Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Pediatric Clinic, Maggiore Hospital, ASST Crema, Crema, Italy.
Cephalalgia. 2022 Apr;42(4-5):409-425. doi: 10.1177/03331024211043452. Epub 2021 Sep 26.
Parry-Romberg syndrome is a neuro-cutaneous disease characterized by progressive hemifacial atrophy. Although common, headache in this population is scarcely reported in the literature.
To evaluate the clinical features of headache in pediatric and adult patients with Parry-Romberg syndrome, and to discuss diagnostic and treatment approaches of headache in Parry-Romberg syndrome.
We conducted a systematic review in accordance with PRISMA guidelines. We searched the MEDLINE database to identify eligible studies and identified patients with Parry-Romberg syndrome and headache. We further reported a paradigmatic case with a complex headache disorder and described its management and outcome.
We identified 74 articles, 41 of which were included in the analysis. A total of 52 patients (55.8% female) were included for data analysis. The main age at onset of headache was 20 years (SD 15.2; range 3-56). A diagnosis of migraine was made in 53.9%. Abnormal brain imaging was found in 82.2% of patients.
Long-term follow-up of patients is required, because headache may develop (and evolve) at any time over the course of the disease. Primary and secondary headaches often co-occur in patients with Parry-Romberg syndrome. Further research into the underlying etiopathogenesis and therapeutic targets would be recommended.
进行性半侧颜面萎缩症是一种神经皮肤疾病，其特征为进行性半侧面部萎缩。尽管较为常见，但此类人群的头痛在文献中鲜有报道。
评估儿童和成年 Parry-Romberg 综合征患者头痛的临床特征，并讨论 Parry-Romberg 综合征头痛的诊断和治疗方法。
我们按照 PRISMA 指南进行了系统评价。我们检索了 MEDLINE 数据库以确定符合条件的研究，并确定了患有 Parry-Romberg 综合征和头痛的患者。我们进一步报告了一个具有复杂头痛障碍的范例病例，并描述了其管理和结果。
我们确定了 74 篇文章，其中 41 篇被纳入分析。共有 52 名患者（55.8%为女性）纳入数据分析。头痛的主要发病年龄为 20 岁（标准差 15.2；范围 3-56）。偏头痛的诊断占 53.9%。82.2%的患者存在异常脑影像学表现。
需要对患者进行长期随访，因为头痛可能会在疾病过程中的任何时间（并演变）出现。原发性和继发性头痛在 Parry-Romberg 综合征患者中经常同时发生。建议进一步研究潜在的病因发病机制和治疗靶点。
