Abstract
Purpose
To profile the detailed clinical features of sporadic amyotrophic lateral sclerosis
(ALS) on large-scale samples in Japan.
Methods
We assessed the clinical features of sporadic ALS patients in Japan, based on the
nationwide registration system of the Ministry of Health, Labor and Welfare of Japan.
We described 3428 new cases registered cases between 2003 and 2006 to analyze initial
symptoms and related clinical features, 4202 cases registered in the single year of
2005 to describe the cross-sectional overview of the ALS patients, and a total of
2128 cases with tracheostomy positive pressure ventilation (TPPV) from all of the
registration data from 2003 to 2006 to describe the features of ALS patients with
TPPV.
Results
The patients with an older age at onset progressed more rapidly to the TPPV stage
than those with a younger age at onset. The subpopulation of patients with long-standing
TPPV showed ophthalmoplegia, while its appearance rate was less in the patients with
an older age at onset than in those with a younger age at onset. Furthermore, age
at onset strongly influenced the frequency of initial symptoms: dysarthria, dysphagia,
neck weakness and respiratory disturbance were more frequent in patients with an older
age at onset, while upper or lower limb weakness was observed more frequently in patients
with a younger age at onset. In addition, those initial symptoms were still the most
prominent at the follow-up stage, suggesting that the initial symptoms determine the
major clinical features even in advanced illness.
Conclusions
Our present study demonstrated that symptomatic features of ALS are strongly influenced
by the age at onset by the large scale of samples.
Abbreviations:
ALS (amyotrophic lateral sclerosis), TPPV (tracheostomy positive pressure ventilation)Keywords
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Article info
Publication history
Accepted:
September 16,
2008
Received in revised form:
July 19,
2008
Received:
March 17,
2008
Identification
Copyright
© 2008 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
