Department of Neurology, San Raffaele Scientific Institute, via Olgettina 48, 20132 Milan, Italy.
J Clin Neurosci. 2013 Sep;20(9):1320-2. doi: 10.1016/j.jocn.2012.08.020. Epub 2013 Mar 22.
Parry Romberg Syndrome (PRS) is a rare condition of unknown cause and pathophysiology. It is characterized by progressive facial hemiatrophy, and neurological abnormalities are found in 20% of cases. We describe a 50-year-old woman with PRS and severe neurological involvement (lateralised epileptic seizure activity and facial pain refractory to medication). Pain intensity and frequency was reduced and control of epileptic crises was improved using levetiracetam as an additional therapy. In previous published cases associated with facial pain, the most frequent diagnoses were migraine and trigeminal neuralgia. Our findings suggest that in this patient PRS-related persistent pain has peculiar features possibly attributed to the underlying musculoskeletal abnormalities.
帕里-罗伯格综合征(PRS)是一种病因和病理生理学不明的罕见疾病。其特征为进行性面偏侧萎缩,20%的病例存在神经学异常。我们描述了一例 50 岁 PRS 伴严重神经受累(偏侧癫痫发作活动和药物难治性面部疼痛)的女性患者。加用左乙拉西坦作为辅助治疗后,疼痛强度和频率降低,癫痫发作得到控制。在既往与面部疼痛相关的已发表病例中,最常见的诊断是偏头痛和三叉神经痛。我们的发现提示,在本例患者中,PRS 相关持续性疼痛具有特殊特征,可能与潜在的肌肉骨骼异常有关。