Attacks of unconsciousness in association with fused cervical vertebrae.

There are more than 140 references in the Quarterly Cumulative Index Medicus to papers on fusion of cervical vertebrae, including the KlippelFeil syndrome. A particularly useful review of the Klippel-Feil syndrome is that by Erskine (1946). He wrote that 'Essential features of the cervical deformity are synostosis of two or more cervical vertebrae and flattening and widening of the vertebrae bodies. A numerical reduction of the vertebrae is an incidental rather than an essential part of the disease, as is spina bifida. There is evidence that the anomaly has a genetic basis. . Spina bifida is frequently found. It is of a false type, as some of the laminae curve down to meet in the midline in a fused mass in the lower cervical region, so that a triangular opening is found, with the apex directed downward and the base toward the occiput. The triangular deficiency in the arches is covered by a layer of fibrous tissue, and occasionally it extends to the upper thoracic region.' As a result of the fusion of vertebrae there is limitation of mobility together with shortening of the neck. It may be associated with other abnormalities, such as platybasia, narrowing or asymmetry of the foramen magnum, Sprengel's deformity, scoliosis, torticollis, oxycephaly and cervical rib. Numerous neurological disturbances have been described in association with the Klippel-Feil syndrome. They include syringomyelia, weakness of the arms or legs, attacks of quadriplegia, abducens paralysis, deaf mutism, cholesteatoma of the cerebellum, nystagmus, facial weakness, sensorimotor changes in the arms, and bimanual synkinesia, or mirror movements, whereby every movement of one hand is imitated by the other hand, so that reciprocal movements like the climbing of a ladder are impossible. Below are accounts of two children seen on account of attacks of unconsciousness. One was obviously an example of the Klippel-Feil syndrome. The other showed no clinical evidence of an abnormality of the cervical spine, but because of the resemblance of his symptoms to those of the child with the Klippel-Feil syndrome a radiograph of the cervical spine was asked for, and an abnormality was found. I have been unable to find any reference in the literature to such a condition.