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Parry-Romberg syndrome (PRS) is a sporadic disease of unknown etiology with typical onset in childhood or in young adults. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. The neurological symptoms usually include focal epilepsy, migraine, and unilateral brain lesions on the same side as the atrophy. A common neuroimaging finding of the syndrome is white matter high signal intensity on brain magnetic resonance (MR) imaging. Rasmussen encephalitis (RE) is a rare and chronic inflammatory disease of the brain that begins in the first decade of life and more rarely in adolescents and adults. It usually involves one hemisphere with focal cortical inflammation. Neurologic symptoms are intractable seizures and progressive hemiplegia. Both PRS and RE are often associated with other inflammatory or autoimmune disorders and only 1 case of both syndromes has been reported in literature. We report the clinical and neuroradiological findings in a 6-year-old boy, presenting with focal hemifacial and arm motor seizures and progressive facial hemiatrophy. Serial MR imaging studies revealed progressive brain hemispheric signal alterations and atrophy. This would thus suggest acoexistence of PRS and RE.
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http://dx.doi.org/10.1111/j.1552-6569.2009.00398.x | DOI Listing |
Cleft Palate Craniofac J
November 2024
Department of Maxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objective: To characterize mandibular morphology in patients with Parry-Romberg syndrome (PRS).
Design: Retrospective study.
Setting: A craniofacial center.
Dent Med Probl
December 2024
Department of Oral Pathology, Wroclaw Medical University, Poland.
Symmetry is present in various aspects of everyday life. A symmetrical face is considered attractive, whereas a lack of facial symmetry is regarded as a source of functional and aesthetic problems. Most of the people exhibit slight asymmetries, but some of them reveal severe asymmetries.
View Article and Find Full Text PDFAesthetic Plast Surg
October 2024
Department of Plastic and Cosmetic Surgery, Nanfang Hospital, Southern Medical University, 1838 Guangzhou North Road, Guangzhou, 510515, Guangdong, People's Republic of China.
Background: Alar atrophy associated with Parry-Romberg syndrome (PRS) can lead to significant nasal contour deformities. The primary objective of this study was to address nasal alar deformities in PRS patients through the application of a nasolabial transfer flap combined with an auricular cartilage graft after sequential fat grafting.
Methods: All PRS patients with alar deformities who underwent our reconstructive surgery were included in this case series.
Plast Reconstr Surg
October 2024
Department of Plastic and Reconstructive Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Background: In 2021, a meta-analysis showed fat graft retention varied from 26 to 83%. In a retrospective study including patients with Parry-Romberg syndrome, the younger age group had higher satisfaction scores (3.8 vs.
View Article and Find Full Text PDFRadiol Case Rep
December 2024
Radiology Directorate, Komfo Anokye Teaching Hospital (KATH). P.O. Box 1934, Kumasi Ghana.
Parry Romberg syndrome (PRS) is a rare self-limiting disease, typically occurring in children and young adults, that causes slow progressive atrophy of one-half of the face. It primarily affects the subcutaneous tissue and skin with some cases exhibiting deeper extension to glandular, osseous and muscular structures. Neurologic and ocular involvement is variable.
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