Abstract
BACKGROUND: Amyotrophic lateral sclerosis (ALS) affects people of all ages, but
whether the wide range of age at onset is due to distinct diseases or merely
reflects phenotypic variability of the same disorder is still unknown. The
purpose of this study is to describe clinical and prognostic features of
young-adult ALS, with onset before age 40 years, and to compare them with
features of the common adult-onset type. METHODS: We analyzed clinical features
and long-term follow-up of 57 young-adult ALS patients, with disease onset
between 20 and 40 years, and compared them with 450 patients affected by
adult-onset ALS. RESULTS: We found that the majority of young-adult patients
showed a predominant upper motor neuron (p-UMN) ALS, characterized by marked
spastic paraparesis, with lower motor neuron signs confined to the upper limbs.
The proportion of patients with p-UMN ALS phenotype was 59.6% in the young-adult
patients and 17.4% in the adult-onset form (p < 0.0001). Young-adult ALS with
p-UMN phenotype had longer survival than did the classic phenotype: median
survival was 74 months (range 10-226, 95% CI 60.61-87.38) in the former and 56
months (range 6-106, 95% CI 48.65-63.34) in the latter (p = 0.03). In the
young-adult patients, a marked male excess was observed in the p-UMN ALS group
(5.8:1), whereas the ratio of men to women was 1.1:1 in the classic phenotype (p
= 0.01). CONCLUSIONS: Our findings show that young-adult amyotrophic lateral
sclerosis with the predominant upper motor neuron phenotype represents a
distinctive clinical variant characterized by a unique clinical pattern, longer
survival, and male prevalence.
Lingua originale | English |
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pagine (da-a) | 876-881 |
Numero di pagine | 6 |
Rivista | Neurology |
Stato di pubblicazione | Pubblicato - 2008 |
Keywords
- ALS