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Ambras syndrome

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Hypertrichosis lanuginosa also called "Congenital hypertrichosis lanuginosa", and "congenital hypertrichosis universalis", is a genetic condition characterized by hair all over the body. This condition affects less than 1 person in a billion. Fewer than 40 cases of CHL and Ambras Syndrome have been documented worldwide. There is no known treatment other than depilation. The genetic anomaly is found on chromosome 8, though an X-linked syndrome of hypertrichosis associated with gingival hyperplasia has been described.

In the 19th century many people with hypertrichosis performed in sideshow acts.

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PAPERBACKS
From Independent, The (London), 4/5/98 by LILIAN PIZZICHINI

The Diving-Bell and The Butterfly by Jean-Dominique Bauby, Fourth Estate pounds 5.99. "Locked-in syndrome" hit the world's headlines last year following the publication of a miraculous account of this mercifully rare condition. One day, Jean Dominique Bauby, editor-in- chief of French Elle, awoke in a hospital bed, his alert mind trapped within the diving-bell of his paralysed body. Only his left eyelid was mobile, but by blinking his way through the French alphabet he was able to "dictate" his story. What is remarkable is the grace and wit with which he tells it, communiques from his fluttering lid bear no trace of the laboriousness of their transmission. This former man-about-town comes to appreciate the slowness of the world, the changing pattern of light and shade on the walls of his room, and summons up the imaginative energy to transform the daily round of hospital visits, therapies and baths into a series of droll, painful or gloriously epiphanic moments. At times, his book is unbearably moving - his daughter performs cartwheels in the sea, while he sits frozen in his wheelchair, silently weeping - but pages later, he alights on a favourite Breton landscape, a delicious boeuf en gelee, or the court of King Midas. Four days after the book's publication, Bauby died. He left behind a great, heart-breaking testament to his indomitable imagination.

Sappho's Immortal Daughters by Margaret Williamson, Harvard pounds 9.95. "Why read the works of a female poet primarily as the key to her emotional and sexual life, when those of her male contemporaries are examined for literary qualities?" asks Williamson in her reconstruction of the social and historical context of Sappho's poetry. Despite the poet's status as a lesbian icon, her poems, dating back to 600 BC, survive only in fragments. But Williamson finds enough textual evidence to place Sappho at the centre of her cultural milieu rather than on the outskirts, where many scholars are keen to relegate her. She is the subversive from within, and whereas her contemporaries made love and war gender-specific, Sappho's goddesses, "gliding in a straight line like quivering doves/ approach the battle", and turn love itself into the theatre of war. Stories have been woven around this enigmatic, "honey-voiced" figure, but Williamson cuts through them to present Sappho singing to her followers on the isle of Lesbos of the rites of love and war, and the blurring of identities therein.

! Journey to Ithaca by Anita Desai, Vintage pounds 6.99. Herman Hesse has a lot to answer for. In this intriguingly ambiguous novel, his Journey to the East is the inspiration behind a young couple's quest for spiritual nourishment in the ashrams of India. But the "awakening" Hesse speaks of is exposed as gullibility on the part of Matteo, and despair for his more cynical bride, Sophie. Desai's languorous prose delves into the myths, dirt and colours of India which become as shimmeringly seductive as its gurus. She constructs an epic narrative that plays on our need for faith, and refuses to deliver an easy answer. The Enchanted World of Sleep by Peretz Lavie, Yale pounds 9.95. Peretz Lavie provides us with a panoramic view of the landscape that opens up to us once we close our eyes to sleep. Lavie heads one of the largest sleep laboratories in the world, and deftly mixes anecdotal, first-hand accounts with statistical charts to make this an engaging study of sleep and dreams in animals, toddlers, insomniacs and narcoleptics. When his grandfather - "a farmer who tilled the land with every fibre of his being" - heard Lavie intended to devote himself to the study of sleep, he told him to get a proper job. But Lavie persisted, and shares the fruits of his labours, the examination of Greek and Judaic traditions, the theories of Pavlov and Proust, to make this an entertaining, poetic journey into the mysterious realm of Lethe. The Dog King by Christoph Ransmayr, Vintage pounds 6.99. This novel is a stark and solemn read. Its subject - the aftermath of the Second World War - is a familiar one, but his treatment of it is daringly original. His vision is of a small town in occupied Germany forced by the Allies to revert to a pre-industrial society. His protagonists are a reluctant blacksmith who dreams of feats of engineering ingenuity; Ambras, the Dog King, who lives in a derelict mansion inhabited by wild hounds; and Lily, a mysterious figure who digs up rusty weapons. The villagers are forced by their guards to re-enact ceremonies of torture dressed as concentration- camp inmates. (What Ransmayr seems to be saying in this complex, at times overwrought, fable is that both the guards and their prisoners are attempting, in vain, to cleanse themselves of guilt by ritualising the horrors of war.) The plot comes to a head when the Dog King and his consorts make a bid for escape - and the tension mounts to a -literally - inflammatory climax. In Greece cats are tough - they roam in packs and hang on street corners with classic insouciance. Hans Silvester's photographic study More Cats of the Greek Islands (Thames and Hudson pounds 14.95) contains 137 dazzling, vibrant photographs of everyday downtown cats with the occasional chicken, dog and sardine thrown in for dramatic interest

Copyright 1998 Newspaper Publishing PLC
Provided by ProQuest Information and Learning Company. All rights Reserved.

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