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ALARM BELLS RINGING FOR MELKERSSON-ROSENTHAL SYNDROME?
  1. LT Evans,
  2. MD Willis,
  3. R Corkill
  1. Department of Neurology, University Hospital of Wales

Abstract

Melkersson-Rosenthal Syndrome (MRS) is a rare mucocutaneous disorder, characterised by a clinical triad of facial nerve palsy, facial oedema, and tongue furrowing (lingua plicata). We present the case of a 28 year-old, right handed male who presented to general medicine with two discreet episodes of left facial paralysis occurring over a 4-year period. Both episodes were treated with prednisolone and aciclovir for presumed Bell's palsy. In recent months, the patient had experienced numerous episodes of facial swelling, affecting his upper lip and gums and was subsequently referred to neurology. At presentation, mild left sided facial weakness was noted, with otherwise normal neurological findings. Brain MRI showed enhancement of descending elements of the facial nerves bilaterally. A diagnosis of MRS was made and the patient started on NSAID therapy. Unfortunately, the patient's symptoms have progressed with almost constant facial oedema and tongue furrowing.

MRS is rare but needs to be considered in the differential diagnosis of recurrent facial nerve palsy. In particular, it can be associated with autoimmune diseases such as sarcoidosis and Crohn's disease and both monosymptomatic and oligosymptomatic presentations occur. Definitive diagnosis can be made with a labial biopsy showing non-caseating granulomas but unfortunately treatment is often ineffective.

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