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Parry Romberg Syndrome: The Socially Appalling Yet Less Acknowledged Diagnosis: Case Series and Literature Review

机译:帕里·罗姆伯格综合症:令人震惊的社会诊断却少为人知:病例系列和文献综述

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The dilemma of facing a facial cosmetic issue at a young age is the most troublesome symptom hampering social well-being. Beginning in the teen ages and progressing to variable extents, Parry Romberg Syndrome (PRS) leads to facial asymmetry in the form of hemiatrophy secondary to loss of subcutaneous tissues, often fat but sometimes the underlying muscles and bones. It is indistinctly associated with seizure disorders at times or with other neurological sequelae. The first case was an 18 year old Pakistani male who presented with progressive wasting of the left side of the face for the past two and a half years. The second case was an 18-year-old Pakistani girl who presented to the neurology OPD with primary complaints of a seizure disorder for 3 years. In addition, she had progressive subtle asymmetry of her face. Over the course of time, a faint curvilinear grooved line showed up towards the right side of her forehead reaching on to her head up to the vertex. She also had hair loss ipsilateral to the line. The presentation of above cases depicts that although some features of PRS like facial asymmetry is characteristic, the presentation of a patient primarily with seizures should ideally include a thorough search for a secondary cause or of possible associations so that prognosis can be defined and appropriate interventions planned.
机译:年轻时面临面部美容问题的困境是困扰社会福祉的最麻烦症状。帕里·罗姆贝格综合症(PRS)从十几岁开始,并发展到不同程度,其继发于皮下组织(通常是脂肪,但有时是下层的肌肉和骨骼)丢失的肥大性贫血形式的面部不对称。有时与癫痫发作或其他神经系统后遗症无关。第一例是一名18岁的巴基斯坦男性,在过去的两年半中,其面部左侧逐渐消瘦。第二例是一名18岁的巴基斯坦女孩,在主诉癫痫病的3年中向神经科OPD提出了主诉。此外,她的脸部渐进式微妙不对称。随着时间的流逝,一条淡淡的曲线凹槽线出现在额头的右侧,一直延伸到其头部直至顶点。她在该线的同侧也有脱发。上述病例的描述表明,尽管PRS的某些特征(例如面部不对称)具有特征性,但理想情况下,主要是癫痫发作的患者表现应包括彻底寻找继发原因或可能的关联,以便可以确定预后并计划适当的干预措施。

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