Mixed Connective Tissue Disease

Mixed connective tissue disease is characterized by a combination of overlapping features of systemic lupus erythematous, scleroderma, and polymyositis. Typical presentation is with Raynaud's phenomenon, arthralgias, inflammatory myopathy, lymphadenitis, skin or mucosal lesions, and serositis. A key distinguishing factor of the disorder is a high titer of antibody to ribonucleoprotein, a finding absent in any of these three disorders (SLE, scleroderma, and polymyositis). Neurologic dysfunction is present in approximately 10% to 15% of cases, usually presenting with facial pain, facial paresthesias, or aseptic meningitis (96,97). Facial nerve involvement, although far less common than trigeminal nerve involvement, has been described and is felt to be an early manifestation of the disorder (98). Although CSF analysis has suggested an inflammatory involvement of the cranial nerves at the meningeal level, there remains an absence of direct evidence for the neurologic dysfunction found in these patients. Current theories implicate a vasculitis as the mechanism of neural injury (99). The prognosis for the disease is quite poor without treatment, but immunosuppressive therapy such as corticosteroids has a dramatic efficacy. Case reports have shown complete facial nerve recovery after corticosteroid treatment (99).

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