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Título

Melkersson-Rosenthal syndrome: a case report

RESUMO

Case presentation: A 44-years-old woman presented to our service due repeted episodies of right Bell’s palsy. The begin of symptoms was five years ago, with right facial nerve palsy diagnosed as Bell’s plasy and treated with prednisone and aciclovir, showing gradual improvement. One year later, she developed facial disesteshya in right face, associated with right facial edema. In the following 3 years, she had two more episodes of facial nerve palsy, associated with ipsilateral facial edema and tongue fissures, referred to our service. Brain MRI were normal, and laboratory tests showed reagent antinuclear antibody (1/320), with no other biomarkers present and serological tests for the infectious diasease negative. CSF showed 16 cells, 65 proteins, with negative infectious tests. Salivary gland biopsy unchanged. The hypothesis of recurrent facial paralysis due to Melkersson-Rosenthal Syndrome was raised and treatment with corticosteroids (prednisone 60mg per day) for two weeks was performed, without recorrence of symptoms.
Discussion: Melkersson–Rosenthal syndrome (MRS) is a rare syndrome with unclear etiology, comprised of the triad of recurrent facial nerve paralysis, nonpitting orofacial edema and fissured tongue (lingua plicata. All the three features are present in 20–75% of cases. Other neurological manifestations may be seen in MRS, like migraine, facial paresthesias, tinnitus, dizziness, hypogeusia and pharyngeal neuralgias. Non-neurological symptoms include uveitis, diverticulitis, and ulcerative colitis.
The investigation must be exclude systemic pathologies, mainly sarcoidosis, Crohn's disease, tuberculosis, herpes and other infectious diseases. Biopsy of a region with orofacial edema demonstrating noncaseating granulomas supports the diagnosis of MRS.
There is no specific treatment for MRS. Therapy with corticosteroids leads to improvement in 50–80% of patients and reduced relapse frequency by 60–75%. However, randomized trials are lacking and frequency, dosing or duration are not established. Other options described are NSAIDs, antibiotics (like doxycycline and dapsone) and immunosuppressants (methotrexate, infliximab). Intralesional triamcinolone acetonide injection also are used in local edema.
Final comments: MRS is a cause of recurrent facial nerve palsy, and etiologies such as multiple sclerosis and infections should be investigated. In idiopathic cases, steroids are often used. More studies are needed to understand and manage these cases.

Palavras Chave

Melkersson-Rosenthal syndrome; granulomatosis disease; facial nerve palsy; orofacial edema; fissured tongue.