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帕里-罗姆伯格综合征:一例病例报告及讨论

Parry Romberg syndrome: A case report and discussion.

作者信息

Madasamy Ramasamy, Jayanandan Muruganandhan, Adhavan Uma Revathy, Gopalakrishnan Sivakumar, Mahendra Lodd

机构信息

Department of Orthodontics, Sri Venkateswara Dental College and Hospital, Chennai, Tamil Nadu, India.

出版信息

J Oral Maxillofac Pathol. 2012 Sep;16(3):406-10. doi: 10.4103/0973-029X.102498.

Abstract

Parry Romberg syndrome is a rare disorder of unknown etiology, seen most commonly as an asymmetry of the face, rarely affecting the limbs. Trophic malfunction of sympathetic system has been proposed as a cause. The syndrome presents with characteristic skeletal, dental, and soft tissue changes in the affected half of the face, with or without neurological signs and symptoms. Imaging studies sometimes reveal lesions in the brain corresponding with the neurological defects. The disfiguring nature of the disease Results in psychological disturbance and communication disorders like speech defects, as also dental anomalies. The present article reports such a case of an 8-year-old girl who presented with mainly hard tissue changes, without neurological or intraoral soft tissue changes. There has to be prompt multi-disciplinary management of such cases keeping in mind development, aesthetics, speech, and masticatory function, along with symptomatic treatment of neurological deficits.

摘要

帕里-龙贝格综合征是一种病因不明的罕见疾病,最常见的表现是面部不对称,很少累及四肢。有人提出交感神经系统的营养功能障碍是其病因。该综合征在患侧面部会出现特征性的骨骼、牙齿和软组织变化,伴有或不伴有神经症状和体征。影像学研究有时会发现大脑中与神经缺陷相对应的病变。该疾病的毁容性质会导致心理障碍和沟通障碍,如言语缺陷以及牙齿异常。本文报道了一名8岁女孩的病例,她主要表现为硬组织变化,无神经或口腔内软组织变化。对于此类病例,必须迅速进行多学科管理,同时要考虑到发育、美学、言语和咀嚼功能,以及对神经功能缺损进行对症治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aacc/3519218/8ccdcdb118d5/JOMFP-16-406-g001.jpg

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