Abstract
Objectives
The clinical spectrum of amyotrophic lateral sclerosis (ALS) is characterized by a
considerable variation. Different phenotypes have been described by previous studies.
We assessed clinical variability and prognostic relevance of these phenotypes in a
prospective, population-based cohort of ALS patients in Rhineland-Palatinate, Germany.
Methods
Incident ALS cases, diagnosed between October 2009 and September 2012, were prospectively
enrolled and classified according to established ALS phenotype classification (bulbar,
classic, flail arm, flail leg, pyramidal, respiratory). Survival probability was described
using Kaplan–Meier method. Moreover, the influence of an additional frontotemporal
dementia (FTD) was analysed.
Results
Phenotypes of all 200 patients were determined. Bulbar and classic phenotypes accounted
for 75% of all cases. Deterioration of functional impairment during disease progression
was lowest in flail leg and pyramidal variants, and most pronounced in bulbar and
classic phenotypes. A poor survival prognosis was observed for bulbar, classic or
respiratory phenotypes. Patients with an additional FTD showed an even worse outcome.
Conclusions
Results suggest that ALS is a heterogeneous disease, as ALS phenotypes differ in disease
progression and survival time. Patients classified as suffering from bulbar, classic
and respiratory ALS, as well as those with an additional FTD, show a marked reduction
of survival time.
Keywords
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Article info
Publication history
Published online: July 19, 2014
Accepted:
July 14,
2014
Received:
June 11,
2014
Identification
Copyright
© 2014 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
