ALSの診断と告知，そして治療—臨床神経内科医としての40年の経験からの個人的な展望

Diagnosis, Notification, and Managements of ALS: A Personal Perspective from 40 years of Experience as a Clinical Neurologist

筆者の神経内科医としての40年に及ぶALS患者の診療の経験を叙述的にまとめた。前半の20年の大学とその関連病院の患者から5人を選び，後半20年の現在の病院での経験から，赴任以前から31年間闘病を続けた患者と私自身が初診した24例における問題点をまとめた。診断では四肢発症例における下垂足についてと球麻痺発症例における嗄声について述べ，さらに家族，告知，療養生活について問題点を検討した。

Abstract

This narrative summary presents the author's 40-year experience as a clinical neurologist who treated patients with amyotrophic lateral sclerosis (ALS). Five representative cases from the author's first 20 years at Chiba University Hospital and its affiliated hospitals were selected, including a patient of respiratory-onset who was ignorantly extubated by a female relative for patient's distress to the intratracheal tube. Based on the latter 20 years of experience at the author's current hospital, the author first describes a famous patient with ALS who was being treated at this medical center before the author was assigned to this hospital and fought against ALS for 31 years before eventually succumbing to total locked-in syndrome. Thereafter, the author has summarized the ages, sex, phenotypes, comorbidities, responses to the available treatment options, and total number of years that have elapsed for the 24 patients that the author initially examined in the outpatient clinic. In terms of diagnostic delay, the author describes “foot drop” in patients who developed lower limb symptoms, and hoarseness in those who developed bulbar palsy. Furthermore, the author discusses issues regarding family caregiving capacity, patient's and families' understanding of notification, and medical management (i.e., medications, rehabilitation for ADL, nutrition and respiration, complications of frontotemporal dementia, and medical cooperation with other clinics and hospitals).