Yuki Nakayama

Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: A multicenter study in Japan

Malnutrition in the early stage has been reported as an independent predictor of survival in amyotrophic lateral sclerosis (ALS). We analyzed retrospectively the effect of variation of body mass index (BMI) on survival in ALS patients. In total, 77 consecutive ALS patients were enrolled from nine hospitals in Japan. Reduction rate of BMI was calculated from BMI before the disease onset and at the time of the first visit to each hospital. We analyzed the correlation between BMI reduction rate and total disease duration. Results showed that the median BMI reduction rate was 2.5 per year (interquartile range 1.3–3.8). The BMI reduction rate was significantly correlated with survival length (p <0.0001). There was also a significant difference in survival between ALS patients with a BMI reduction rate ≥ and < 2.5 (Kaplan-Meier survival analysis and the log-rank test, p < 0.0001; hazard ratio by the Cox model, 2.9816). In conclusion, faster reduction of BMI at the initial stage before the first visit to hospital predicts shorter survival length also in Japanese ALS patients.

Systematic review and meta-analysis of studies involving telehome monitoring-based telenursing for patients with chronic obstructive pulmonary disease.

AIM This systematic review evaluated the effects of telehome monitoring-based telenursing (THMTN) on health outcomes and use of healthcare services and compared them with the effects of conventional treatment in patients with severe and very severe chronic obstructive pulmonary disease (COPD). METHODS An extensive published work search of several databases was performed in May and October 2011. Randomized controlled trials and non-randomized controlled clinical trials were evaluated. Parameters included hospitalization rate, number of visits to the emergency department, exacerbations, mean number of hospitalizations, mean duration of bed days of care, mortality, and health-related quality of life by the duration of THMTN and COPD severity. A random effects model was applied. Risk ratio and mean difference were calculated. Heterogeneity was assessed using the I(2) statistic. RESULTS Nine original articles involving 550 participants were identified in the meta-analysis. THMTN decreased hospitalization rates, emergency department visits, exacerbations, mean number of hospitalizations, and mean duration of bed days of care in severe and very severe COPD patients. Hospitalization rates and emergency department visits were comparable between patients undergoing THMTN of different durations. In addition, THMTN had no effect on mortality. CONCLUSION THMTN significantly decreases the use of healthcare services; however, it does not affect mortality in severe and very severe COPD patients.

Predictors of impaired communication in amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation

Predictors of communication impairment in patients with amyotrophic lateral sclerosis (ALS) using tracheostomy-invasive ventilation (TIV) were investigated. Seventy-six ALS patients using TIV were enrolled and classified into three subgroups of communication ability: patients who could communicate with communication devices (Stage I), patients who had difficulty with communication (Stage II, III, or IV), and patients who could not communicate by any means (Stage V). Predictors of communication impairment were analysed by the Cox proportional hazard model. Results demonstrated that there were no significant differences in disease duration between subgroups. Within 24 months after disease onset, patients who needed TIV and tube feeding, developed oculomotor impairment or became totally quadriplegic and progressed from Stage I to II and V significantly earlier. Multivariate analyses revealed that within 24 months from onset, the need for TIV and progression to total quadriplegia were significant events in patients who progressed to Stage II, whereas the development of oculomotor limitation was significant in patients who progressed to Stage V. In conclusion, TIV, impaired oculomotor movement and total quadriplegia are predictors of severe communication impairment. Rapid disease progression might indicate future communication impairment after the use of TIV. We highly recommend early detection of impaired communication and identification of the best methods of communication.

Marked preservation of the visual and olfactory pathways in ALS patients in a totally locked-in state.

MATERIALS AND METHODS The present paper examines the brains and spinal cords in 7 patients with amyotrophic lateral sclerosis (ALS) receiving artificial respirator support in a totally locked-in state (TLS) neuropathologically in order to clarify whether any anatomical structures in the central nervous system are preserved. RESULTS AND CONCLUSION We found that the visual and olfactory pathways, hypothalamus, nucleus basalis of Meynert, and commissura anterior were remarkably well preserved, whereas the somatosensory, auditory, and gustatory pathways in the brain stem and/or spinal cord showed severe deterioration.

ALS patients with ability to communicate after long-term mechanical ventilation have confined degeneration to the motor neuron system

OBJECTIVE To clarify the position in the amyotrophic lateral sclerosis (ALS) spectrum, of a subgroup of patients who maintained the ability to communicate after long-term mechanical ventilation (LTMV) by tracheostomy. METHODS We undertook a clinicopathological investigation of sporadic ALS in three patients who maintained the ability to communicate after approximately 30-year survival on LTMV by tracheostomy. RESULTS The age of onset and duration of disease was 48 years and 31 years in patient 1, 55 years and 29 years in patient 2, and 31 years and 33 years in patient 3, respectively. Each patient displayed slow disease progression. In all patients, both upper and lower motor neurons were markedly degenerated, while other neuronal systems and the brainstem tegmentum were spared. A few normal-looking motor neurons remained in the anterior horn of the spinal cord. There were no TAR DNA-binding protein 43-immunoreactive inclusions in the lower motor neurons in any patient and only occasional inclusions in the cerebral cortex of one patient. CONCLUSION The clinicopathological findings of these three patients suggest that there is a distinct subgroup of ALS patients characterized by the above-mentioned features.

The measurement and estimation of total energy expenditure in Japanese patients with ALS: a doubly labelled water method study

Abstract Appropriate nutritional therapy has not been established for patients with amyotrophic lateral sclerosis (ALS). Our objective was to measure the total energy expenditure (TEE) and determine an equation to estimate the energy requirements for Japanese patients with ALS. Twenty-six Japanese patients with ALS participated in the study. The TEE was measured using the doubly labelled water (DLW) method for a 14-day period. Using a range of clinical parameters and multiple regression analyses, we determined an adequate equation to calculate TEE. Results showed that the median value of total energy intake (TEI) was 1581 (interquartile 1278–1782) kcal/d. TEE and TEE/body weight were 1628 kcal/d (1352–1865) and 31.3 kcal/kg (29.2–34.4), respectively. The ratio of TEE/estimated TEE by the Harris-Benedict equation was 1.14 (1.09–1.26). The difference between TEI and TEE was –63 kcal (–221 – 122), and 15 patients (57.7%) showed a negative balance. From regression analyses, we determined an equation to estimate TEE using the resting metabolic rate estimated by the Harris-Benedict equation (RMR-HB) and scores of the revised ALS Functional Rating Scale (ALSFRS-R): TEE = (1.67 × RMR-HB) + (11.8 × ALSFRS-R) – 680 (p < 0.0001). In conclusion, energy expenditure of Japanese patients with ALS was higher than expected, and we proposed a preliminary equation to estimate TEE for future nutritional intervention.

Non-motor manifestations in ALS patients with tracheostomy and invasive ventilation

This study aimed to investigate non‐motor manifestations in amyotrophic lateral sclerosis (ALS) patients with tracheostomy and invasive ventilation (TIV) and their relevance to disease progression.

Predictive factors for prognosis following unsedated percutaneous endoscopic gastrostomy in ALS patients

Introduction: This study aimed to determine the prognostic factors and the values that predict survival after percutaneous endoscopic gastrostomy (PEG) tube placement in patients with amyotrophic lateral sclerosis (ALS). Methods: We retrospectively analyzed the correlations for 97 consecutive patients with ALS between clinical parameters and survival following PEG tube placement using the log‐rank test and Cox proportional‐hazards models. Results: The log‐rank test showed that an arterial carbon dioxide pressure (PaCO2) of ≤ 40 mmHg (P = 0.0054), a forced vital capacity (FVC) of ≥ 38% of predicted (P = 0.0003), and bulbar‐onset (P = 0.0121) were significantly associated with better post‐PEG survival. Multivariate analysis showed that the FVC and PaCO2 were associated with better post‐PEG survival (P = 0.0081 and P = 0.0265, respectively). Conclusions: PEG tube placement in ALS is recommended when FVC is ≥ 38% of predicted and when PaCO2 is normal. Muscle Nerve 54: 277–283, 2016

Macroglossia in advanced amyotrophic lateral sclerosis

Introduction: An enlarged tongue (macroglossia) has been reported in advanced‐stage patients with amyotrophic lateral sclerosis (ALS). Methods: In this study we examined the prevalence of macroglossia and analyzed clinical correlations in 65 ALS patients on tracheostomy‐invasive ventilation (TIV). Results: Macroglossia was found in 22 patients (33.8%). Compared with those without macroglossia, patients with macroglossia had a younger age of onset, longer duration of disease and TIV use, lower ALS Functional Rating Scale score, higher body mass index, lower energy intake, more severe communication impairment, and lower oral function. Logistic multivariate analysis showed that body mass index (BMI; P = 0.007) and communication impairment (P = 0.029) were significantly correlated with macroglossia. The duration of TIV use was at the cut‐off level of significance (P = 0.05). Conclusions: Macroglossia may be the result of overfeeding and replacement by fat during long‐term TIV use in patients with advanced ALS. Muscle Nerve, 2016 Muscle Nerve 54: 386–390, 2016

Sensory cortex hyperexcitability predicts short survival in amyotrophic lateral sclerosis

Objective To investigate somatosensory cortex excitability and its relationship to survival prognosis in patients with amyotrophic lateral sclerosis (ALS). Methods A total of 145 patients with sporadic ALS and 73 healthy control participants were studied. We recorded compound muscle action potential and sensory nerve action potential of the median nerve and the median nerve somatosensory evoked potential (SEP), and we measured parameters, including onset-to-peak amplitude of N13 and N20 and peak-to-peak amplitude between N20 and P25 (N20p-P25p). Clinical prognostic factors, including ALS Functional Rating Scale–Revised, were evaluated. We followed up patients until the endpoints (death or tracheostomy) and analyzed factors associated with survival using multivariate analysis in the Cox proportional hazard model. Results Compared to controls, patients with ALS showed a larger amplitude of N20p-P25p in the median nerve SEP. Median survival time after examination was shorter in patients with N20p-P25p ≥8 μV (0.82 years) than in those with N20p-P25p <8 μV (1.68 years, p = 0.0002, log-rank test). Multivariate analysis identified a larger N20p-P25p amplitude as a factor that was independently associated with shorter survival (p = 0.002). Conclusion Sensory cortex hyperexcitability predicts short survival in patients with ALS.