[The rigid spinal column syndrome. Aspects of hypotrophy and predominance of type I muscular fibers].

The case of a boy developing a "rigid spine syndrome" from 6 years of age is reported. Weakness and amyotrophy of shoulder girdle were noticed. The muscular biopsy showed a pattern of predominance and hypotrophy of type I fibers, as already reported in comparable cases. The pathophysiology of this exceptional syndrome is discussed.