Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients

Watanabe, H.; Atsuta, Naoki; Nakamura, Ryoichi; Hirakawa, Akihiro; Watanabe, Haruo; Ito, Mizuki; Senda, Joe; Katsuno, Masahisa; Izumi, Yuishin; Morita, Mitsuya; Tomiyama, Hiroyuki; Taniguchi, Akira; Aiba, Ikuko; Abe, Kōji; Mizoguchi, Kouichi; Oda, Masaya; Kano, Osamu; Okamoto, Koichi; Kuwabara, Satoshi; Hasegawa, Kazuko; Imai, Takashi; Akiyama, Masashi; Tsuji, Shoji; Nakano, Imaharu; Kaji, Ryuji; Sobue, Gen

doi:10.3109/21678421.2014.990036

Cited by 80 publications

(75 citation statements)

References 32 publications

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“…Notably, only some of these factors resulted also independent prognostic factors for ALS survival (age, site of onset and DFS), suggesting that survival and ALSFRS-R can be influenced by different factors. These findings, are in accordance with a recent Japanese study [16], and could be explained by the : a progressive decline in the ALSFRS-R total score may represent loss of one or more functions affecting in different ways ALS survival as patients with the same score may have very different progression and clinical presentation. For example, score 0 or 1 at item 12 (respiratory insufficiency) of the ALSFRS-R has a different meaning in terms of survival than a score 0 or 1 at item 9 (climbing stairs).…”

Section: Discussionsupporting

confidence: 91%

“…Since now ALSFRS-R has been studied in patients included in clinical trials or in patients attending a tertiary ALS Centre [16], with a limited generalizability of the studies' findings to the entire ALS population, due to exclusion of the elderly and more advanced patients [21], and to exclusion of the patients who do not meet El Escorial Criteria for probable or definite ALS.…”

Section: Discussionmentioning

confidence: 99%

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Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

Mandrioli¹,

Biguzzi

Guidi³

et al. 2015

Neurol Sci

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Very few studies examined trend over time of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and factors influencing it; previous studies, then, included only patients attending tertiary ALS Centres. We studied ALSFRS-R decline, factors influencing this trend and survival in a population-based setting. From 2009 onwards, a prospective registry records all incident ALS cases among residents in Emilia Romagna (population: 4.4 million). For each patient, demographic and clinical details (including ALSFRS-R) are collected by caring physicians at each follow-up. Analysis was performed on 402 incident cases (1279 ALSFRS-R assessments). The average decline of the ALSFRS-R was 0.60 points/month during the first year after diagnosis and 0.34 points/month in the second year. ALSFRS-R decline was heterogeneous among subgroups. Repeated measures mixed model showed that ALSFRS-R score decline was influenced by age at onset (p < 0.01), phenotype (p = 0.01), body mass index (BMI) (p < 0.01), progression rate at diagnosis (ΔFS) (p < 0.01), El Escorial Criteria-Revised (p < 0.01), and FVC% at diagnosis (p < 0.01). Among these factors, at multivariate analysis, only age, site of onset and ΔFS independently influenced survival. In this first population-based study on ALSFRS-R trend, we confirm that ALSFRS-R decline is not homogeneous among ALS patients and during the disease. Factors influencing ALSFRS-R decline may not match with those affecting survival. These disease modifiers should be taken into consideration for trials design and in clinical practice during discussions with patients on prognosis.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

Mandrioli¹,

Biguzzi

Guidi³

et al. 2015

Neurol Sci

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“…Previous epidemiological studies have shown several factors related to shorter survival in ALS: older age (6,15,16), bulbar onset (15,16,17), a reduction in forced vital capacity (16), a rapid reduction in body mass index (18,19), a rapid decrease in the revised ALS Functional Rating Scale (ALSFRS-R) score (16,20), and psychosocial factors (21). A high ALSFRS-R score at TIV initiation also predicts a long-term survival after TIV (22).…”

Section: Discussionmentioning

confidence: 99%

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

Nakayama

Shimizu

Mochizuki

et al. 2015

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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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.

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“…In Japan and South Korea, the mean age of disease onset is 61 and 58 years, respectively,11 12 but other Asian cohorts have suggested a younger age of disease onset compared with Caucasians. In China, the mean age is approximately 52 years,13–15 whereas in a single study from India, the age of onset was 46 years 16.…”

Section: Epidemiologymentioning

confidence: 99%

Amyotrophic lateral sclerosis and motor neuron syndromes in Asia

Shahrizaila

Sobue

Kuwabara

et al. 2016

J Neurol Neurosurg Psychiatry

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While the past 2 decades have witnessed an increasing understanding of amyotrophic lateral sclerosis (ALS) arising from East Asia, particularly Japan, South Korea, Taiwan and China, knowledge of ALS throughout the whole of Asia remains limited. Asia represents >50% of the world population, making it host to the largest patient cohort of ALS. Furthermore, Asia represents a diverse population in terms of ethnic, social and cultural backgrounds. In this review, an overview is presented that covers what is currently known of ALS in Asia from basic epidemiology and genetic influences, through to disease characteristics including atypical phenotypes which manifest a predilection for Asians. With the recent establishment of the Pan-Asian Consortium for Treatment and Research in ALS to facilitate collaborations between clinicians and researchers across the region, it is anticipated that Asia and the Pacific will contribute to unravelling the uncertainties in ALS.

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Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients

Abstract: Background

Cited by 80 publications

References 32 publications

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

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

Amyotrophic lateral sclerosis and motor neuron syndromes in Asia

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