Diagnosis and management of anomalies of the craniovertebral junction.

The term craniovertebral junction refers to an area comprising the inferior portion of the occipital bone surrounding the foramen magnum and the first two cervical vertebrae. A variety of anomalies of bony, meningeal, and neural elements are known to occur in this region, producing a wide spectrum of clinical symptoms. These can range from dizziness, ataxia, and nuchal headaches to obvious quadriparesis and cranial nerve palsies. Often the symptoms are vague with an evolution marked by temporary exacerbations and remissions. The diagnosis can be difficult, and in our experience is best achieved by combining a neurotologic and neurologic evaluation. We discuss here the symptoms and diagnosis in ten patients with lesions at the craniovertebral junction. The surgical management of two cases of basilar invagination is described, and the controversies regarding the technique are discussed.