This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (11)  • Contact me when this article is cited  Related Content  • Related letters  • Similar articles in this journal  Topic Collections  • Amyotrophic Lateral Sclerosis  • Motor Neuron Disease  • Neuromuscular diseases  • Neurology, Other  • Diagnosis  • Alert me on articles by topic  Social Bookmarking   What's this?

Examination of Symptoms and Signs at Disease Onset and During Follow-up

Maria Carmela Tartaglia, MD; Ann Rowe, RN; Karen Findlater, BScPT; J. B. Orange, PhD; Gloria Grace, PhD; Michael J. Strong, MD

Arch Neurol. 2007;64(2):232-236.

Background  Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum.

Objective  To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS.

Design  Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up.

Setting  Tertiary referral center.

Patients  Six hundred sixty-one patients with ALS and 43 patients with PLS were included in the study.

Results  At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS (observed in 47% and 4% of patients, respectively; P<.001). During follow-up, limb wasting was rare in patients with PLS (2%, compared with 100% in patients with ALS; P<.001). Disease duration was significantly longer in patients with PLS compared with patients with ALS (mean ± SD, 11.2 ± 6.1 vs 3.8 ± 4.2 years, respectively; P<.001). During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS (only 33% vs 89%, respectively; P<.001).

Conclusion  Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS.

Author Affiliations: Clinical Neurological Sciences, London Health Sciences Centre (Drs Tartaglia, Grace, and Strong, and Mss Rowe and Findlater), and School of Communication Sciences and Disorders (Dr Orange), University of Western Ontario, London.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED LETTERS

Clinical Differentiation Between Primary Lateral Sclerosis and Upper Motor Neuron Predominant Amyotrophic Lateral Sclerosis
Marc Gotkine and Zohar Argov
Arch Neurol. 2007;64(10):1545.
EXTRACT | FULL TEXT  

Clinical Differentiation Between Primary Lateral Sclerosis and Upper Motor Neuron Predominant Amyotrophic Lateral Sclerosis—Reply
Maria Carmela Tartaglia and Michael J. Strong
Arch Neurol. 2007;64(10):1545.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration
Ravits and La Spada
Neurology 2009;73:805-811.
ABSTRACT | FULL TEXT  

Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS
Gordon et al.
Neurology 2009;72:1948-1952.
ABSTRACT | FULL TEXT  

Brain atrophy in primary lateral sclerosis
Tartaglia et al.
Neurology 2009;72:1236-1241.
ABSTRACT | FULL TEXT  

Differentiation of Hereditary Spastic Paraparesis From Primary Lateral Sclerosis in Sporadic Adult-Onset Upper Motor Neuron Syndromes
Brugman et al.
Arch Neurol 2009;66:509-514.
ABSTRACT | FULL TEXT  

Clinical Differentiation Between Primary Lateral Sclerosis and Upper Motor Neuron Predominant Amyotrophic Lateral Sclerosis
Gotkine and Argov
Arch Neurol 2007;64:1545-1545.
FULL TEXT