Chiari Malformation and Syringomyelia Associated with Hirayama Disease

To the best of our knowledge, the presence of Chiari formation and Hirayama disease in the same patient has not been previously reported. On the basis of the presented case, we have attempted to identify the common pathogenesis of both of these clinical entities.

We have reported a case of a 23-year-old male patient who had presented with complaints of pain in the nape of neck and shoulders of 9 months' duration, weakness and stiffness in all 4 limbs, and wasting and weakness of muscles of both hands of 6 months' duration. The investigations showed Chiari formation and syringomyelia. Additional investigations depicted the characteristic radiological features associated with Hirayama disease. The patient was treated by atlantoaxial and multisegmental subaxial cervical spinal fixation.

Spinal instability seems to be the nodal point of the pathogenesis of both Chiari formation and Hirayama disease.