Primary lateral sclerosis: further clarification.

J Neurol Sci

Fédération de Neurologie Mazarin Professeurs Delattre et Meininger, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75013, Paris, France.

Published: April 2001

Primary lateral sclerosis (PLS) has been defined as a rare. Non-hereditary disease characterized by progressive spinobulbar spasticity, related to the exclusive involvement of precentral pyramidal neurons, with secondary pyramidal tract degeneration and a preservation of anterior horn motor neurons, the latter allowing PLS to be distinguish from amyotrophic lateral sclerosis (ALS). However, a clear distinction between the two diseases remains a subject of debate. With this in mind, we assessed patients with meeting the previously published criteria for PLS in a prospective, longitudinal study. At regular intervals, we analyzed various clinical and electrophysiological parameters in nine patients with a diagnosis of PLS. We made a deltoid muscle biopsy and PET study.Our results provide evidence that degeneration in PLS is not restricted to the upper motor neurons but also affects the lower motor neurons. The distinction between ALS and PLS is related to the degree and stability of lower motor neuron involvement. In view of the similarities with ALS, we consider that PLS may represent a slowly progressive syndrome closely related to this disease.

Download full-text PDF

Source
http://dx.doi.org/10.1016/s0022-510x(01)00469-5DOI Listing

Publication Analysis

Top Keywords

lateral sclerosis
12
motor neurons
12
primary lateral
8
lower motor
8
pls
7
sclerosis clarification
4
clarification primary
4
sclerosis pls
4
pls defined
4
defined rare
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!