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Limb gigantism, neurofibromatosis and royal heredity in the Ancient World 2500 years ago: Achaemenids and Parthians

2010; Elsevier BV; Volume: 64; Issue: 4 Linguagem: Inglês

10.1016/j.bjps.2010.08.025

1878-0539

Hutan Ashrafian,

Dupuytren's Contracture and Treatments

I read with interest the cases of limb gigantism recently presented in this journal. These cases are rare and causes include congenital diseases such as Proteus syndrome, Klippel-Trenaunay-Weber syndrome, neurofibromatosis and macrodystrophia lipomatosa. They can also be acquired in cancers and lymphoedema. Unilateral upper limb gigantism has only been formally recognised in recent centuries, although it has been described in antiquity. As early as two and a half millennia ago, the emperor Artaxerxes I of the Persian empire was recognised by ancient authors as ‘Long hand’ (MacrocheirGreek or Longimanus-Latin) as his right hand was reported to be longer than his left. Artaxerxes I was the grandson of Darius the Great and the son of Xerxes who had attempted to invade Greece. He ruled the largest world-empire at that time for forty-one years. This covered an area of land that was subsequently expanded by Alexander the Great and would only be surpassed by Genghis Khan over a millennium later. Later Persian dynasties would claim hereditary descent from the Achaemenids. Of these, the kings of the Parthian Empire (also known as the Arsacids ruling between 247 BCe224 AD) contentiously claimed Achaemenid descent. It has recently been proposed that based on numismatic evidence of skin lesions (Figure 1), many of the Parthian kings suffered from familial neurofibromatosis. This empire began its rule approximately 200 years after the Achaemenids, and if there was a hereditary connection between these dynasties, neurofibromatosis could also account for Artaxerxes’ macrodactyly. This hereditary pathology might therefore add to the evidence of a familial link between these two ancient Persian dynasties.