Many of those affected with the proximal limb weakness will eventually require assistive devices such as canes, braces or a wheelchair. As with all surgical procedures, they come with many risk factors. As the dysphagia becomes more severe, patients become malnourished, lose significant weight, become dehydrated and suffer from repeated incidents of aspiration pneumonia. These last two are often the cause of death. Epidemiology
The disease is found across 5 continents (30 countries) and is frequently seen in French Canadians, with a prevalence 1:1000. OPMD affects males and females equally, and affected individuals have been found in Europe (France), Jewish Ashkenazi, and Spanish Americans. See also
Muscular dystrophy
PABPN1

References
Further reading
Raz, Yotam; Raz, Vered (2014-01-01). "Oculopharyngeal muscular dystrophy as a paradigm for muscle aging". Frontiers in Aging Neuroscience. 6: 317. doi:10.3389/fnagi.2014.00317. PMC 4226162. PMID 25426070. Hill, M. E.; Creed, G. A.; McMullan, T. F. W.; Tyers, A. G.; Hilton-Jones, D.; Robinson, D. O.; Hammans, S. R. (1 March 2001). "Oculopharyngeal muscular dystrophy". Brain. 124 (3): 522–526. doi:10.1093/brain/124.3.522. ISSN 0006-8950. PMID 11222452. Gómez-Torres, Antonio; Abrante Jiménez, Antonio; Rivas Infante, Eloy; Menoyo Bueno, Alicia; Tirado Zamora, Isabel; Esteban Ortega, Francisco (November 2012). "Cricopharyngeal Myotomy in the Treatment of Oculopharyngeal Muscular Dystrophy". Acta Otorrinolaringologica (English Edition). 63 (6): 465–469. doi:10.1016/j.otoeng.2012.11.009. ISSN 2173-5735. PMID 22898142. Oculopharyngeal muscular dystrophy at NIHs Office of Rare Diseases


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Diagnosis
Diagnosis is based on clinical history and examination. Examination is clinical, via anterior rhinoscopy and fibreoptic, using nasendoscopy. A CT scan can be used to confirm the diagnosis and review disease extent. Treatment
Treatment of atrophic rhinitis can be either medical or surgical. Medical measures include:

Nasal irrigation using normal saline
Nasal irrigation and removal of crusts using alkaline nasal solutions. 25% glucose in glycerine can be applied to the nasal mucosa to inhibit the growth of proteolytic organisms which produce foul smell. Local antibiotics, such as chloromycetine. Vitamin D2 . Estradiol spray for regeneration of seromucinous glands and vascularization of mucosa. Systemic streptomycin (1g/day) against Klebsiella organisms. Oral potassium iodide for liquefaction of secretion. Placental extract injected in the submucosa. Rifampicin 600 mg once daily for 12 weeks.Surgical interventions include:

Youngs operation. Modified Youngs operation. Narrowing of nasal cavities, submucosal injection of Teflon paste (Williams operation), section and medial displacement of the lateral wall of the nose. Transposition of parotid duct to maxillary sinus or nasal mucosa (Wittmacks operation)
Complete inversion of mucoperiosteum of maxillary antrum to create a neoturbinate (Raghav Sharans operation)
Repeated Stellate ganglion block (Sardanas operation)
Vestibuloplasty

History
This disorder was known since the time of ancient Egypt, almost 4,000 years ago, and descriptions of it are found in the historical medical papyri. In the Edwin Smith Papyrus (1700 BC) it was prescribed a treatment based on wine and breast milk to cure this disease. The ancient Greek and Indian civilizations were aware of atrophic rhinitis. See also
Empty nose syndrome

References


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