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This Article Figures Only Full Text Full Text (PDF) All Versions of this Article: 01.wnl.0000312378.94737.45v1 01.wnl.0000312378.94737.45v2 71/12/876    most recent Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Google Scholar Articles by Sabatelli, M. Articles by Tonali, P. PubMed PubMed Citation Articles by Sabatelli, M. Articles by Tonali, P. Related Collections Prognosis Amyotrophic lateral sclerosis Natural history studies (prognosis)

Natural history of young-adult amyotrophic lateral sclerosis

From Istituto di Neurologia (M.S., F.M., A.C., M.L., M.L.M., P.T.); Istituto di Genetica Medica (M.Z., I.M.), Università Cattolica del Sacro Cuore; and UILDM sez. Laziale (G.L.), Rome, Italy; and Fondazione Don Gnocchi (P.T.), Milan, Italy.

Address correspondence and reprint requests to Dr. Mario Sabatelli, Istituto di Neurologia. Pol. "A: Gemelli", Largo Gemelli, 8, 00168 Rome, Italy msabatelli{at}rm.unicatt.it

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.

Abbreviations: ALS = amyotrophic lateral sclerosis; p-UMN = predominant upper motor neuron.

Editorial, page 872.

e-Pub ahead of print on July 2, 2008, at www.neurology.org.

Supported by Fondazione Roma and by the Association for ALS research I.CO.M.M., Onlus, Rome, Italy.

Disclosure: The authors report no disclosures.

Received November 7, 2007. Accepted in final form February 6, 2008.

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