Longitudinally Extensive Transverse Myelitis Revealing Systemic Lupus Erythematosus: A Case Report and Literature Review

Abstract

Introduction: The American College of Rheumatology recognizes 19 main forms of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). Acute confusional states, cerebrovascular disease, peripheral neuropathies and seizure are the most common. We report a rare case of SLE-related myelopathy in teenager girl. Case report: 16 years-old teenager girl, with no previous health history, presented 8 days before her admission, rapidly progressive flaccid tetra paresis with paresthesia and sphincter disturbance. These symptoms were associated to polyarthritis and facial lupus discoid lesion. Spinal MRI objectified Longitudinal Extensive Transverse Myelitis (LETM) to more than 12 vertebral segments. Cerebrospinal Fluid (CSF) showed normal cytology with mild high protein level (0.8 g/l). Immunologic tests went positive to anti-nuclear and anti-DNA antibodies, with negative anti-aquaporin 4 and anti-MOG antibodies. Several infectious serologies went negative. The diagnosis of acute LETM secondary to NPSLE was established. The patient was treated by bolus methylprednisolone and cyclphosphamide, relayed by oral prednisolone. The course was favorable with clinical and radiological improvement, apart from sequelar spinal atrophy on the MRI. Discussion: Neuropsychiatric Systemic Lupus Erythematosus (NP SLE) is one of the most challenging forms of SLE, as one-third of patient with NPSLE are primary manifestation of lupus autoimmunity. Lupus myelitis is occurring in 1 to 2% of SLE patients. In above 22% of reported cases, LETM was the revealing syndrome of SLE. Several differential diagnoses cause LETM, and must be eliminated according to clinical peculiarities and adequate para clinical tests (infection, neuromyelitis optica spectrum disorders, Sjogren’s syndrome, paraneoplastic syndrome). Early methyprednisolone bolus and cyclophosphamid have shown better prognosis with sensori-motor and sphincter improvement. Conclusion: LETM may be the initial presentation of NPSLE. The absence of other systemic symptoms makes the diagnosis a real challenge. This went to procession of clinical and biological arguments. Randomized studies should be led, to report clear guidelines concerning therapies and prognosis.