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帕里-龙贝格综合征与颞叶难治性癫痫:病例报告

Parry-Romberg Syndrome and Temporal Lobe Refractory Epilepsy: Case Report.

作者信息

Velandia-Martínez A, Ortega-Sanchez M A, Martínez-Perez S O, Peña I, Pradilla O, Gomez R, Martínez-Perez A

机构信息

Department of Neurology, Carlos Ardila Lulle Medical Center, Santander, Colombia.

Department of Dermatology, Carlos Ardila Lulle Medical Center, Santander, Colombia.

出版信息

J Epilepsy Res. 2019 Dec 31;9(2):157-160. doi: 10.14581/jer.19020. eCollection 2019 Dec.

Abstract

The Parry-Romberg syndrome (PRS), also known as hemifacial atrophy, is a rare neurocutaneous disease with the prevalence of 1/700,000 cases. It is more common in women than men, with an early onset of disease usually within the first two decades of life. Even though the etiology of PRS is unknown, it is thought to be a multifactorial disease that involves hereditary, posttraumatic, autoimmune, infectious, and neoplastic factors. There are a variety of systemic manifestations described in PRS including neurological conditions that range from intractable headache to refractory epilepsy. The manifestations must be identified in a timely manner to ensure an early therapeutic intervention, considering that an appropriate approach during the initial phase might halt the disease progression and markedly improve the quality of life in these patients. This article is aimed to describe the case of a 23 years old female with left hemifacial atrophy and dermatologic, dental, and neurologic compromise, associated with refractory temporal lobe epilepsy evidenced in neuroimaging and electrodiagnostic testings.

摘要

帕里-罗姆伯格综合征(PRS),又称半侧面部萎缩症,是一种罕见的神经皮肤疾病,患病率为1/700,000。女性比男性更常见,疾病通常在生命的头二十年早期发病。尽管PRS的病因尚不清楚,但它被认为是一种多因素疾病,涉及遗传、创伤后、自身免疫、感染和肿瘤因素。PRS有多种全身表现,包括从顽固性头痛到难治性癫痫等神经系统疾病。考虑到在初始阶段采取适当的方法可能会阻止疾病进展并显著改善这些患者的生活质量,必须及时识别这些表现以确保早期治疗干预。本文旨在描述一名23岁女性的病例,该患者患有左侧面部萎缩以及皮肤、牙齿和神经功能受损,并伴有神经影像学和电诊断检查证实的难治性颞叶癫痫。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8ae/7251345/db13c488b517/jer-19020f1.jpg

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