Concurrent Gullain-Barre Syndrome and Acute Transverse Myelitis as an Initial Presentation of Systemic Lupus Erythematosus

길랑 바레 증후군과 횡단척수염으로 발현한 전신성 홍반성 루프스 1례

전신성 홍반성 루프스는 전신을 침범하는 자가 면역 질환으로서, 중추 신경과 말초 신경을 침범하는 질환이 동반될 수 있다. 대부분의 증례에서 두통 및 인지 장애와 같은 질환들이 보고 되고 있다. 본 저자들은 길랑 바레 증후군과 횡단성 척수염이 전신성 홍반성 루프스의 첫 증상으로 발현한 1례를 경험하였기에 보고하는 바이다.

Systemic lupus erythematosus (SLE) is an autoimmune disorder involving multiple organs. Neuropsychiatric symptoms are frequently associated in SLE, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contains both central and peripheral nervous systems, which includes transverse myelitis, and Guillain-Barre syndrome (GBS). We report our experience of concurrent manifestation of transverse myelitis and GBS as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 14-year-old boy with SLE who first presented with features of GBS. The patient developed ascending weakness starting from low extremities, experienced difficulty voiding, and had a facial rash. An initial diagnosis of GBS was made on the basis of clinical findings and nerve conduction studies. But he did not respond to intravenous immunoglobulin therapy and following spine MRI displayed T2 weighted high signal intensities from the cervical to thoracic region of the spinal cord, and serological analysis revealed the presence of antidsDNA, anti-smAb, anti nuclear antibody with decreased level of complements. The diagnosis was revised to GBS and acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. This was followed by oral prednisolone and cyclophosphamide pulse therapy. This is the first case of concurrent manifestation of GBS and transverse myelitis as initial presentation of SLE. The cross-reactivity of autoantibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Clinicians should consider a diagnosis of SLE as an etiology of GBS or transverse myelitis.