Life-Threatening Complication of Spastic Cerebral Palsy: Atlantoaxial Instability

Case Study: The development of atlantoaxial instability with spinal cord injury following a cervical trauma is more likely in children because of functional and anatomical reasons. In patients with spastic cerebral palsy, many factors additionally increase this risk. However, there are only a few cases reported in this group of patients.

An 8-year-old boy with spastic cerebral palsy, mental retardation, and epilepsy was referred to us because of tilting his head and neck with painful limitation of mobility of the cervical spine over the last 7 months. We saw a decrease in muscle tone of the arms. The MRI of the cervical spine demonstrated an older fracture of dens axis with dislocation of dens and axis together with a compression and edema of the cervical cord. A curative neurosurgical therapy was not possible because of pronounced osteoporosis.

Our second patient is a 6-year-old boy with spastic cerebral palsy and epilepsy who was admitted for injections of Botox. Just before sedation, we saw decreased oxygen saturation and mild hypercapnia. The following day he developed acute respiratory failure with the need of intensive care. The MRI of the cervical spine showed a dislocation of the dens and narrowing of the spinal canal with cystic defect of the spinal cord as a sign of irreversible damage. Here again, there was no surgical option and the patient received palliative care until his death 5 weeks later.

With our poster, we aim to draw the attention on the increased risk of spinal cord injury by atlantoaxial instability in patients with spastic cerebral palsy. Maybe, this life-threatening complication is frequently overlooked. The neurological signs are subtle and have not led to medical consultation by the parents. A diagnosis delay restricts therapeutic options and deteriorates the prognosis.

Keywords: spastic cerebral palsy, atlantoaxial instability.