Tianjin Key Laboratory of Cerebrovascular and Neurodegenerative Diseases, Clinical College of Neurology, Neurosurgery and Neurorehabilitation, Tianjin Huanhu Hospital, Tianjin Medical University, Tianjin, China.
Background: Respirator failure (RF) is a severe malignant complication in both lateral medullary infarction (LMI) and medial medullary infarction (MMI) patients. However, the differences in clinical and radiological manifestations associated with RF between patients with LMI and MMI have not been fully elucidated.
Methods: A total of 435 consecutive patients with MI within 7 days of onset in our institute were retrospectively enrolled from January 2017 to January 2024.
Survival Prognostic Factors of Non-Invasive Ventilation in Amyotrophic Lateral Sclerosis: A Systematic Review.
Life (Basel)
December 2024
Department of Pathological Physiology, Institute of Digital Biodesign and Modelling of Living Systems, I.M. Sechenov First Moscow State Medical University (Sechenov University), 119991 Moscow, Russia.
Objective: Amyotrophic lateral sclerosis is a neurodegenerative disease with high rates of disability and mortality. Non-invasive ventilation (NIV) is an effective method of treating patients, increasing life expectancy, but currently, predictors available to determine the best outcome of therapy in this category of patients are unknown. This systematic review aimed to determine the impact of prognostic factors on benefits from NIV application compared with non-NIV tools of treatment (invasive ventilation and standard care) in case of survival of ALS patients.
View Article and Find Full Text PDFClinical features of FOSMN syndrome in Korea: A comparative analysis with bulbar-onset amyotrophic lateral sclerosis.
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 PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!