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Hemiatrophy parry romberg

Klara Uhrhan, Monika Rabenstein, Carsten Kobe, Alexander Drzezga, Gereon R Fink, Lothar Burghaus
Parry-Romberg syndrome is a rare disorder characterized by a progressive facial hemiatrophy of the skin, subcutaneous tissue, musculature, bone, and cartilage. It is often associated with neurological symptoms such as trigeminal neuropathy, paresthesia of the face, migraine, and seizures and can be paired with ocular problems and ipsilateral progressive body atrophy. Here, we present a young woman with progressive facial hemiatrophy, who was referred for FDG-PET/CT. Hypometabolism was observed in the left cingulate and postcentral gyrus, left cerebellum, and right basal ganglia...
May 2017: Clinical Nuclear Medicine
Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...
September 30, 2016: Neurology International
Rakesh Gupta, Harshad Patil
Parry-Romberg syndrome (PRS) is a rare, poorly understood degenerative condition characterized by atrophic changes affecting one side of the face. The cause of these changes remains obscure. Migraine and facial pain such as trigeminal neuralgia are the most common neurological symptoms in this patient group. Sometimes, it causes epilepsy and rarely cerebral hemiatrophy, meningeal thickening, cortical dysgenesis, calcified lesions, aneurysms, and intracranial vascular malformations. Herein, the author reports the first case of PRS with multiple large intracranial cysts producing raised intracranial pressure...
April 2016: Journal of Pediatric Neurosciences
Elzbieta Klimiec, Aleksandra Klimkowicz-Mrowiec
Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry-Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment...
2016: Neurologia i Neurochirurgia Polska
Yun-Jin Lee, Kee-Yang Chung, Hoon-Chul Kang, Heung Dong Kim, Joon Soo Lee
Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied...
September 2015: Korean Journal of Pediatrics
Hisashi Nomura, Shohei Egami, Tomoaki Yokoyama, Makoto Sugiura
A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.
2015: Case Reports in Dermatological Medicine
Viviana Gómez Ortega, Daniel Sastoque
UNLABELLED: Parry-Romberg syndrome (PRS) is an infrequent, acquired disorder characterized by progressive hemiatrophy of the skin and soft tissue of the face and, in some patients, results in atrophy of muscles, cartilage, and the underlying bony structures.The disease process exhibits varying speeds of development, with onset occurring in early infancy or adolescence. Clinicians have classically reserved treatment until the end of the process. Various treatment modalities have been attempted with differing results...
September 2015: Journal of Craniofacial Surgery
M Wong, C D Phillips, M Hagiwara, D R Shatzkes
Parry Romberg syndrome is a rare progressive hemiatrophy of the face that typically occurs in children and young adults and has a peculiar progression that ceases without apparent cause after a highly variable period. Only a subset of patients with Parry Romberg syndrome will develop secondary neurologic or ophthalmologic symptoms, and prognosis is highly variable. Inconsistency in the pattern of atrophy and the development of associated symptoms in patients with Parry Romberg syndrome has made it challenging to diagnose, prognosticate, and treat...
July 2015: AJNR. American Journal of Neuroradiology
Stanislav N Tolkachjov, Nirav G Patel, Megha M Tollefson
BACKGROUND: Progressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a spectrum (; Adv Exp Med Biol 455:101-4, 1999; J Eur Acad Dermatol Venereol 19:403-4, 2005)...
April 1, 2015: Orphanet Journal of Rare Diseases
Nader A Al-Aizari, Saleh Nasser Azzeghaiby, Hashem Motahir Al-Shamiri, Shourouk Darwish, Bassel Tarakji
Parry-Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS...
April 2015: Avicenna Journal of Medicine
Mohsin Khan, Mubeen Khan, Raju Negi, Nikita Gupta
Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome remains unclear. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The syndrome overlaps with "en coup de sabre" morphea, with an ill defined relationship existing between the two...
July 2014: Journal of Clinical and Experimental Dentistry
Zhao Jianhui, Yi Chenggang, Lu Binglun, Han Yan, Yang Li, Ma Xianjie, Su Yingjun, Guo Shuzhong
BACKGROUND: Progressive facial hemiatrophy, also called Parry-Romberg syndrome (PRS), is characterized by slowly progressive atrophy of one side of the face and primarily involves the subcutaneous tissue and fat. The restoration of facial contour and symmetry in patients affected by PRS still remains a challenge clinically. Fat graft is a promising treatment but has some shortcomings, such as unpredictability and low rate of graft survival due to partial necrosis. To obviate these disadvantages, fat graft assisted by bone marrow-derived mesenchymal stem cells (BMSCs) was used to treat PRS patients and the outcome was evaluated in comparison with the conventional treatment by autologous fat graft...
September 2014: Annals of Plastic Surgery
Marta Janowska, Katarzyna Podolec, Sylwia Lipko-Godlewska, Anna Wojas-Pelc
Parry-Romberg syndrome or progressive facial hemiatrophy was first described by Caleb Parry in 1825 and Moritz Romberg in 1846. This disorder is characterized by slowly progressing acquired unilateral hemifacial atrophy, which affects subcutaneous tissue together with the muscles and underlying bones. The pathogenesis and precise incidence of the syndrome remain unclear. Immune-mediated processes and disturbed central regulation, leading to the hyperactivity of the sympathetic nervous system, are primarily considered in the pathogenesis of this disorder...
December 2013: Postȩpy Dermatologii i Alergologii
Paolo Scolozzi, Georges Herzog
Although its pathogenesis remains obscure, Parry-Romberg syndrome (PRS) has been associated with the linear scleroderma en coup de sabre. PRS is characterized by unilateral facial atrophy of the skin, subcutaneous tissue, muscles, and bones with at least 1 dermatome supplied by the trigeminal nerve. Facial asymmetry represents the most common sequela and can involve the soft tissues, craniomaxillofacial skeleton, dentoalveolar area, and temporomandibular joint. Although orthognathic procedures have been reported for skeletal reconstruction, treatment of facial asymmetry has been directed to augmentation of the soft tissue volume on the atrophic side using different recontouring or volumetric augmentation techniques...
May 2014: Journal of Oral and Maxillofacial Surgery
Rajesh Verma, Hari Ram, Mani Gupta, Mukund R Vidhate
Progressive facial atrophy or Parry-Romberg syndrome is characterized by slowly progressive facial atrophy involving skin, subcutaneous tissue, cartilage and bony structures. Apart from facial atrophy, it can be associated with diverse clinical manifestations including headache, partial seizures, trigeminal neuralgia, cerebral hemiatrophy and ocular abnormalities. The exact etiology is unknown although sympathetic system dysfunction, autoimmune disorders, focal scleroderma, trauma and genetic factors have been postulated...
January 2013: National Journal of Maxillofacial Surgery
Cassio Eduardo Raposo-Amaral, Rafael Denadai, Daniella Nunes Camargo, Thiago Olivetti Artioli, Yara Gelmini, Celso Luiz Buzzo, Cesar Augusto Raposo-Amaral
BACKGROUND: The aims of this study were to (1) describe the main clinical findings of Parry-Romberg syndrome (PRS), (2) evaluate surgical strategies and outcomes, and (3) investigate the quality of life of patients according to their disease severity. METHODS: This retrospective observational study involved 14 patients treated between 2005 and 2011. The surgical treatment strategies were based on the proposed system for grading severity, and postoperative outcomes were assessed...
August 2013: Aesthetic Plastic Surgery
Gary E Borodic, Paul Caruso, Martin Acquadro, Sarah Chick
Parry-Romberg syndrome is a rare condition characterized by progressive, hemifacial atrophy, hair loss, enophthalmos, retinal vasculopathy occasionally associated with hemicranial pain syndrome (secondary trigeminal neuralgia). The cause of the condition is unknown; however, substantial evidence suggests that vasculopathy plays a significant role in the genesis of the neurologic damage and facial lipodystrophy. Herein describes a case of Parry-Romberg syndrome treated with repetitive botulinum type A toxin injections, with almost complete resolution of severe chronic pain...
January 2014: Ophthalmic Plastic and Reconstructive Surgery
I Rauscher, D J Loeffelbein, E J Rummeny, M Eiber
No abstract text is available yet for this article.
August 2013: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
Gloria Dalla Costa, Bruno Colombo, Dacia Dalla Libera, Vittorio Martinelli, Giancarlo Comi
Parry Romberg Syndrome (PRS) is a rare condition of unknown cause and pathophysiology. It is characterized by progressive facial hemiatrophy, and neurological abnormalities are found in 20% of cases. We describe a 50-year-old woman with PRS and severe neurological involvement (lateralised epileptic seizure activity and facial pain refractory to medication). Pain intensity and frequency was reduced and control of epileptic crises was improved using levetiracetam as an additional therapy. In previous published cases associated with facial pain, the most frequent diagnoses were migraine and trigeminal neuralgia...
September 2013: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Yanko Castro-Govea, Oscar De La Garza-Pineda, Jorge Lara-Arias, Hernán Chacón-Martínez, Gabriel Mecott-Rivera, Abel Salazar-Lozano, Everardo Valdes-Flores
Progressive facial hemiatrophy, also known as Parry-Romberg syndrome, is a progressive and self-limited deformation of the subcutaneous tissue volume on one side of the face that creates craniofacial asymmetry. We present the case of a patient with a five-year history of progressive right facial hemiatrophy, who underwent facial volumetric restoration using cell-assisted lipotransfer (CAL), which consists of an autologous fat graft enriched with adipose-derived stem cells (ASCs) extracted from the same patient...
November 2012: Archives of Plastic Surgery
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