Regional Spread Pattern Affects Prognosis in Patients with Amyotrophic Lateral Sclerosis (ALS)

GARGIULO MONACHELLI GM; JANOTA F; BETTINI M; RODRIGUEZ G; GONZALEZ DENISELLE M.C; SHOESMITH C; STRONG MJ; SICA REP

Congreso; 63rd Annual Meeting of the American Academy of Neurology.; 2011

ALS comprises focal onset and contiguous spread. We sought to describe spread patterns (SP) from disease onset to diagnosis, compare them with phenotypes and establish their contribution to prognosis. Patterns of spread were retrospectively determined in 177 sporadic ALS patients at the Ramos Mejia hospital1 (2003-2009). We established 8 SP: rostro-caudal (bulbar to cervical to lumbar), caudo-rostral, crossed, circular, superior interposed (bulbar to lumbar to cervical), middle interposed (cervical to bulbar to lumbar, or cervical to lumbar to bulbar) inferior interposed (lumbar to bulbar to cervical) and isolated patterns. Variables studied were age, gender, classic phenotypes, time onset to diagnosis (TOD), first region spread (FRS) and first region spread time (FRST). Survival was analyzed by Kaplan-Meier log-rank and Cox proportional hazards. Among groups, older age of onset was seen in superior interposed with a median of 65 years. Gender was predominantly masculine, except in rostro-caudal patients. TOD and FRST were not significantly different between groups. FRS was more frequent and slower to contiguous areas than to non-contiguous. Survival curves showed worse prognosis for both superior and inferior interposed patterns with a median survival of 23 and 27.5 months. Factors independently affecting survival were TOD and FRST; while age, riluzole use and phenotype exerted no influence. In this cohort, SP influenced survival. Symptom spread which skipped regions (interposed spread) occurred infrequently but was associated with a worse prognosis.  Our findings underline the importance of considering spread patterns when assessing ALS subtypes and may assist in unraveling pathogenic mechanisms of disease.