• DOI:10.7326/0003-4819-150-8-200904210-00010
• Corpus ID: 24766974

Akhenaten and the Strange Physiques of Egypt's 18th Dynasty

@article{Braverman2009AkhenatenAT,
  title={Akhenaten and the Strange Physiques of Egypt's 18th Dynasty},
  author={Irwin M. Braverman and Donald Bruce Redford and Philip A Mackowiak},
  journal={Annals of Internal Medicine},
  year={2009},
  volume={150},
  pages={556-560}
}

• I. Braverman, D. Redford, P. Mackowiak
• Published 21 April 2009
• Medicine
• Annals of Internal Medicine

Key Summary Points Akhenaten (also known as Amenophis IV) was one of ancient Egypt's most controversial pharaohs because of his devotion to Aten, the Sun-disc, as his one-and-only god; he has a strange appearance in images produced after the introduction of his radical new religion. Whether the floridly androgynous images of Akhenaten were symbolic representations, meant to concretize the concept of him as the father and mother of all mankind, or realistic representations indicative of some deforming genetic disorder is the subject of continuing debate. The bizarre physical features portrayed in these images (specifically, gynecomastia and cranial abnormalities) were shared by many members of Egypt's 18th Dynasty and are therefore realistic rather than symbolic. The physical abnormalities of Egypt's 18th Dynasty are best explained by either 2 different familial disordersthe aromatase excess syndrome and the sagittal craniosynostosis syndromeor a variant of the AntleyBixler syndrome. When Akhenaten, also known as Amenophis IV, ascended Egypt's Horus Throne of the Living in 1377 bce, he was ill-prepared to rule the most powerful empire on earth. He had previously been excluded from court functions, possibly because of his strange physique. Although some speculate that excessive inbreeding caused his deformities, others point out that he was the product of a gene pool that had not been corrupted by close intermarriage for at least 2 generations (1). A Case Summary The distinctive physical features of Akhenaten depicted in statues and reliefs are at once odd, strikingly diverse, and inconsistent. Likenesses produced at the beginning of his reign depict him in traditional pharaonic guise, with a relatively normal face and physique. However, after he created his radical new religion that focused on Aten (the Sun-disc) and became history's first monotheist, his images became floridly androgynous, with an elongated head, almond-shaped eyes, lantern-like jaw, protruding teeth, and large ears (Figure 1). Some representations show similar distortions of the head, body, and extremities in Akhenaten's children and his principal wife, Nefertiti (Figure 2). Figure 1. Alabaster statuette of Akhenaten. Reproduced with permission from Bildarchiv Preussischer Kulturbesitz/Art Resource, NY Aegyptische Museum, Staatliche Museen zu Berlin, Berlin, Germany. Figure 2. Relief of Akhenaten, Nefertiti, and their 3 eldest daughters. The second daughter, Meketaten, sitting on Nefertiti's lap, is 5 to 7 years old and has prominent breasts and wide hips. Reproduced with permission from Vanni/Art Resource, NY Aegyptisches Museum, Staatliche Museen zu Berlin, Berlin, Germany. Only a bit more is known of the health of Akhenaten or of his family. He had 6 children by Nefertiti; all were daughters. Many members of his family died during a plague that ravaged his kingdom. Akhenaten survived the plague only to die in the summer of 1359 bce under obscure circumstances (1). The Heretic Pharaoh Amenophis was born in ancient Egypt's royal city of Memphis in approximately 1385 bce. His father, Amenophis III, ruled an empire stretching nearly 2000 miles from the central Sudan to the mountains of Anatolia. An elder brother, named Thutmose after his grandfather, stood in line to inherit the throne but died suddenly in the third decade of his father's reign, leaving Prince Amenophis the heir apparent. As a youth, Prince Amenophis was assigned undistinguished tutors. When his father became ill, the ailing king moved his court south to the more salubrious climate of Thebes, where the prince seems to have been hidden from public view until becoming Pharaoh. Amenophis III died in his 38th year on the throne (around 1377 bce) and was immediately succeeded by Prince Amenophis (now Amenophis IV). At first, little changed. The new king continued to reside at Thebes in his father's palace and, shortly after his accession, married a strikingly beautiful woman named Nefertiti. Some believe she was a distant relative. In the first or second year of their marriage, she bore a daughter, Meret-aten. Sometime during the new king's second year on the throne, he experienced a revelation regarding the gods; their temples; their cult images; and the sacred, prescriptive literature. This new insight induced him to decree all the gods of the Egyptian pantheon nonexistent except one, the solar deity. Pursuant to this revelation, he forbade the depiction of any god or goddess except the falcon-headed human figure bearing a large sun-disc on its head. He then had the great house of the Sun-disc at Karnak transformed into a new, open-air temple, wholly devoid of cultic apparatus except for the large altar on which offerings to the sun could be made. He diverted the incomes of the other temples to the new establishment, which caused all other shrines to close their doors. In decorating his new structures, the king commissioned a new artistic style which depicted him in a greatly distorted form as something akin to a humanoid praying mantis (1), with all of the bizarre and effeminate features we have enumerated. Explanations for these strange images have followed 2 opposing lines. One maintains that they were purely symbolic and intended to concretize the concept of Amenophis IV as the father and mother of all mankind. The other holds that they were realistic and conformed, in the main, to what the king actually looked like. With regard to the former hypothesis, Egyptian art is replete with hermaphroditic representations; however, none is of the character of the images of Amenophis IV. In the fifth year of his reign, conflict with the priesthood of Amun induced the king to move to a new site in Middle Egypt, near the modern-day village of Amarna, and build a new capital (Akhet-aten, the Horizon of the Sun-disc). On leaving Thebes, he closed the Temple of Amun-Re, king of the gods, and expunged the deity's representations and name from all monuments. He also discarded Amenophis (Amun is satisfied) in favor of a new title, Akhenaten (useful for the Sun-disc). Once settled in Akhet-aten, he seems never to have traveled abroad, apparently content to closet himself in his new capital, oblivious to the economic calamity his new belief system brought about. Something of Akhenaten's personality can be discerned from his diplomatic correspondences and the few inscriptions that survived his later anathematization. He was not an evangelical and had no wish to proselytize or convert his people. He confined his teaching to an inner circle of courtiers, all of whom remarked on the beauty of his mellifluous voice. In curious contrast to the single-mindedness with which he pressed his new belief system, he was frequently irresolute about the outside world and procrastinated constantly in his negotiations with foreign ambassadors. Akhenaten died during his 17th year on the throne. The cause of his death is unknown. Contemporary texts speak of a plague that ravaged the eastern Mediterranean, Anatolia, and (undoubtedly) Egypt, in which many of the Amarna faithful are thought to have perished. Akhenaten died some time later and was interred in an unknown location, possibly in an Amarna tomb he had had carved for himself. Some suspect that shortly after his capital was abandoned, his remains were moved to KV 55 in the Valley of the Kings (26), although they have yet to be found. The Dynastic Disorders Akhenaten's gynecomastia and androgynous appearance have previously been attributed to a host of genetic disorders, including the Marfan syndrome, Frohlich syndrome (adiposogenital dystrophy), Klinefelter syndrome, and androgen insensitivity syndrome (1, 7, 8). Although each of these has features in common with the abnormalities depicted in the Amarna images, each also has features inconsistent with what we know of Akhenaten's clinical history. The Marfan syndrome is not associated with either gynecomastia or an androgynous habitus, and patients with each of the other syndromes are nearly always sterile (Akhenaten fathered 6 daughters). In 1980, Dr. Bernadine Paulshock called attention to evidence of familial gynecomastia in Egypt's 18th Dynasty, Akhenaten's extended family (9). She diagnosed familial gynecomastia, mechanism unknown but probably dominantly transmitted, on the basis of presence of the physical finding in images of Amenophis III (Akhenaten's father), Amenophis IV (Akhenaten), and Tutankhamun and Smenkhkare (his brothers) (Table) (1019). She also detected evidence of gynecomastia in a sculpture of Thutmose IV (his grandfather) but apparently was unaware that a statue of Thutmose I, the patriarch of Akhenaten's family, also shows evidence of breast enlargement. We believe the presence of gynecomastia in no fewer than 4 generations of the 18th Dynasty without apparent impairment of male fertility is best explained by the aromatase excess syndrome, a dominantly transmitted disorder first described in 1977 (20). Table. Images Depicting Endocrine and Cranial Abnormalities in the Principal Rulers of Egypt's 18th Dynasty Aromatase (estrogen synthetase) is one of many P450 enzyme systems. In the aromatase excess syndrome, aromatase converts the C19 steroids androstenedione, testosterone, and 16-hydroxyandrostenedione into estrone, estradiol, and estriol, respectively. This occurs not just in gonadal tissues but also in extragonadal tissues, such as subcutaneous fat and skin fibroblasts. In affected males, mutations of the aromatase gene (CYP19 on chromosome 15q21) and promoters from adjacent genes modulate CYP19 to produce increased aromatase, which results in gynecomastia, a eunuchoid habitus with normal fertility, and (potentially) the mellifluous (female) voice Akhenaten is purported to have had. Affected females have isosexual precocity and, in some instances, macromastia (2123). Gynecomastia in images of the patrilineal line of the 18th Dynasty is both striking and widespread (Figure 3, A throug