Pontine and extrapontine myelinolysis revealing undiagnosed systemic lupus erythematosus: case report and literature review

Background. Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are rare demyelinating disorders most often linked to the rapid correction of hyponatremia, malnutrition, or chronic alcoholism. Their occurrence in autoimmune diseases, particularly systemic lupus erythematosus (SLE), is exceptionally rare. Case presentation. We report the case of a 54-year-old woman who developed both CPM and EPM as the initial manifestations of SLE. She presented with inflammatory arthralgia, lower limb edema, and acute neuropsychiatric symptoms. Laboratory investigations revealed nephrotic-range proteinuria, positive antinuclear antibodies (ANA), positive anti-DNA antibodies, and low complement levels. Magnetic resonance imaging (MRI) demonstrated characteristic findings of both pontine and extrapontine myelinolysis. After ruling out other potential etiologies, she was treated with corticosteroids and cyclophosphamide, leading to both clinical and radiological improvement. Conclusion. This case highlights the rare but significant neurological manifestation of SLE. Early recognition and prompt immunosuppressive treatment can result in favorable outcomes, emphasizing the importance of considering CPM and EPM in SLE patients presenting with neuropsychiatric symptoms.