Search

Objective: We proposed a novel palliative care scale (Amyotrophic Lateral Sclerosis Palliative Care Scale: ALSPCS) for patients with ALS, and analyzed the suffering reported by patients., Methods: Thirty-one patients participated in the study. The ALSPCS has 15 items to evaluate physical and psychological suffering; patients scored their subjective suffering on a scale of 0-5 for each item. This study analyzed 13 of 15 items., Results: The mean scores obtained from the patients were as follows: 'dyspnea', 2.5; 'pain', 2.4; 'restlessness', 2.4; 'thirst', 3.0; 'burning sensation', 2.0; 'choking', 2.0; 'nausea', 0.4; 'constipation', 1.5; 'insomnia', 2.5; 'anxiety', 3.5; 'loneliness', 2.4; 'irritation', 2.1; and 'communication difficulty', 2.3. Multiple correlation analysis using Spearman's rank correlation coefficient showed significant correlations of dyspnea with restlessness, thirst, burning sensation and anxiety; of restlessness with dyspnea, thirst, loneliness and irritation; and of anxiety with dyspnea, thirst and loneliness (P < 0.0038 after Bonferroni's correction). In the principal component analysis, every item showed a positive loading value in the first principal component. Dyspnea, restlessness, thirst, anxiety, loneliness and irritation had loading values >0.7; thus, these symptoms might be the main features in ALS patients. The total scores or each ALSPCS score showed no significant association with post-assessment survival period., Conclusion: This study, using ALSPCS, showed that the subjective suffering of ALS patients was variable and strongly correlated with each other. Appropriate and comprehensive assessment of physical and psychological affliction with ALSPCS could be potentially useful in verifying the effectiveness of palliative care for end-of-life stage ALS patients in the future.

The effect of malnutrition on intractable neurological diseases, including neurodegenerative diseases, is variable. Nutritional status is dependent on various factors such as disease characteristics; various symptoms including dysphagia, respiratory dysfunction, motor weakness, muscle rigidity, involuntary movement, and ataxia; and changes in energy metabolism caused by diseases and its stage. Nutritional therapy for patients with intractable neurological diseases requires the provision of tailor-made supports for individual patients based on sharing of knowledge and experience in multidisciplinary members. Evidence on nutritional management in the field of neurology is limited compared to that in the fields of surgery and internal medicine. This article thus aimed to describe the activities of the nutrition support team (NST) at our hospital that is specialized in intractable neurological diseases and the knowledge obtained through the activities.

The Awaji criteria proposed in 2008 re-evaluated electrophysiological findings and emphasized the diagnostic significance of fasciculation potentials (FPs) in amyotrophic lateral sclerosis (ALS). FPs were regarded to be equivalent to denervation potentials (fib-psw) in terms of clinical significance. The implication is that FPs are closely related to the progressive denervation process of muscles in ALS. The characteristic features of FP in ALS are complexity and instability. Complex FPs (CFPs) are thought to originate in distal axonal sprouts, associated with the reinnervation process. These differ from FPs observed in other pathological states, which are thought to originate from anterior horn or nerve root. CFPs may be useful for early diagnosis of ALS patients and should be evaluated in the EMG examination.