Carvalho et al.1919 Carvalho MV, Nascimento GJ, Andrade E, Andrade M, Sobral APV. Association of aesthetic and orthodontic treatment in Parry-Romberg syndrome. J Craniofac Surg. 2010;21(2):436-9. http://dx.doi.org/10.1097/SCS.0b013e3181cfe917. PMid:20216455. http://dx.doi.org/10.1097/SCS.0b013e3181...
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A 22-year-old woman; right side affected. |
Aesthetic complaint. Laboratory tests. She underwent 10 months of drug treatment and 12 months of follow-up. |
Patient had areas of skin hyperpigmentation; oral changes involving the jaw and teeth. Aesthetics improved with drug treatment. |
Duymaz et al.99 Duymaz A, Karabekmez FE, Keskin M, Tosun Z. Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg. 2009;63(4):457-61. http://dx.doi.org/10.1097/SAP.0b013e31818bed6d. PMid:19745718. http://dx.doi.org/10.1097/SAP.0b013e3181...
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A 35-year-old man; right side affected. |
Aesthetic complaint. Clinical examination, laboratory tests, imaging studies, and evaluation of cranial nerves. Treatment was not described. |
Parry-Romberg syndrome associated with atrophy of the inferior contralateral member. The abnormalities found suggest autoimmune disorder as an etiological mechanism of the disease. Facial asymmetry was observed, with marked hypoplasia in the face and scarring alopecia affecting the temporal region. In the facial computed tomographic images, atrophy was observed in the sternocleidomastoid, masseter, pterygoid, and soft tissues of the right side of the face. |
Longo et al.1010 Longo D, Paonessa A, Specchio N, Delfino LN, Claps D, Fusco L, et al, and the Clinical and Neuroimaging Features. Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features. J Neuroimaging. 2011;21(2):188-93. http://dx.doi.org/10.1111/j.1552-6569.2009.00398.x. PMid:19555404. http://dx.doi.org/10.1111/j.1552-6569.20...
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A 6-year-old boy; left side affected. |
Complaint of epilepsy and seizure crises. Imaging tests, laboratory tests, and serological and neurological examinations. Drug treatment. Follow-up for 2 years. |
During the first 6 months of treatment, seizure episodes have decreased from 25% to 50%. After 2 years, a diagnosis of progressive facial hemiatrophy was made. At the last follow-up, the patient had a mild learning disability and denied headaches. The results of this study indicate the association between Parry-Romberg syndrome and encephalitis/Rasmussen syndrome. |
Menascu et al.1111 Menascu S, Padeh S, Hoffman C, Ben-Zeev B. Parry-Romberg syndrome presenting as status migrainosus. Pediatr Neurol. 2009;40(4):321-3. http://dx.doi.org/10.1016/j.pediatrneurol.2008.11.007. PMid:19302950. http://dx.doi.org/10.1016/j.pediatrneuro...
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A 13-year-old boy; left side affected. |
Complaint of migraine. Physical examination, laboratory test, diagnostic imaging, and biopsy. The patient was followed up for 18 months with drug treatment. |
Patient with en coup de sabre, whose examination led to the diagnosis of the syndrome. The patient did not improve with initial drug treatment. When he began treatment with dexamethasone, his migraine ceased. After 18 months, the patient had mild migraines. The symptoms were controlled by using mild analgesics. |
Gomez-Diez et al.11 Gomez-Diez SG, López LG, Escobar ML, Gutiérrez LJ, Oliva NP. Progressive facial hemiatrophy with associated osseous lesions. Med Oral Patol Oral Cir Bucal. 2007;12(8):E602-4. PMid:18059248.
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A 65-year-old woman; left side affected. |
Anatomopathological studies, laboratory tests, and serological test. Treatment was not described. |
The patient had progressive facial hemiatrophy at age 45 years, with bruises that evolved into a suggestive morphea plate and with bone lesions involving the maxilla and mandible. Progressive resorption of the maxilla and mandible were observed, showing significant bone tissue atrophy and pathological mandibular fracture. The left half of the lips showed a decrease in thickness. The diagnosis was based on clinical and histopathological results, and patient history. |
Paprocka et al.1212 Paprocka J, Jamroz E, Adamek D, Marszal E, Mandera M. Difficulties in differentiation of Parry-Romberg syndrome, unilateral facial sclerodermia, and Rasmussen syndrome. Childs Nerv Syst. 2006;22(4):409-15. http://dx.doi.org/10.1007/s00381-005-1262-x. PMid:16247619. http://dx.doi.org/10.1007/s00381-005-126...
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A 10-year-old girl; left side affected. |
Neurological examinations, laboratory tests, diagnostic imaging, and eye examination. Medical and surgical treatments for seizure control. |
The patient had characteristics specific to encephalitis/Rasmussen Syndrome. The patient had about 40-50 seizures per day, with indication for implementation of vagus nerve stimulator as treatment. |
Hulzebos et al.2020 Hulzebos CV, de Vries TW, Armbrust W, Sauer PJJ, Kerstjens-Frederikse WS. Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair. Acta Paediatr. 2004;93(12):1665-9. http://dx.doi.org/10.1111/j.1651-2227.2004.tb00861.x. PMid:15918232. http://dx.doi.org/10.1111/j.1651-2227.20...
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A 7-year-old boy; right side affected. |
Aesthetic complaint. Laboratory tests and imaging studies. Treatment was not described. |
The patient had progressive facial hemiatrophy on the right side of the face, with prominent unilateral chin loss and hyperpigmented but not hardened and retracted skin. A deviation from the middle jawline to the right and overbite were observed. A diagnosis of progressive facial hemiatrophy was made, with no intracranial lesions. |
Shah et al.1313 Shah JR, Juhász C, Kupsky WJ, Asano E, Sood S, Fain D, et al. Rasmussen encephalitis associated with Parry-Romberg syndrome. Neurology. 2003;61(3):395-7. http://dx.doi.org/10.1212/WNL.61.3.395. PMid:12913207. http://dx.doi.org/10.1212/WNL.61.3.395...
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A 7-year-old boy; right side affected. |
Physical and neurological examination. Medical and surgical treatment. Monitoring for 1 year after surgery. |
The patient had Parry-Romberg Syndrome and epilepsy seizures that were difficult to control. The clinical diagnosis and evaluation led to the diagnosis of encephalitis/Rasmussen syndrome. According to the authors, this is the first report of a pathologically proven case of encephalitis/Rasmussen Syndrome associated with Parry-Romberg Syndrome. |
Viana et al.22 Viana M, Glastonbury CM, Sprenger T, Goadsby PJ. Trigeminal neuropathic pain in a patient with progressive facial hemiatrophy (parry-romberg syndrome). Arch Neurol. 2011;68(7):938-43. http://dx.doi.org/10.1001/archneurol.2011.126. PMid:21747035. http://dx.doi.org/10.1001/archneurol.201...
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A 37-year-old woman; left side affected. |
Complaint about facial pain. Physical examination, thermal testing, diagnostic imaging, and electromyography. Drug treatment. |
The patient had gradual loss of tissue, including chewing muscle volume, which led to mild facial asymmetry and difficulty in chewing. There was atrophy of the soft tissue in the left cheek region and the zygomatic arch; mild depression of the nasolabial fold, and en coup de sabre sign. The patient had difficulty chewing, the EMG signal in the chewing muscles on the left side remained normal compared with those on the right side. |
Hu et al.33 Hu J, Yin L, Tang X, Gui L, Zhang Z. Combined skeletal and soft tissue reconstruction for severe Parry-Romberg syndrome. J Craniofac Surg. 2011;22(3):937-41. http://dx.doi.org/10.1097/SCS.0b013e31820fe27d. PMid:21558914. http://dx.doi.org/10.1097/SCS.0b013e3182...
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6 men and 17 women with an mean age of 21.3 years; the side involved varied between subjects. |
Aesthetic complaint. Classification of atrophy as mild, moderate, and severe according to physical examination results. Surgical treatment was required, and follow-up was conducted for 30.8 months. |
Report of 23 patients with progressive facial hemiatrophy treated with multiple surgical techniques. With the reconstruction of skeletal and soft tissues, the occlusal plane and malocclusions were corrected, thus improving facial asymmetry. |
Qureshi et al.1414 Qureshi UA, Wani NA, Altaf U. Parry-Romberg syndrome associated with unusual intracranial vascular malformations and Phthisis bulbi. J Neurol Sci. 2010;291(1-2):107-9. http://dx.doi.org/10.1016/j.jns.2010.01.003. PMid:20144465. http://dx.doi.org/10.1016/j.jns.2010.01....
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A 10-year-old boy; left side affected. |
Physical examination, eye examination, diagnostic imaging and antibody tests. |
The patient had Parry-Romberg Syndrome associated with intracranial vascular malformation. Facial asymmetry was observed on the left side with hyperpigmentation spots. |
Miao et al.44 Miao J, Liu R, Lin H, Su C, Li H, Lei G, et al. Severe bilateral pyramidal tract involvement in a patient with Parry-Romberg syndrome. Am J Med Sci. 2009;337(3):212-4. http://dx.doi.org/10.1097/MAJ.0b013e31818226f9. PMid:19174689. http://dx.doi.org/10.1097/MAJ.0b013e3181...
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A 22-year-old woman; left side affected. |
Neurological examinations, laboratory tests and diagnostic imaging, and needle electromyography. Treatment was not described. |
The patient had bilateral increased muscle tone, muscle strength, and normal sensitivity, indicating bilateral insult in the pyramidal tract. Disease progression was considered as a rare variant syndrome. |
Okumura et al.1515 Okumura A, Ikuta T, Tsuji T, Kato T, Fukatsu H, Naganawa S, et al. Parry-Romberg syndrome with a clinically silent white matter lesion. AJNR Am J Neuroradiol. 2006;27(8):1729-31. PMid:16971623.
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A 22-month-old boy; left side affected. |
Aesthetic complaint. Neurological examination, diagnostic imaging, and hearing test. Treatment was not conducted, and follow-up was conducted for only 65 months. |
The patient had extensive lesions of the white matter, facial asymmetry, and skin atrophy around the region of the eyelids and maxilla. In the last follow-up, the patient had normal psychomotor development and neurological examinations. |
Pinheiro et al.55 Pinheiro TPS, Silva CC, Silveira CSL, Botelho PCE, Pinheiro M, Pinheiro JJ. Progressive hemifacial atrophy--case report. Med Oral Patol Oral Cir Bucal. 2006;11(2):E112-4. PMid:16505785.
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A 8-year-old girl; right side affected. |
Complaint of dental changes. Orthodontic evaluation, physical examination, and radiography. No treatment was conducted, only follow-up. |
The patient had progressive facial hemiatrophy, presenting facial asymmetry with a marked hypoplasia on the right-side face, lips and nose deviation, large linear scar, and dark en coup de sabre on the right side of the jaw and chin, hyperpigmentation areas of the affected skin, unilateral tongue atrophy, atrophic root development, or pathological resorption of the number of permanent teeth. |
Anderson et al.2121 Anderson PJ, Molony D, Haan E, David DJ. Familial Parry-Romberg disease. Int J Pediatr Otorhinolaryngol. 2005;69(5):705-8. http://dx.doi.org/10.1016/j.ijporl.2004.12.004. PMid:15850693. http://dx.doi.org/10.1016/j.ijporl.2004....
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A 9-year-old boy; left side affected. A 14-year-old girl; right side affected. |
Complaint of pain. Clinical evaluation. Drug treatment and follow-up. |
Report of two cases with Parry-Romberg Syndrome in first cousins. Case 1: developed white lesions on his left cheek, muscle weakness, upper lip anesthesia, asymmetry in his right cheek, and progression of left cheek atrophy, leading to the diagnosis of Parry-Romberg Syndrome. In the evaluation, loss of soft tissue with dentition change was observed. Case 2: developed a depression on the right forehead extending to the neck, facial asymmetry, en coup de sabre with skin groove and fat on the right cheek, hypoplasia of the right side of the maxilla and mandible, and malocclusion. |
Guo et al.77 Guo ZN, Zhang HL, Zhou HW, Lan WJ, Wu J, Yang Y. Progressive facial hemiatrophy revisited: a role for sympathetic dysfunction. Arch Neurol. 2011;68(9):1195-7. http://dx.doi.org/10.1001/archneurol.2011.190. PMid:21911700. http://dx.doi.org/10.1001/archneurol.201...
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A 63-year-old man; right side affected. |
Aesthetic and pain complaint. Physical examination, laboratory and imaging tests, Doppler ultrasonography, and nerve conduction studies. Conducted assessment and drug treatment. |
Patient with progressive facial hemiatrophy with unusual features of brain atrophy detected by Doppler ultrasonography. On physical examination, rightside face atrophy, nose and lip deviations, and enophthalmos of the right eye were observed. The patient had brain hemiatrophy to the contralateral side of facial atrophy. For the authors, this is the first case of contralateral cerebral hemiatrophy. This finding extended the progressive facial hemiatrophy spectrum, indicating the possibility of a bilateral disorder. |
Haldar & Mukherjee1616 Haldar A, Mukherjee A. Parry Romberg’s disease with intractable partial epilepsy. Neurol India. 2007;55(2):160-2. http://dx.doi.org/10.4103/0028-3886.32791. PMid:17558124. http://dx.doi.org/10.4103/0028-3886.3279...
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A 17-year-old woman; right side affected |
Complaints of aesthetics and intractable epilepsy. Physical examination, laboratory tests, neurological examinations, and imaging tests. Assessment and drug treatment were conducted. |
The patient had skin discoloration, initially diagnosed with scleroderma. Parry-Romberg Syndrome was diagnosed at the onset of facial atrophy in the temporal mandibular region. The authors concluded the report addressing the issue of the association between the two diseases. |
O’Flynn & Kinirons66 O’Flynn S, Kinirons M. Parry-Romberg syndrome: a report of the dental findings in a child followed up for 9 years. Int J Paediatr Dent. 2006;16(4):297-301. http://dx.doi.org/10.1111/j.1365-263X.2006.00730.x. PMid:16759329. http://dx.doi.org/10.1111/j.1365-263X.20...
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A 7-year-old boy; left side affected. |
Aesthetic complaint. Extraoral and intraoral examination, diagnostic imaging, and orthodontic evaluation. Treatment was not described. The patient was reevaluated with intervals of 6 to 12 months, with the last revaluation at 15 years old. |
The patient had Parry-Romberg Syndrome and tearing of tooth root. Jaw reduction and area of skin depigmentation, angle atrophy of the mouth and tongue, delayed tooth eruption, facial atrophy, and reduction in a subcutaneous tissue, fat, and cartilage were observed. Tooth lacerations may have been caused by pressure of muscles and soft tissue in the alveolar bone. |