Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis.

Autor: Gargiulo-Monachelli GM; Department of Neurology, Ramos Mejia Hospital. School of Medicine, Buenos Aires University, Buenos Aires, Argentina. gargiulo.gisella@ibyme.conicet.gov.ar, Janota F, Bettini M, Shoesmith CL, Strong MJ, Sica RE
Jazyk: angličtina
Zdroj: European journal of neurology [Eur J Neurol] 2012 Jun; Vol. 19 (6), pp. 834-41. Date of Electronic Publication: 2012 Jan 17.
DOI: 10.1111/j.1468-1331.2011.03616.x
Abstrakt: Background and Purpose: Sporadic amyotrophic lateral sclerosis (sALS) is a disease with a focal clinical onset and contiguous spread. We examined patterns of disease spread following symptoms onset in sALS and whether the pattern of spread predicted survival.
Methods: Review of medical records (2003-2009) at London Ontario and Buenos Aires clinic cohorts retrieved 318 patients with sporadic sALS. According to patient self-report, we determined eight spread patterns: rostro-caudal, caudo-rostral, crossed, circular, superior interposed, middle interposed, inferior interposed and isolated. The variables studied were as follows: age, gender, sALS phenotypes, time from onset to diagnosis and time and direction of the spreading to the first region. Survival from symptoms onset was analysed by Kaplan-Meier, Tarone-Ware and Cox proportional hazards methods.
Results:   The direction of first spread was horizontal in 33%, rostral to caudal in 32% and caudal to rostral in 21%, whereas spread to remote regions was observed in 14% of patients. Survival curves and 3- and 5-year survival rates favoured patients with an isolated and caudo-rostral pattern of spread compared to patients progressing to distant regions without involvement in the intervening region, or 'superior and inferior interposed patterns' (Tarone-Ware P = 0.001, χ(2) = 0.002 and χ(2) = 0.006, respectively). Factors affecting survival were gender, time to diagnosis, flail arm phenotype and age at diagnosis.
Conclusions:  We have provided evidence that not all spread in ALS is contiguous and that the nature of symptom progression influences survival. Patients with sALS with 'interposed patterns' had a worse prognosis, whereas patients with caudo-rostral pattern fared better than the rest.
(© 2012 The Author(s). European Journal of Neurology © 2012 EFNS.)
Databáze: MEDLINE
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