Amyotrophic lateral sclerosis & frontotemporal degeneration
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2018
"ALS reversals": demographics, disease characteristics, treatments, and co-morbidities.
To identify differences in demographics, disease characteristics, treatments, and co-morbidities between patients with "amyotrophic lateral sclerosis (ALS) reversals" and those with typically progressive ALS. ⋯ When compared to patients with typically progressive ALS, patients with reversals differed in their demographics, disease characteristics, and treatments. While some of these patients may have had a rare antibody-mediated ALS mimicker, such as atypical myasthenia gravis, details of their exams, EMGs and family histories argue that this was unlikely. Instead, our data suggest that ALS reversals warrant evaluation for mechanisms of disease resistance and that treatments associated with multiple ALS reversals deserve further study.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2018
Very late-onset amyotrophic lateral sclerosis in a Portuguese cohort.
Although amyotrophic lateral sclerosis (ALS) incidence has been stable among Western countries, population-ageing effect will probably increase the proportion of very-old ALS patients. We aim to study this population. ⋯ Very-old patients represent a minor but distinctive ALS group. A predominant bulbar presentation was disclosed and it could probably explain the shorter disease duration before first visit as well as survival. Older age was not an exclusion factor for good health care practices, in particular NIV and riluzole prescription.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2018
Randomized Controlled TrialRelationships between slow vital capacity and measures of respiratory function on the ALSFRS-R.
As declining respiratory muscle function commonly leads to disability and death in amyotrophic lateral sclerosis (ALS), respiratory measurements such as slow vital capacity (SVC) may predict disease progression. This study evaluated the relationship between SVC and symptoms measured by the revised ALS Functional Rating Scale (ALSFRS-R). ⋯ The higher correlation between %predicted SVC and specific ALSFRS-R symptom scores in patients with rapidly versus more slowly progressing disease reinforces the importance of continually monitoring respiratory function throughout the disease course.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2018
Caregiver burden and quality of life of patients with amyotrophic lateral sclerosis in India.
Amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) is a progressive degenerative disorder that can have significant debilitating impact. Few studies have explored living with ALS in the developing countries. The study aims to understand the relationship between functionality, quality of life, and caregiver burden in ALS in the sociocultural scenario in India. ⋯ ALS patients and caregivers would benefit from structured care plan that is sensitive to the impact of the illness on the specific domains of quality of life as well as the deterioration in the neurological functioning.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2018
Epidemiology and diagnostic process of amyotrophic lateral sclerosis as distinct from myelopathy: 5-year cohort study of whole-population in South Korea.
To investigate the incidence, prevalence, and demographic factors of all amyotrophic lateral sclerosis (ALS) patients diagnosed in South Korea from 2011 to 2015, and to analyze cases misdiagnosed as myelopathy. ⋯ This whole-population nationwide demographic study confirmed the data from previous studies. Clinicians should consider the possibility of ALS when making a myelopathy diagnosis, especially if the symptoms are sufficiently severe to require surgery.