Odontoid compression of the brain stem in a patient with rheumatoid arthritis

Case report

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✓ Cervical spine involvement by rheumatoid arthritis is common; brain-stem compression secondary to vertical subluxation of the odontoid in patients with rheumatoid arthritis is rare. Vertical subluxation results from 1) destruction of the transverse atlantal, apical, and alar ligaments of the atlas and odontoid, and 2) bone resorption in the occipital condyles, lateral masses of the atlas, and basilar processes of the skull. Neurological symptoms result from direct compression of the brain stem or from ischemia secondary to compression of vertebral arteries, anterior spinal arteries, or small perforating arteries of the brain stem and spinal cord. A case is reported in which a slowly progressive neurological deficit developed in a woman with rheumatoid arthritis following a fall from a stretcher. Neurological symptoms represented direct compression of the medulla by the dens, a mechanism confirmed at operation and autopsy.

Recognition of progressive neurological deficit is often difficult in patients with rheumatoid arthritis because of their inactivity and their atrophic and immobile joints, but is essential if appropriate decompressive or stabilizing procedures are to be done. In patients with vertical subluxation of the dens, the transoral approach with removal of the odontoid is recommended. Decompression should be extensive, including the fibrous capsule around the odontoid and overlying synovial tissue as well as the odontoid itself.

Article Information

Address reprint requests to: Harold P. Smith, M.D., Section on Neurosurgery, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103.

© AANS, except where prohibited by US copyright law.

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Figures

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    Anteroposterior (left) and lateral (right) tomograms of the foramen magnum and posterior fossa demonstrating projection of the odontoid process (arrows) into the posterior fossa and the region of the medulla. Destruction of the posterior arch of C-1 can be seen on the lateral tomogram.

  • View in gallery

    The ventral surface of the medulla oblongata showing the deep impression made by the odontoid process (arrow) to the left of midline. Postmortem clots are present in the vertebral arteries.

  • View in gallery

    Microscopic sections of the medulla. Luxol fast blue-hematoxylin, × 4.5. Left: Section at the level of the impression showing flattening near the pyramid (arrow). There is decreased density of myelin beneath the impression in comparison to the opposite pyramid (crossed arrow). The posterior columns are demyelinated. Right: Section at a higher level shows no vascular lesions.

  • View in gallery

    Autopsy specimen. × 2.5. Left: The fibrous sheath around the odontoid process is shown. Center: Longitudinal section showing the thickness of the wall and the cavity resulting from operative removal of bone. Right: Microscopic section showing a thick fibrous sheath and a residual spicule of bone at the tip (arrow).

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