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Journal of Neurology
Published in: Journal of Neurology 10/2020

01-10-2020 | Amyotrophic Lateral Sclerosis | Original Communication

Nationwide survey of 780 Japanese patients with amyotrophic lateral sclerosis: their status and expectations from brain–machine interfaces

Authors: Yu Kageyama, Xin He, Toshio Shimokawa, Jinichi Sawada, Takufumi Yanagisawa, Morris Shayne, Osamu Sakura, Haruhiko Kishima, Hideki Mochizuki, Toshiki Yoshimine, Masayuki Hirata

Published in: Journal of Neurology | Issue 10/2020

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Abstract

Background

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that causes eventual death through respiratory failure unless mechanical ventilation is provided. Brain–machine interfaces (BMIs) may provide brain control supports for communication and motor function. We investigated the interests and expectations of patients with ALS concerning BMIs based on a large-scale anonymous questionnaire survey supported by the Japan Amyotrophic Lateral Sclerosis Association.

Methods

We surveyed 1918 patients with ALS regarding their present status, tracheostomy use, interest in BMIs, and their level of expectation for communication (conversation, emergency alarm, internet, and writing letters) and movement support (postural change, controlling the bed, controlling household appliances, robotic arms, and wheel chairs).

Findings

Seven hundred and eighty participants responded. Fifty-eight percent of the participants underwent tracheostomy. Approximately, 80% of the patients experienced stress or trouble during communication. For all nine supports, > 60% participants expressed expectations regarding BMIs. More than 98% of participants who underwent tracheostomy expected support with conversation and emergency alarms. Participants who did not undergo tracheostomy exhibited significantly greater expectations than participants with tracheostomy did regarding all five movement supports. Seventy-seven percent of participants were interested in BMIs. Participants aged < 60 years had greater interest in both BMIs.

Interpretation

This is the first large-scale survey to reveal the present status of patients with ALS and probe their interests and expectations regarding BMIs. Communication and emergency alarms should be supported by BMIs initially. BMIs should provide wide-ranging and high-performance support that can easily be used by severely disabled elderly patients with ALS.
Appendix
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Literature
1.
Haverkamp LJ, Appel V, Appel SH (1995) Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 118:707–719CrossRef
2.
Bauer G, Gerstenbrand F, Rumpl E (1979) Varieties of the locked-in syndrome. J Neurol 221:77–91CrossRef
3.
4.
Murguialday AR, Hill J, Bensch M et al (2011) Transition from the locked in to the completely locked-in state: a physiological analysis. Clin Neurophysiol 122:925–933CrossRef
5.
McKelvey M, Evans DL, Kawai N, Beukelman D (2012) Communication styles of persons with ALS as recounted by surviving partners. Augment Altern Commun 28:232–242CrossRef
6.
Sellers EW, Vaughan TM, Wolpaw JR (2010) A brain-computer interface for long-term independent home use. Amyotroph Lateral Scler 11:449–455CrossRef
7.
Hochberg LR, Bacher D, Jarosiewicz B et al (2012) Reach and grasp by people with tetraplegia using a neurally controlled robotic arm. Nature 485:372–375CrossRef
8.
Collinger JL, Wodlinger B, Downey JE et al (2013) High-performance neuroprosthetic control by an individual with tetraplegia. Lancet 381:557–564CrossRef
9.
Vansteensel MJ, Pels EGM, Bleichner MG et al (2016) Fully implanted brain-computer interface in a locked-in patient with ALS. N Engl J Med 375:2060–2066CrossRef
10.
De Massari D, Ruf CA, Furdea A et al (2013) Brain communication in the locked-in state. Brain 136:1989–2000CrossRef
11.
ALS Questionnaire Report (1993) JALSA 29:27–41
12.
Furukawa Y, Komai K, Ishida C et al (2012) Cause of death in Japanese patients with amyotrophic lateral sclerosis on tracheostomy-positive pressure ventilation. Eur Neurol 68:261–263CrossRef
13.
Huggins JE, Wren PA, Gruis KL (2011) What would brain-computer interface users want? Opinions and priorities of potential users with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 12:318–324CrossRef
14.
Lahr J, Schwartz C, Heimbach B et al (2015) Invasive brain-machine interfaces: a survey of paralyzed patients’ attitudes, knowledge and methods of information retrieval. J Neural Eng 12:043001CrossRef
15.
Kageyama Y, Hirata M, Yanagisawa T et al (2014) Severely affected ALS patients have broad and high expectations for brain-machine interfaces. Amyotroph Lateral Scler Frontotemporal Degener 15:513–519CrossRef
16.
Cedarbaum JM, Stambler N (1997) Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials. J Neurol Sci 152(suppl 1):S1–9CrossRef
17.
Cedarbaum JM, Stambler N, Malta E et al (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169:13–21CrossRef
18.
Bradley WG, Anderson F, Bromberg M et al (2001) Current management of ALS: comparison of the ALS CARE database and the AAN practice parameter. The American Academy of Neurology. Neurology 57:500–504CrossRef
19.
Gil J, Funalot B, Verschueren A et al (2008) Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study. Eur J Neurol 15:1245–1251CrossRef
20.
Ritsma BR, Berger MJ, Charland DA et al (2010) NIPPV: prevalence, approach and barriers to use at Canadian ALS centers. Can J Neurol Sci 37:54–60CrossRef
21.
Beukelman D, Fager S, Nordness A (2011) Communication support for people with ALS. Neurol Res Int 2011:714693CrossRef
22.
Mitsumoto H, Del Bene M (2000) Improving the quality of life for people with ALS: the challenge ahead. Amyotroph Lateral Scler Other Motor Neuron Disord 1:329–336CrossRef
23.
Rousseau MC, Pietra S, Nadji M, de Villemeur Billette T (2013) Evaluation of quality of life in complete locked-in syndrome patients. J Palliat Med 16:1455–1458CrossRef
24.
Silvoni S, Volpato C, Cavinato M et al (2009) P300-based brain-computer interface communication: evaluation and follow-up in amyotrophic lateral sclerosis. Front Neurosci 3:60PubMedPubMedCentral
25.
Silvoni S, Cavinato M, Volpato C, Ruf CA, Birbaumer N, Piccione F (2013) Amyotrophic lateral sclerosis progression and stability of brain-computer interface communication. Amyotroph Lateral Scler Frontotemporal Degener 14:390–396CrossRef
26.
Borasio GD, Gelinas DF, Yanagisawa N (1998) Mechanical ventilation in amyotrophic lateral sclerosis: a cross-cultural perspective. J Neurol 245(suppl 2):S7–12CrossRef
27.
Ganzini L, Block S (2002) Physician-assisted death–a last resort? N Engl J Med 346:1663–1665CrossRef
28.
Lemoignan J, Ells C (2010) Amyotrophic lateral sclerosis and assisted ventilation: how patients decide. Palliat Support Care 8:207–213CrossRef
29.
Stutzki R, Weber M, Reiter-Theil S, Simmen U, Borasio GD, Jox RJ (2014) Attitudes towards hastened death in ALS: a prospective study of patients and family caregivers. Amyotroph Lateral Scler Frontotemporal Degener 15:68–76CrossRef
30.
Tsai CP, Chang BH, Lee CT (2013) Underlying cause and place of death among patients with amyotrophic lateral sclerosis in Taiwan: a population-based study, 2003–2008. J Epidemiol 23:424–428CrossRef
31.
Rousseau MC, Pietra S, Blaya J, Catala A (2011) Quality of life of ALS and LIS patients with and without invasive mechanical ventilation. J Neurol 258:1801–1804CrossRef
32.
Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M (1993) Home ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family, and physician attitudes. Neurology 43:438–443CrossRef
33.
Lule D, Zickler C, Hacker S et al (2009) Life can be worth living in locked-in syndrome. Prog Brain Res 177:339–351CrossRef
34.
Bruno MA, Bernheim JL, Ledoux D, Pellas F, Demertzi A, Laureys S (2011) A survey on self-assessed well-being in a cohort of chronic locked-in syndrome patients: happy majority, miserable minority. BMJ open 1:e000039CrossRef
35.
Cupp J, Simmons Z, Berg A, Felgoise SH, Walsh SM, Stephens HE (2011) Psychological health in patients with ALS is maintained as physical function declines. Amyotroph Lateral Scler 12:290–296CrossRef
36.
Hirose T, Kimura F, Tani H et al (2018) Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis. Muscle Nerve 58:770–776CrossRef
37.
Watanabe Y, Raaphorst J, Izumi Y et al (2020) Cognitive and behavioral status in Japanese ALS patients: a multicenter study. J Neurol 267:1321–1330CrossRef
Metadata
Title
Nationwide survey of 780 Japanese patients with amyotrophic lateral sclerosis: their status and expectations from brain–machine interfaces
Authors
Yu Kageyama
Xin He
Toshio Shimokawa
Jinichi Sawada
Takufumi Yanagisawa
Morris Shayne
Osamu Sakura
Haruhiko Kishima
Hideki Mochizuki
Toshiki Yoshimine
Masayuki Hirata
Publication date
01-10-2020
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 10/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-020-09903-3

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