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

Abstract

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.

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

“…Recent developments in ventilation management have considerably improved the survival time of patients with ALS . However, while patients are able to survive longer by use of TIV, their ALS continues to progress, leading to total quadriplegia, ophthalmoplegia and communication impairments including a totally locked‐in state, and unexpected non‐motor manifestations or complications . While the most common cause of death in ALS patients with TIV is respiratory tract infection, we often encounter other critical manifestations and complications, which were summarized in the present study.…”

“…The communication impairment seen in advanced stages of ALS is caused by not only motor disability but also by ophthalmoplegia and cognitive dysfunction. Recently, we have developed a method to categorize the stages of ALS by focusing on communication impairments, particularly for patients with TIV . This method can be easily applied to patients with severely deteriorating motor functions who cannot be fully assessed using conventional grading scales, such as the revised ALS functional rating scale or the Norris score .…”

“…For clinical assessments, we evaluated the following factors: sex, age at onset, body region affected at onset, and disease duration. The time from ALS onset to the following clinical events was determined as reported previously: need for TIV, feeding tube placement, development of overt oculomotor limitations, and progression of motor disability to total quadriplegia. Overt oculomotor limitations were defined as persistently abnormal ocular movements during a bedside examination by at least 2 neurologists.…”

“…Patients were classified into two subgroups by three types of categorization: patients with TIV use for <5 or ≥ 5 years, patients with total disease duration <10 or ≥ 10 years, and patients with different stages of communication impairment at the individual terminal stages or at the end of the study (Stages I–III or Stages IV–V). The cutoff duration of TIV use (5 years) was adopted according to the previous report and was considered as an adequate duration for the appearance of ophthalmoplegia in ALS patients using TIV . Statistical analyses were performed using the Student's t ‐test or chi‐square test for data comparisons between subgroups, and for analysis of the prevalence of non‐motor manifestations or complications.…”

“…Mechanical ventilation is effective for prolonging the survival of patients with ALS beyond respiratory failure, and tracheostomy and invasive ventilation (TIV) in particular may extend survival by several years and maintain the quality of life of patients . In Japan, 29.3% of ALS patients decide to live with TIV, resulting in survival for 10 to 30 years or even longer . Multiple factors are involved in survival prolongation with TIV, including the provision of multidisciplinary care at home, the use of respiratory and nutritional care techniques, and infection management.…”