title: Factors affecting the diagnostic delay in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  Although amyotrophic lateral sclerosis (ALS) is a relentlessly progressive disorder, early diagnosis allows a prompt start with the specific drug riluzole and an accurate palliative care planning. ALS at onset may however mimic several disorders, some of them treatable (e.g., multifocal motor neuropathy) or epidemiologically more frequent (e.g., cervical myelopathy).
  
  
  OBJECTIVE
  To study the delay from onset to diagnosis in a cohort of ALS patients and to the variables that may affect it.
  
  
  METHODS
  We performed a retrospective analysis of the diagnostic delays in a cohort of 260 patients affected by ALS (M/F = 1.32) followed at our tertiary referral ALS Center between 2000 and 2007.
  
  
  RESULTS
  The median time from onset to diagnosis was 11 months (range: 6-21) for the whole ALS cohort, 10 months (range: 6-15) in bulbar-onset (n = 65) and 12 months (range: 7-23) in spinal-onset (n = 195) patients (p = 0.3). 31.1% of patients received other diagnoses before ALS and this led to a significant delay of the correct diagnosis in this group (other diagnoses before ALS, n = 81: median delay, 15 months [9.75-24.25] vs ALS, n = 179, median delay, 9 months [6-15.25], p < 0.001).
  
  
  CONCLUSIONS
  The diagnostic delay in ALS is about one year, besides the growing number of tertiary centres and the spread of information about the disease through media and internet. Cognitive errors based on an incorrect use of heuristics might represent an important contributing factor. Furthermore, the length of the differential diagnosis from other disorders and delays in referral to the neurologist seems to be positively associated with the delay in diagnosis.
authors.affiliation: ALS Clinical Research Center, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, via G La Loggia, 1 90129 Palermo, Italy.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Cellura
authors.forename: Rossella
authors.initials: R
authors.lastname: Spataro
authors.forename: Alfonsa Claudia
authors.initials: AC
authors.lastname: Taiello
authors.forename: Vincenzo
authors.initials: V
authors.lastname: La Bella
mesh_terms: D017668:Age of Onset; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis*; D015331:Cohort Studies; D057210:Delayed Diagnosis; D003951:Diagnostic Errors; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies