Acquired atlantoaxial instability in children with spastic cerebral palsy

The development of nontraumatic atlantoaxial instability in children with spastic cerebral palsy has not been reported. The authors present three patients with severe spastic quadriplegia who developed C1-C2 instability and cervical myelopathy at mean age 12.6 years. These patients demonstrated a similar clinical picture with symptoms attributed to cervical myelopathy in varied severity including apneic episodes, opisthotonus, alteration in muscle tone, torticollis, respiratory problems, hyperreflexia, and bradycardia. Patient 1 was scheduled for surgery but died due to an apneic episode. Patient 2 refused surgery and has been followed for 3 years while his neurologic condition remains unchanged. Patient 3 underwent occipitocervical decompression and fusion, recovered neurologically, and resumed his previous functional skills. Patients demonstrating considerable functional deterioration or insidious change in their established neurologic status should undergo detailed screening to rule out developing upper cervical instability. Early surgical intervention consisting of spinal decompression and fusion may prevent the development of myelopathy.