HISTORICAL VIGNETTE

Why Atlas, Why Not Heracles: Reections on the Rheumatoid Cervical Spine
Kari Laiho, MD, PhD, Markku Kauppi, MD, PhD, and Yrj T. Konttinen, MD, PhD
Rheumatoid involvement of the 24 joints of the cervical spine leads to prominent changes in the occipito-atlanto-axial area. Eight different subtypes of such changes are recognized, depicted, and dened. The frequencies of these 8 subtypes in rheumatoid arthritis and other arthritides are tabulated. A central role in these disorders is played by a small-size, heavy-duty tting piece or adapter, atlas, between the occiput and C2. The history of the fate of Atlas, who led the ght of godlike titans against the new gods of Olympos, is recapitulated. In particular, for a short moment Atlas was released from his heavy physical ordeal by another strongman, the heavy-weight wrestler of his times, Heracles. The reasons for the current nomenclature and answer to the question Why Atlas, why not Heracles are provided. Semin Arthritis Rheum 34:637-641 2004 Elsevier Inc. All rights reserved. KEYWORDS cervical spine, subluxation, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthropathy, atlas, Heracles, axis

he cervical spine contains 7 cervical vertebrae and 24 joints. Changes occurring in the occipito-atlanto-axial junction and cervical spine in rheumatoid arthritis and other rheumatic diseases are classied to the 8 following different categories (1,2): anterior atlanto-axial subluxation (aAAS), vertical atlantoaxial subluxation or atlanto-axial impaction (AAI), lateral atlanto-axial subluxation (lAAS), posterior atlanto-axial subluxation (pAAS), rotatory atlanto-axial subluxation, nonreducible rotational head tilt (NRRHT), subaxial subluxation (SAS), and apophyseal joint ankylosis. The rst 5 of the changes listed above affect the occipito-atlanto-axial junction. An illustration and denitions of these various subsets are provided in Fig. 1 and their prevalence according to subtype and rheumatic disease is provided in Table 1 (3-16).

Anatomy
If the joints at the interface to the occiput and to the thoracic spine are included, the cervical spine contains 24 joints. The anatomy of the cervical spine has been detailed elsewhere (17,18). There are 6 intervertebral joints (there is no such joint at the atlanto-occipital or atlanto-axial junction), 2 atlanto-occipital joints, 2 lateral atlanto-axial joints (19), 12 apophyseal or facet joints, 1 central atlanto-axial joint, and 1 joint between the dens and the transverse ligament (20). Inclusion of the joint between the dens and the transverse ligament as a joint can be disputed. In addition to these, the joints of Luschka are often mentioned. In the original description, they were reported to contain a joint cavity lined by hyaline articular cartilage and surrounded by a brous joint capsule. These bilateral joints are supposed to be located between the uncinate process of the cervical vertebra and the body of the overlying cervical vertebra. The uncinate process is of great signicance in the cervical spine because it acts as a barrier to the extrusion of disc material posterolaterally, preventing compression of the nerve roots (17). However, in his 191 autopsies, John H. Bland was not able to nd true joints of Luschka (18). The key vertebra of the cervical spine is the atlas (Fig. 2), which has the form of a ring. The body of the atlas does not exist 637

Rheumatism Foundation Hospital, Heinola, Finland; Department of Medicine/Invrtes Medicin, Helsinki University Central Hospital, Helsinki, Finland; and ORTON Orthopaedic Hospital of the Invalid Foundation, Helsinki, Finland. Supported by Academy of Finland, TEKES, Ministry of Education, Invalid Foundation, Juselius Foundation, Finska Lkaresllskapet, and Helsinki University Central Hospital evo-funds, TEKES, and Ministry of Education. There is no conict of interest due to commercial afliation, consultancies, stock or equity interests, or patent-licensing arrangements. Address reprint requests to Yrj T. Konttinen, Biomedicum, P.O. Box 700, FIN-00029 HUS, Finland. .E-mail: yrjo.konttinen@helsinki.

0049-0172/05/$-see frontmatter 2004 Elsevier Inc. All rights reserved. doi:10.1016/j.semarthrit.2004.07.012

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K. Laiho, M. Kauppi, and Y.T. Konttinen

Figure 1 Classication of rheumatoid cervical spine changes. In aAAS (anterior atlanto-axial subluxation 3 mm) the atlas moves abnormally forward due to laxity of the transverse ligament. AAI (atlanto-axial impaction) results from inammatory changes in lateral atlanto-axial joints. Erosions in the atlanto-odontoid joint or fracture of the odontoid are seen as pAAS (posterior atlanto-axial subluxation). Unilateral atlanto-axial joint involvement causes the atlas to move to the opposite side, a subluxation known as lAAS (lateral atlanto-axial subluxation 2 mm). Rotational atlanto-axial subluxation indicates a xed and nonreducible rotation of atlas with respect to axis. Lateral mass collapse connected with tilt to the same side is called NRRHT (nonreducible rotational head tilt). Subluxation of a vertebra below the C2 level forms SAS (subaxial subluxation 2 mm). Apophyseal joint ankylosis is detected most commonly between the C2 and C3 vertebrae.

as in regular vertebrae of its kin but has fused with the body of the second vertebra forming the axis, dens, or odontoid process of it. What remains of the atlas forms the anterior and posterior arch of the atlas connected with the right and left lateral masses, which house the transverse and superior and inferior articular processes. This ring-formed backbone contains the usual extensions for xation of muscles and ligaments and for articulation with the occiput and C2, in an antero-posterior direction: the anterior tubercle (where the superior oblique bers of the m.

longus colli are inserted), the right and left transverse processes containing a hole for the vertebral artery and vein, the superior articulating processes (which form a part of the bilateral atlantooccipital joint), inferior articulating processes (which form a part of the bilateral atlanto-axial joint), and the posterior tuberculum (instead of the spinous process), extending backwards from the middle point of the posterior arch of the atlas. The atlas forms a heavy-duty tting piece or adapter, which apparently carries a huge weight on it. Most of our colleagues know that is why it is

Table 1 Prevalence (%) of Subluxations of the Cervical Spine in Various Rheumatic Diseases* Rheumatoid Arthritis 2338 (35) 2628 (35) 04 (35) 618 (35) 14 (5) 10 (5) 726 (35, 15) 11 (16) Ankylosing Spondylitis 421 (69) 27 (8, 9) 1 (9) Psoriatic Arthritis 523 (1012) 25 (1012) 0 (12) Juvenile Chronic Arthritis* 1720 (13, 14) 25 (14)

Disorder aAAS AAI pAAS IAAS Rotatory AAS NRRHT SAS Apophyseal joint ankylosis

0 (9) 4445 (7, 9)

316 (10, 12) 011 (10, 12)

56 (13, 14) 4152 (13, 14)

*Figures are based on population or hospital cohorts. The number and the severity of the changes increase with the increasing inammatory activity and duration of the disease except that the prevalence of aAAS may decline as AAI progresses. aAAS anterior atlantoaxial subluxation, pAAS posterior AAS, IAAS lateral AAS, AAI atlantoaxial impaction, NRRHT nonreducible rotational head tilt, SAS subaxial subluxation. Includes patients with juvenile rheumatoid arthritis. Numbers in parenthesis are reference numbers.

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639 boys, the youngest male titan being named Cronus. The titans were considered to be the personications of the forces of nature, Cronus referring to time (eg, chronology, chronicles). Unfortunately, Gaia and Uranus also had unwished for monstrous children, possibly because the divine creatures were unaware of the teratogenic effects of alcohol consumption during pregnancy. These unwanted children included the 1-eyed Cyclopes, spirits of punishment, and 3 monsters. Therefore, Gaia did not want to have more children and asked her youngest son Cronus to castrate her husband Uranus. Cronus did this using an adamantine sickle, a bloody deed. The Gigantes, with monstrous appearance and great strength, were conceived from the blood of Uranus, which fell to earth from his open wound. The bright side of this was

Figure 2 The atlas (vertebra) in anterior (a), lateral (b), and cranial view (c). 1 anterior tubercle, 2 transverse process, 3 anterior arch, 4 posterior arch, 5 lateral mass, 6 posterior tubercle, 7 superior articular facet.

called atlas, but the story behind it and, in particular, the answer to the interesting question of why Atlas, why not Heracles, are not very well known. The present minireview deals with the reason this vertebral body is called atlas. More modern history of the cervical spine is nicely presented by John H. Bland (21).

Gaia and Uranus

Gaia is the Earth or Mother Earth. The name is derived from the Greek common noun for land, ge or ga, as in geography. She had no mother or father but was born from Chaos. Her rst son, born without male intervention (parthenogenetically), was named Uranus after the sky. He also became the rst husband of Gaia and the rst emperor of the universe.

Titans
Gaia and Uranus had several children. Twelve of them were giants known as titans. Six of them were girls and 6 were

Figure 3 Cronus eating his offspring. It symbolizes the fact that time saves nobody, and all will pass away. This is a painting by Francisco de Goya Cronus Devouring His Children, which is published with the kind permission of Museo Del Prado, Madrid, Spain. (Color version of gure is available online.)

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Figure 4 Radiographs of the upper cervical spine in a patient with rheumatoid arthritis. Anterior atlanto-axial subluxation (6 mm) is seen in exion of the neck (a), but disappears in extension (b). Anterior view of occipito-atlanto-axial junction of the same patient (c) demonstrates the lateral atlanto-occipital joints and atlanto-axial facet joints on both sides of the dens of the axis.

that the New York Giants would be nameless without this historical event. Cronus became the second emperor of the Universe. A wise man oracle, however, informed Cronus that 1 of his children would depose him. Being suspicious of each and every 1 of them, Cronus ate his own children (Fig. 3). One dear child, Zeus, was saved by Gaia, who put a stone in his diapers instead of the child. Later, in his manhood, Zeus, an Olympian, attracted the new gods of Olympos around him. They were disgusted by the deeds of Cronus and a war started between Zeus troops and the titans.

Heracles and ordered him to perform 12 labors. The eleventh of the labors was to take the golden apples from the garden of Atlas, close to the Atlas Mountains (garden of Hesperides, see below). These golden apples had been planted from an apple which Zeus and Hera had received as a wedding present from their grandmother Gaia. They were entrusted to the Hesperides (daughters of the evening, Fig. 5), which, as we remember, were daughters of Atlas. The apples were guarded by a dragon, Ladon, who had a hundred heads, all speaking different languages. The advantage of this was that the dragon

Atlas
The titans were led by Atlas. Atlas had been fathered by a titan, Iapetus, and his mother was the nymph Clymene. He is supposed to have been the king of the legendary Atlantis. He was also the father of the Hesperides or the daughters of the evening, to whom we will come back to later. Atlas led the storming of the gods of the Olympos in heaven, but the titans lost the war. Zeus punished Atlas so that he had to carry the Heavens (often in the ne arts depicted as Earth) on his shoulders forever. Atlas refers to bearer or endurer. The site of this punishment is supposed to be in Northern Africa, in Libya, where the tops of the mighty Atlas Mountains are often covered by heavy clouds, symbolizing Atlas with the Heavens on his huge shoulders. To come back to where we started, this also symbolizes the heavy task of the rst cervical vertebra. The dry weight of the atlas is only approximately 10 to 20 g and carries the 3- to 5-kg head. Sometimes the strength of atlas fails (see Fig. 4 for radiographs from a case with an anterior atlanto-axial subluxation).

Why Atlas, Why Not Heracles?

Heracles (Latin: Hercules) was a son of the Olympos god, Zeus, and Alcmene. He is famous for his strength. In his cradle, he strangled 2 serpents, and killed a lion before manhood. However, he became mad and killed his own children. In his anguish and despair, he contemplated suicide, but was advised by the oracle of Apollo to serve the king of Mykene, who was a difcult emperor to serve. If he was successful, he would become free from his anguish. The king of Mykene wanted to make this impossible for

Figure 5 Hesperides, the beautiful daughters of the evening, guarding the golden apples of Hesperides in a garden often named according to them. This is a painting by Sir Edward Burne-Jones Garden of the Hesperides, published with the kind permission of the Bridgeman Art Library, London, UK. (Color version of gure is available online.)

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641

Acknowledgment
We dedicate our work to Professor John H. Bland, a Titan in this eld of research, for lifetime pioneering work on the rheumatoid cervical spine and thank him for the inspiration he has given to the 2 senior authors of this article.

References
1. Konttinen YT, Santavirta S, Kauppi M, Moskovich R. The rheumatoid cervical spine. Curr Opin Rheumatol 1991;3:429-40. 2. Santavirta S, Kankaanp U, Sandelin J, Laasonen E, Konttinen YT, Sltis P. Evaluation of patients with rheumatoid cervical spine. A review. Scand J Rheumatol 1987;16:9-16. 3. Kauppi M, Hakala M. Prevalence of cervical spine subluxations and dislocations in a community-based rheumatoid arthritis population. Scand J Rheumatol 1994;23:133-6. 4. Neva MH, Kaarela K, Kauppi M. Prevalence of radiological changes in the cervical spinea cross sectional study after 20 years from presentation of rheumatoid arthritis. J Rheumatol 2000;27:90-3. 5. Castro S, Verstraete K, Mielants H, Vanderstraeten G, de Reuck J, Veys EM. Cervical spine involvement in rheumatoid arthritis: a clinical, neurological and radiological evaluation. Clin Exp Rheumatol 1994;12: 369-74. 6. Hunter T. The spinal complications of ankylosing spondylitis. Semin Arthritis Rheum 1989;19:172-82. 7. Meikle JAK, Wilkinson M. Rheumatoid involvement of the cervical spine. Ann Rheum Dis 1971;30:154-61. 8. Ramos-Remus C, Gomez-Vargas A, Gusman-Guzman JL, Jimenez-Gil F, Gamez-Nava JI, Gonzalez-Lopez L, et al. Frequency of atlantoaxial subluxation and neurologic involvement in patients with ankylosing spondylitis. J Rheumatol 1995;22:2120-5. 9. Laiho K, Kauppi M. The cervical spine in patients with ankylosing spondylitis. Clin Exp Rheumatol 2002;20:738 10. Salvarani C, Macchioni P, Cremonesi T, Mantovani W, Battistel B, Rossi F, et al. Ann Rheum Dis 1992;51:73-7. 11. Lassoued S, Hamidou M, Fournie B, Fournie A. Cervical spine involvement in psoriatic arthritis. J Rheumatol 1989;16:251-2. 12. Laiho K, Kauppi M. The cervical spine in patients with psoriatic arthritis. Ann Rheum Dis 2002;61:650-2. 13. Espada G, Babini JC, Maldonado-Cocco JA, Garca-Morteo O. Radiological review: the cervical spine in juvenile rheumatoid arthritis. Semin Arthritis Rheum 1988;17:185-95. 14. Laiho K, Savolainen A, Kautiainen H, Kekki P, Kauppi M. The cervical spine in juvenile chronic arthritis. Spine J 2002;2:89-94. 15. Halla JT, Fallahi S, Hardin JG. Nonreducible rotational head tilt and lateral mass collapse. A prospective study of frequency, radiographic ndings, and clinical features in patients with rheumatoid arthritis. Arthritis Rheum 1982;25:1316-24. 16. Cabot A, Becker A. The cervical spine in rheumatoid arthritis. Clin Orthop 1978;131:130-40. 17. Bland JH, Boushey DR. Anatomy and physiology of the cervical spine. Semin Arthritis Rheum 1990;20:1-20. 18. Bland JH. Basic anatomy. In: Bland JH, editor. Disorders of the Cervical Spine. Diagnosis and Medical Management. Second ed. Philadelphia, PA: Saunders, 1994:41-70. 19. Santavirta S, Hopfner-Hallikainen D, Paukku P, Sandelin J, Konttinen YT. Atlanto-axial facet joint arthritis in the rheumatoid cervical spine. A panoramic zonography study. J Rheumatol 1988;15:217-23. 20. Konttinen YT, Bergroth V, Santavirta S, Sandelin J. Inammatory involvement of cervical spine ligaments in rheumatoid arthritis patients with atlanto-axial subluxation. J Rheumatol 1987;14:531-4. 21. Bland JH. The cervical spine: a historical perspective. In: Santavirta S, Konttinen YT, editors. The Rheumatoid Cervical Spine. Forssa: Forssan Kirjapaino, 1994:5-17.

Figure 6 Heracles, now with the golden apples of Hesperides, ees Atlas, who was tricked into taking the Earth on his shoulders again. (Color version of gure is available online.)

never slept or closed his eyes, a formidable advantage for a guard. Several heads were always on guard. Heracles succeeded in killing the dragon, Ladon, by shooting an arrow over the wall of the garden. However, he was gentleman enough not to harm the pretty daughters of the evening. Instead, he asked for help from Atlas, who carried Earth on his shoulder and who was the father of the daughters of the evening, to retrieve the apples. He offered to hold the Earth for Atlas for a while, if Atlas would go and pick up the golden apples for him. Atlas did this and picked up the golden apples of Hesperides. However, when he came back, he did not want to take the Earth on his shoulders again. Instead, he insisted that he would bring the apples to the king of Mykene, to perform a labor. After a long argument, which led nowhere, Heracles tricked Atlas into believing that he was going along with him. He complained that his position was uncomfortable and that the Earth was pressing his shoulder. He had naturally not known that he would have to, against the agreement, continue to carry the Earth forever. He asked Atlas to carry the Earth briey so that he could get a pillow for his aching shoulder. Atlas put the golden apples down and took the Earth on his mighty shoulders. Now Heracles could pick up the apples, take to ight (Fig. 6), and complete his eleventh labor ordered by the unpleasant king of Mykene. This story has a happy ending. Although Heracles was later poisoned to death, he was endowed immortality among the gods of Olympos. However, Atlas, the bearer or endurer, is even more famous today and continues his vital task under the heavy weight of the skull.