title: Seven amyotrophic lateral sclerosis patients diagnosed only after development of respiratory failure.
abstract: Amyotrophic lateral sclerosis (ALS) is a progressive, neurodegenerative disorder that causes muscle weakness, disability, respiratory failure, and eventually death. However, some ALS patients are diagnosed only after development of respiratory failure. To study the reason for delayed diagnosis of ALS, we reviewed cases of ALS patients with respiratory failure. We retrospectively reviewed all 200 patients diagnosed with sporadic ALS in our hospital from January 2001 to April 2011. Among them, we focused on seven patients who were diagnosed only after developing respiratory failure. We reviewed their clinical characteristics and demographics. The mean time from onset to a correct diagnosis was 15.6±standard deviation of 8.0 months. Two patients had already been intubated at a previous hospital because they presented with severe respiratory failure and required emergency intubation. One patient was intubated upon arrival to our hospital. We identified three reasons for the delay in diagnosis: delayed referral to a neurologist (four patients); a shortage of neurologists in rural areas (three patients); and an atypical clinical course with respiratory failure as the initial symptom (two patients). Three patients had undergone emergency intubation without giving informed consent. To provide an informed choice and to avoid unwanted intubation for ALS patients, we suggest extending neurological knowledge of ALS to general practitioners.
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Kota
authors.initials: K
authors.lastname: Sato
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Morimoto
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Deguchi
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikeda
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Tohru
authors.initials: T
authors.lastname: Matsuura
authors.affiliation: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan. Electronic address: kosatou@cc.okayama-u.ac.jp.
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D057210:Delayed Diagnosis; D018450:Disease Progression; D005260:Female; D006801:Humans; D007440:Intubation; D008297:Male; D008875:Middle Aged; D012017:Referral and Consultation; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000628:therapy; D012189:Retrospective Studies; D012424:Rural Population; D013997:Time Factors
keywords: ALS; Emergency medicine; Intubation; Neurodegenerative disorder; Respiratory failure