Sclerosing bone dysplasias: neurologic assessment and management

Current Opinion in Otolaryngology & Head and Neck Surgery
Louis M Hofmeyr, H Hamersma

Abstract

Sclerosing bone dysplasias are rare genetic disorders of bone remodeling in which excessive bone formation takes place, resulting in encroachment on neural structures. The infant usually appears normal at birth, and the first sign of a problem only comes when a neurologic deficit develops, usually in the form of an acute facial palsy. Although less than 300 cases have been published, these conditions should always be considered in the differential diagnosis of facial nerve palsy, especially in children. This review highlights the neurologic presentation and assessment as well as the management of sclerosing bone dysplasias. An exciting development is the recent discovery of the sclerosteosis (SOST) gene, which is involved with excessive bone formation in sclerosteosis and Van Buchem disease. Researchers in bone metabolism and pharmaceutical companies are now utilizing this knowledge to develop a medicine for osteoporosis. In children and young adults an acute facial palsy, especially if it is recurrent, as well as a conductive hearing loss may be the first sign of a sclerosing bone dysplasia.

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Citations

Dec 19, 2013·Surgical Neurology International·Emerson Magno de AndradeManoel Jacobsen Teixeira
Mar 20, 2014·The Journal of Laryngology and Otology·J M PotgieterH Hamersma
Mar 27, 2012·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·M E van EgmondO F Brouwer
Oct 28, 2008·Orthodontics & Craniofacial Research·T ArntsenL Sonnesen
Feb 3, 2018·Medicine·Shang-Fu Hsu, Chen-Chun Lin
Dec 20, 2007·The Journal of Biological Chemistry·Alexander G RoblingCharles H Turner
Oct 10, 2020·Seminars in Musculoskeletal Radiology·Nick Van de VoordeFilip M Vanhoenacker

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