Jain Rajendra Singh, Kumar Sunil, Srivastava Trilochan
Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.
Department of Neurology, Shri Ram Murti Smarak Institute of Medical Sciences (SRMS IMS), Bareilly, Uttar Pradesh, India.
Oxf Med Case Reports. 2016 Aug 29;2016(8):omw031. doi: 10.1093/omcr/omw031. eCollection 2016 Aug.
Parry-Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up.
帕里-罗姆伯格综合征（PRS）的特征是皮肤、皮下结缔组织、肌肉和骨骼进行性退变和萎缩。典型情况下，PRS局限于单侧面部，但20%的患者可能会扩展到身体其他部位，包括同侧或对侧的手臂、躯干和腿部。我们报告一例24岁男性患者，隐匿起病，右下肢逐渐进行性畸形和肌肉萎缩，随后右侧面部和胸部出现症状，病程8年。右侧面部表现为半侧萎缩、剑状痕，鼻子和下巴向同侧偏斜。磁共振成像显示右下肢萎缩。三维面部重建计算机断层扫描显示右侧面部骨骼萎缩。对其进行了物理治疗和对症治疗，并计划在随访时进行面部和踝关节重建手术。
