J Korean Child Neurol Soc.  2012 Jun;20(2):121-128.

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

• Kang SH
• Yum MS
• Lee EH
• Ko TS

Abstract

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 anti-dsDNA, 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.

Keyword

Guillain-Barre syndrome; Transverse myelitis; Systemic lupus erythematosus

MeSH Terms

Adolescent
Autoantibodies
Complement System Proteins
Cyclophosphamide
Exanthema
Extremities
Guillain-Barre Syndrome
Humans
Immunization, Passive
Lupus Erythematosus, Systemic
Methylprednisolone
Myelitis, Transverse
Neural Conduction
Peripheral Nervous System
Prednisolone
Spinal Cord
Spine
Autoantibodies
Complement System Proteins
Cyclophosphamide
Methylprednisolone
Prednisolone