Bosman T, Van Bei Jnum J, Van Walderveen M A A, Brouwer P A
Department of Neurology, Leiden University Medical Centre; Leiden, The Netherlands -
Interv Neuroradiol. 2009 Jul 29;15(2):165-73. doi: 10.1177/159101990901500205. Epub 2009 Sep 1.
Parry-Romberg syndrome (PRS) is a rare acquired syndrome consisting of progressive hemiatrophy of the face.We present a child with PRS and progressive neurological deficit caused by a giant intracranial aneurysm and reviewed the literature concerning all intracranial abnormalities in patients with PRS.A literature search identified 27 articles reporting on 88 patients ith PRS and intracranial abnormalities. Ipsilateral brain calcification and hemiatrophy are the most prominent features on CT scan and hyperintense white matter lesions are most frequently seen on T2-weighted MRI. Although lacking precise prevalence data, intracranial abnormalities are not uncommon in patients with PRS. We found three other PRS patients with intracranial aneurysms. Our case and literature search suggests a possible association between PRS and intracranial aneurysms. We consider this association important for clinical practice and recommend including intracranial vascular diseases in the differential diagnosis when dealing with a PRS patient with neurological symptoms.
帕里-罗姆伯格综合征(PRS)是一种罕见的获得性综合征,其特征为面部进行性半侧萎缩。我们报告了一名患有PRS且因巨大颅内动脉瘤导致进行性神经功能缺损的儿童,并回顾了有关PRS患者颅内所有异常情况的文献。文献检索确定了27篇报道88例患有PRS及颅内异常患者的文章。同侧脑钙化和半侧萎缩是CT扫描上最突出的特征,而在T2加权磁共振成像(MRI)上最常出现高信号白质病变。尽管缺乏精确的患病率数据,但颅内异常在PRS患者中并不少见。我们发现另外3例患有颅内动脉瘤的PRS患者。我们的病例及文献检索表明PRS与颅内动脉瘤之间可能存在关联。我们认为这种关联对临床实践很重要,并建议在处理有神经症状的PRS患者时,将颅内血管疾病纳入鉴别诊断。