Abstract
Background: Primary lateral sclerosis (PLS) is a disorder characterized by spinal and bulbar spasticity due to degeneration of the upper motor neuron. We have recently identified a French-Canadian family where several members present clinical features that mimic closely PLS. Objective: Present detailed phenotypic and genotypic data of a French-Canadian family with a new autosomal dominant disease characterized by progressive upper motor neuron involvement. Methods: A total of 8 affected and 10 unaffected family members underwent a thorough neurological examination and were sampled.
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