Late Progressive Quadriparesis Due to Odontoid Agenesis

Introduction  Congenital absence of the odontoid process of the axis was first described by Robert¹ in 1933. Since that time, an additional 27 cases^2-20 have been reported. Most case reports have dealt with the orthopedic and radiological aspects of the condition. However, neurological complications are relatively frequent. Progressive cervical myelopathy occurred spontaneously in six reported cases, and post-traumatic myelopathy or radicular involvement occurred in an additional five cases closely following trauma.As the cases in which progressive cervical myelopathy occurs can mimic other types of neurological disease, and as little is known concerning the best type of management of these patients, it seems appropriate to report our recent experience with two cases of congenital absence of the odontoid process presenting as progressive spastic quadriparesis of obscure etiology.

Report of Cases  Case 1.—The patient was first admitted to the St. Albans Naval Hospital Jan 9, 1957, at the age of 35