ALARM BELLS RINGING FOR MELKERSSON-ROSENTHAL SYNDROME?. Issue 10 (9th September 2014)

• Record Type: Journal Article
• Authors: Evans, LT; Willis, MD; Corkill, R
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 85:Issue 10(2014)
• Page Start: e4
• Page End: e4
• Publication Date: 2014-09-09
• Subjects: EPILEPSY; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2014-309236.83
• Languages: English
• ISSNs: 0022-3050

Abstract

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.