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PARRY-ROMBERG SYNDROME | DESCRIPTION | CAUSES | SYMPTOMS | | ONLINE RESOURCES | The names Progressive Facial Hemiatrophy, Progressive Hemifacial Atrophy, Romberg Syndrome, PRS, HFA are synonyms for this disorder
The following information is excerpted from the National Organization for Rare Disorders, Inc.database on rare disorders and is posted in compliance with the FAIR USE DOCTRINE and is for educational purposes only and not for commercial use. Description The resulting wasting can sometimes result in distinctive changes of the eyes and hair; and neurological abnormalities including episodes of uncontrolled electrical disturbances in the brain (seizures) and episodes of severe pain in tissues supplied by the fifth cranial nerve (trigeminal nerve) including the mouth, cheek, nose. Trigeminal neuralgia is sometimes one of its complications. Symptoms In individuals with the disorder, initial facial changes usually involve the tissues above the upper jaw (maxilla) or between the nose and the upper corner of the lip (nasolabial fold) and progress to involve the angle of the mouth, the areas around the eye, the brow, the ear, and/or the neck. In most cases, progressive tissue wasting is limited to one half of the face, usually the left side. Affected areas may demonstrate shrinkage and atrophy of tissues beneath the skin (subcutaneous tissue), the layer of fat under the skin (subcutaneous fat), and underlying cartilage, muscle, and bone. In addition, the skin overlying affected areas may become darkly pigmented (hyperpigmentation), with in some cases certain areas of white (depigmented) patches (vitiligo). Many individuals also experience atrophy of half of the upper lip and tongue as well as abnormal exposure, delayed eruption, or wasting of the roots of certain teeth on the affected side. In most affected individuals, hemifacial atrophy typically progresses over approximately three to five years and then ceases. In many cases, hair abnormalities may also appear on the affected side, including whitening (blanching) of the hair as well as abnormal bald patches on the scalp and loss of eyelashes and the middle (median) portion of the eyebrows (alopecia). Individuals with Parry-Romberg Syndrome may experience associated neurological abnormalities. These can include severe headaches that last for extended periods of time and may be accompanied by visual abnormalities, nausea, and vomiting (migraines); facial pain (trigeminal neuralgia); and/or periods of uncontrolled electrical disturbances in the brain (seizures) that usually are characterized by rapid spasms of a muscle group that spread to adjacent muscles (contralateral Jacksonian epilepsy). Causes Online
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