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Parry-Romberg syndrome with hemimasticatory spasm in pregnancy; A dystonia mimic

Akhila Kumar Panda1, Godhavarma Gopinath1, Shaily Singh1

  • 1Department of Neurology, Institute of Human Behaviour and Allied Sciences, Delhi, India.

Journal of Neurosciences in Rural Practice
|June 27, 2014
PubMed

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.

Keywords:
Hemimasticatory spasmParry-Romberg syndromehemifacial spasmjaw dystonia

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