scholarly journals Progressive facial hemiatrophy (Parry-Romberg syndrome): short case report

2019 ◽  
Vol 25 (3) ◽  
pp. 28
Author(s):  
Alexandre Puidupin ◽  
Aline Desoutter ◽  
Sophie Deneuve ◽  
Lara Nokovitch ◽  
Anne-Gaëlle Chaux-Bodard

Introduction: The Parry Romberg syndrome (PRS) is a mosaic disease of unknown aetiology which mostly affects women. The facial hemiatrophy generally begins during the early childhood. It has a great impact on social life, and aesthetic rehabilitation is a major challenge. Observation: A 38 years-old-female patient presented with an enucleation of the left eye, due to multiple hamartomas and progressive facial hemiatrophy. The placement of 2 extraoral implants was proposed to bear an ocular epithesis. Comments: PRS develops between the 2nd and 20th year of life, and stabilizes at the adult age. Its main characteristic is a progressive facial hemiatrophy, which involves skin, fat tissues, muscles and osteocartilaginous tissues. 20% of patients have neurological symptoms, and epilepsy is often described as a side effect of the disease. Conclusion: Diagnosis of localized scleroderma has to be eliminated, as these 2 entities are very similar. Major facial reconstructive surgery is often proposed to restore aesthetics.

2016 ◽  
Vol 25 (4) ◽  
pp. 209-211
Author(s):  
Alexis-Virgil Cochino ◽  
◽  
Sigrid Covaci ◽  
Oana-Maria Farkas ◽  
◽  
...  

Parry-Romberg syndrome, which is also known as progressive facial hemiatrophy, is a variant of localized scleroderma. This is a rare condition (OMIM # 141300), characterized by a progressive but self-limited atrophy of the skin and subcutaneous tissue on one side of the face. We present the case of a 9 year old boy with a typical presentation of hemifacial atrophy, with Borrelia burgdorferi infection without any changes of the nervous system.


2015 ◽  
Vol 14 (3) ◽  
pp. 308-309
Author(s):  
Ramakrishna Beemanapalli ◽  
Dharma Rao Vanamali ◽  
Adapala Hanumaiah ◽  
Bande Sujeeth Reddy

Parry Romberg syndrome is a rare neurocutaneous disorder of unknown etiology characterized by facial hemiatrophy of fat, skin, connective tissue and MRI changes in the brain. Here we report a 30 years old woman who presented with facial hemiatrophy, headache and facial pain, hemi-masticatory spasm, atrophy of tongue, temporal hemianopia and hyperintense lesions on MRI.Bangladesh Journal of Medical Science Vol.14(3) 2015 p.308-309


2021 ◽  
Vol 9 ◽  
pp. 2050313X2110343
Author(s):  
Andrea N Clapp ◽  
Anna DePold Hohler

This case report describes a 46-year-old female with a history of multiple endocrine neoplasia type 1 syndrome status post-parathyroidectomy, thymectomy via robotic video-assisted thoracoscopic surgery, and pituitary adenoma resection presenting with parasympathetic hyperactivity and Parry–Romberg syndrome. Parry–Romberg syndrome is a rare disorder that manifests as facial hemiatrophy. Reported symptoms include cognitive dysfunction, olfactory hallucinations, and parasympathetic hyperactivity: miosis of the right eye, anhidrosis, diarrhea, urinary incontinence, bowel incontinence, and orthostatic hypotension. Previous reports have noted associations between Parry–Romberg syndrome and sympathetic hyperactivity and dysregulation. This case report focuses on an association between Parry–Romberg syndrome and unopposed parasympathetic activity.


2021 ◽  
Vol 12 ◽  
pp. 1-6
Author(s):  
Felipe dos Santos Souza ◽  
Marco Orsini ◽  
Gilberto Canedo Martins Jr ◽  
Marcelo Namen ◽  
Antonio Carlos Garcia Dias Mayall ◽  
...  

2021 ◽  
Vol 5 (10) ◽  
pp. 668-673
Author(s):  
T.V. Matveeva ◽  
◽  
A.Yu. Kazantsev ◽  
A.B. Aleskerova ◽  
R.T. Gaifutdinov ◽  
...  

The most common cause of neurovascular conflict (NVC) is the compression of the sensory portion of the trigeminal nerve root by artery or vein directly at the brainstem. This results in local demyelination manifested as the classic trigeminal neuralgia. The motor portion of the trigeminal nerve is affected less commonly. The authors describe a case history of a 26-year-old man with a verified NVC of the motor portion of the trigeminal nerve. At the onset, the disease manifested as intense pain paroxysms in the left side of the face preceded by a masseter muscle spasm that was accompanied by lockjaw and tongue biting (hemimasticatory spasm). Shortly after disease onset, facial hemiatrophy with local scleroderma developed. The diagnosis of NVC with the compression of the motor portion of the trigeminal nerve accompanied by hemimasticatory spasm and Parry-Romberg syndrome was verified by brain FIESTA MRI and needle EMG. Later on, these symptoms spontaneously regressed. Concepts of hemimasticatory spasm and facial hemiatrophy are discussed. The rarity of this condition, lack of information on the compression of the motor portion of the trigeminal nerve in NVC, unusual disease onset, and presentations make this issue interesting for a wide range of experts. KEYWORDS: neurovascular conflict, hemimasticatory spasm, facial hemiatrophy, Parry-Romberg syndrome, trigeminal neuralgia, trigeminal nerve, local scleroderma, oromandibular dystonia. FOR CITATION: Matveeva T.V., Kazantsev A.Yu., Aleskerova A.B., Gaifutdinov R.T. Neurovascular conflict with the compression of the motor portion of the trigeminal nerve: hemimasticatory spasm and Parry-Romberg syndrome. Case report. Russian Medical Inquiry. 2021;5(10):668–673 (in Russ.). DOI: 10.32364/2587-6821-2021-5-10-668-673.


2013 ◽  
Vol 4 (2) ◽  
pp. 68-71
Author(s):  
Ehsanul Haque Khan ◽  
MA Rashid ◽  
Taslima Hoq Moonmoon ◽  
Monzur Ahmed ◽  
Bidoura Tanim

Parry-Romberg Syndrome is an uncommon, degenerative and poorly understood condition. It is characterized by a slow and progressive atrophy affecting one side of the face. Possible factors that are involved in the pathogenesis include trauma, viral infections, heredity, endocrine disturbances and auto-immunity. Characteristically the atrophy progresses slowly for several years and soon after it become stable. In this case report, the patient of 8 years old girl complaints of gradual wasting of left side of face for 1 year involving cheek, chin, pre-auricular area, forehead, left upper lip which reveals facial asymmetry and mild wasting of left side of face as well as tongue. There was focal alopecia and depression over left side of scalp. After four months back, there was a hypopigmented area over malar prominence of same side. She was diagnosed as Parry Romberg syndrome on the basis of history, clinical examination and investigation. Antinuclear antibody and Anti-Scl-70 antibody were positive. Histopathological diagnosis was compatible with localized scleroderma. The patient was treated with drugs, physical therapy including electric stimulation as well as exercise. DOI: http://dx.doi.org/10.3329/jssmc.v4i2.14424 J Shaheed Suhrawardy Med Coll, 2012;4(2):68-71


Author(s):  
Bahram Alamdary Badlou

We report a rare case of unrepaired Tetralogy_Pantalogy of Fallot (TOF_POF) in a 20 years old Persian girl Mrs Zeynab S., who presented with cyanotic finger tops appearance, ongoing chronic thrombolytic destruction processes, and remarkable thrombocytopenia [1,2], heart ventricular septal defect (VSD), and might atrial septal defect (ASD), anxiety, sleep disorders, nightmares, and limited social life. Additionally, the relationship between underlying mechanisms, possible treatments of the thrombocytopenia, erythrocytosis, and unrepaired cardiovascular leakages remains unknown.


Author(s):  
Giacomo Evangelista ◽  
Fedele Dono ◽  
Claudia Carrarini ◽  
Mirella Russo ◽  
Laura Bonanni

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