Melkersson-rosenthal syndrome complicated with facial hemiespasm and intracranial hypertension

Since it was first described in 1928 by neurologist Ernest Melkersson11, and complemented in 1931 by Curt Rosenthal12, the Melkersson-Rosenthal Syndrome (SMR) has been shown to be a rare disorder and still in need of specific treatment13,14. We describe the case of a young patient, who at 23 years old had the first episode of peripheral facial paralysis on the right. At the time, it was conducted in a protocol manner, with prednisone, paracetamol and eye care, as well as pertinent physiotherapy. There was an improvement in facial asymmetry, when in 2006 she presented a new peripheral facial paralysis, this time on the left, which was conducted in a similar way. Then, she presented 2 more episodes of facial paralysis in the interval of 3 years, already with aesthetic sequelae. In 2009, in the 5th episode of paralysis, she associated facial edema and cheilitis, when biopsy of the upper eyelid was requested, which was unspecific. In 2012, after the 8th facial paralysis, she underwent a new biopsy, in which an inflammatory infiltrate was demonstrated, consistent with clinical suspicion of SMR. Imaging exams (skull resonance and angioresonance), face electroneuromyography - for prognosis, and CSF study, without abnormalities, were performed. That year she began to experience involuntary and rhythmic contractions of the orbicularis musculature of the eyes and mouth on the right, in addition to risory and platysma, featuring facial hemispasm, treated every 4 to 6 months with 100 IU Onabotulinum. Throughout the treatment period, alternating use of 10mg prednisone with deflazacort 6mg, and in 2019, due to persistent headache, she underwent a new resonance with arterial and venous cranial angioresonance, which brought elements suggestive of Intracranial Hypertension (IH). Currently, the patient is undergoing surgical programming for facial nerve decompression bilaterally and undergoing treatment for IH.