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.