Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformation

This is a case report of a male patient who presented with orofacial pain f or a year as the only manifestation of syringobulbia-syringomyelia associat ed with Arnold-Chiari malformation. This article places emphasis on the cli nical presentation and possible differential diagnoses. The pain was contin uous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracran ial pressure. Paroxysmal pain crises developed over this background of cont inuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with a mplification of the foramen magnum.