Patients
We included all patients treated for ALS in the 1.7 million people Hospital District of Helsinki and Uusimaa (HUS) between 2010 and 2014. ALS diagnosis was confirmed by a senior neurologist (HL), using the El Escorial revised version.11 Among 388 patients for whom data were available, 9 (2.3%) patients were lost to follow-up and 52 (13.4%) had been erroneously diagnosed with ALS. Thus, our cohort included a total of 327 patients.
Hospital patient records were reviewed for age at which the first symptoms of motor dysfunction appeared, gender, time from the presentation of first symptoms until death, and the incidence of tracheostomy and PEG. In buALS, we also reported the date of the first visit to a healthcare provider, to any medical doctor, to specialized healthcare, and to a neurologist. The presenting symptoms (dysarthria, dysphagia, breathing symptoms) and findings during clinical and endoscopic examination (presence of vocal nasality, hoarseness, dysphagia, and/or fasciculations of the tongue and laryngeal findings assessed by either ORL, phoniatrician, or SLP) were reported. The first specialty a patient was referred to (neurology, otolaryngology, phoniatrics, or internal medicine), referrals to these specialties later during follow-up, and the date of onset of dysphagia and breathing symptoms were also reported. Furthermore, we collected the incidence of instrumental swallowing tests, FEES, or VFSS, as well as their indications, findings, and conclusions.