Parry-Romberg and Rasmussen's syndromes: are the similarities just coincidental?

Citation
A. Straube et al., Parry-Romberg and Rasmussen's syndromes: are the similarities just coincidental?, NERVENARZT, 72(8), 2001, pp. 641-646
Citations number
26
Categorie Soggetti
Neurology
Journal title
NERVENARZT
ISSN journal
00282804 → ACNP
Volume
72
Issue
8
Year of publication
2001
Pages
641 - 646
Database
ISI
SICI code
0028-2804(200108)72:8<641:PARSAT>2.0.ZU;2-C
Abstract
The Parry-Romberg syndrome is a rare and poorly understood disease characte rized by slowly progressive, localized atrophy of the skin,subcutaneous tis sue, muscles, and bones. The atrophy is typically localized in the face and begins in youth. In some patients, imaging can show the lesions and atroph y of the ipsilateral hemisphere of the brain. We report on a patient in who m the disease has lasted 36 years and discuss the possibility that the Parr y-Romberg syndrome is related to known autoimmune disorders of the soft tis sue (e.g., linear scleroderma) and Rasmussen's syndrome. There are some rem arkable clinical similarities between these two syndromes, including age of onset, unilateral manifestation, and occurrence of focal seizures. It is m ost probable that both diseases have an autoimmunological background.