Related Experiment Video
Updated: Jun 15, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
Primary lateral sclerosis may occur within familial amyotrophic lateral sclerosis pedigrees
Julien Praline1, Anne-Marie Guennoc, Patrick Vourc'h
1ALS Centre, Tours, France. julien.praline@med.univ-tours.fr <julien.praline@med.univ-tours.fr>
Abstract:
Primary lateral sclerosis (PLS) is considered to be a specific sporadic motor neuron disorder, but some reports have shown familial history of motor neuron disorders that may comprise PLS cases. Here we report a novel pedigree highlighting an intrafamilial occurrence of PLS and amyotrophic lateral sclerosis (ALS) cases. These observations strengthen the hypothesis that PLS may represent an ALS phenotype with a long evolution and strongly suggest the involvement of common genetic factors that can lead to upper and lower motor neuron death.
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