[Progressive bulbar paralysis in childhood with pyramidal signs (author's transl)]

We report on a 8 year-old boy who presented with progressive bulbar paralysis. Remarkable were the presence of pyramidal signs, the visual disturbances, the peculiar gait and the intermittent progression of the disease. Our case supports the idea that spinal muscular atrophies form a group of diseases with variable expression. We can classify our patient between the juvenile form and the adult form of spinal muscular atrophy with progressive bulbar palsy. We could follow this boy for almost 4 years. He died at the age of twelve years. Post mortem examination was not permitted.