Panda Akhila Kumar, Gopinath Godhavarma, Singh Shaily
Department of Neurology, Institute of Human Behaviour and Allied Sciences, Delhi, India.
J Neurosci Rural Pract. 2014 Apr;5(2):184-6. doi: 10.4103/0976-3147.131675.
Parry-Romberg syndrome (PRS) with hemimasticatory spasm (HMS) is quite an uncommon overlapping phenomenon which very often mimics jaw closing dystonia. A previously healthy 35-year-old female, during her 5(th) month of pregnancy started developing intermittent unilateral painful spasms of jaw while conversation, clinching of teeth, or eating, which led to frequent tongue bites. The spasms were worsened during pregnancy. She used to do certain manoeuvre like sensory tricks in form of touching involved side of the face to relieve the symptoms. Apart from this, she developed progressive hemifacial and hemitongue atrophy. Other medical and neurological examinations were normal. Laboratory investigations as well as neuroimaging were noncontributory. The spasm responded to carbamazepine but hemifacial atrophy persists. To our best knowledge, onset and worsening of this syndrome in pregnancy has not been described earlier which might be correlated either with some hormonal imbalance or some unknown mechanisms.
伴有偏侧咀嚼肌痉挛（HMS）的帕里-龙贝格综合征（PRS）是一种非常罕见的重叠现象，常常酷似闭口性肌张力障碍。一名既往健康的35岁女性，在怀孕第5个月时开始出现间歇性单侧下颌疼痛性痉挛，在交谈、咬牙或进食时发作，导致频繁咬伤舌头。痉挛在孕期加重。她会做一些动作，如触摸患侧面部来缓解症状，类似感觉技巧。除此之外，她还出现了进行性半侧面部和半侧舌萎缩。其他医学和神经系统检查均正常。实验室检查及神经影像学检查均无异常发现。痉挛对卡马西平有反应，但半侧面部萎缩持续存在。据我们所知，此前尚未有该综合征在孕期发病及病情加重的报道，这可能与某些激素失衡或一些未知机制有关。
