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Título

Myelitis as the first neuropsychiatric manifestation in a systemic lupus erythematosus patient – rare case report

RESUMO

CASE REPORT: M.C.J.S., 30 years old, female, diagnosed with Systemic Lupus Erythematosus (SLE) at the age of 20, presenting, over 10 years, cutaneous, joint, hematological and renal activity. During this period, she received treatment with hydroxychloroquine and prednisone. In June 2021, she started a prodromal condition flu-like associated with paresthesias, distal weakness in the lower limbs and urinary incontinence. After 48 hours, she evolved with worsening of the legs weakness, being unable to stand and walk without assistance, and in less than 24 hours progressed to flaccid tetraparesis. Analysis of the cerebrospinal fluid showed pleocytosis with lymphocytic predominance, hyperproteinorraquia and normal levels of glucose and lactate faced with the suspicion of autoimmune inflammatory myelopathy. In addition, we noticed a high value of anti-double-stranded DNA antibody 400 U/ml, indicating high disease activity. Magnetic resonance evidenced hypersignal lesions, with a diffuse and irregular distribution, longitudinally from the medulla oblongata transition to the level of the conus medullaris, with a slight swelling effect and without contrast enhancement. She was treated with high-dose intravenous methylprednisolone with little initial improvement, showing a better therapeutic response after plasmapheresis. During follow-up, 6 months later, she was able to walk unassisted, maintained temporary treatment with cyclophosphamide, but due to the potential nephrotoxic damage, she started using rituximab with excellent results. As additional data, anti-aquaporin-4 antibody (AQP4) was measured with a negative result.

DISCUSSION: the occurrence of myelitis in SLE is estimated at 1.2%. In cases where signs of gray matter predominate, high disease activity was observed by high levels of anti-double-stranded DNA antibodies, while when white matter disorders predominate there is an association with anti-AQP4 positivity, indicating, in fact, a great possibility of overlap with neuromyelitis optica spectrum disorder (NMOSD).

FINAL COMENTS: although uncommon, promptly diagnosing and treating SLE myelitis can prevent severe neurological damage. Also, it is important to pay attention to the differential diagnosis with other autoimmune demylinating diseases such as NMOSD, which can coexist with SLE and be responsible for the involvement of the spinal cord and not the activity of the underlying systemic disease.

Palavras Chave

Myelitis; Systemic Lupus Erythematosus