title: [The spread pattern and survival in sporadic ALS].
abstract: A misfolding pathology has been suggested to spread from onset site to neighbouring areas in a prion-like manner. We examined the pattern of spread and direction of clinical lower motor neuron involvement over time until the appearance of respiratory symptom. A total of 150 patients with sporadic ALS underwent follow-up until respiratory symptoms. Symptom appearances were determined using ALSFRS-R. The interval from onset to involvement of the second region correlated significantly with survival, independent of particular combinations. No patient with a rapid spread pattern (two regions within 3 months from onset) survived >5 years. Time from onset to spread to the second site, suggesting propagation speed, is a strong predictor for survival. In terms of cumulative occurrence, symptoms spread longitudinally to adjacent regions. In most of ALS patients, the spread appears to have a contiguous pattern rather than a random pattern of progression, although the spread to non-contiguous regions was observed in 13%. This finding supports the notion that the ALS pathology gradually spreads to adjacent regions in a longitudinal manner. Although precise mechanism of symptom spread in ALS is not understood, knowledge about the pattern of onset and the anatomical direction of spread may provide valuable prognostic insights.
authors.affiliation: Division of Neurology, The First Department of Internal Medicine, Osaka Medical College.
authors.forename: Fumiharu
authors.initials: F
authors.lastname: Kimura
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality* / Q000473:pathology*; D006801:Humans