NEUROMYELITIS OPTICA SPECTRUM DISORDERS (NMOSD) AND SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): DOUBLE DISEASE BURDEN AND DILEMMA
- Anna Abou-Raya, Egypt
Abstract
Background and Aims
Neuromyelitis optica (NMO), neuromyelitis optica spectrum disorders (NMOSD) and transverse myelitis are auto-antibody mediated chronic inflammatory demyelinating CNS diseases that can occur idiopathic or in conjunction or may overlap with autoimmune rheumatic diseases. Although rare, these immune-mediated CNS diseases pose a diagnostic and therapeutic challenge to the treating physician posing a dilemma in clinical practice.
Methods
To illustrate the difficulty and dilemma in distinguishing NPSLE from NMOSD we present a case report of a 42-year-old female with a six-year history of SLE that presented with a 2-day complaint of progressive numbness and flaccid paralysis in both legs, fatigue and a malar rash. She then developed urinary and rectal incontinence and was unable to walk or stand unassisted. Flaccid leg paralysis, lower extremity paresthesia, and a positive Babinski response on the right side were noted on physical exam.The workup included serology for antinuclear, anti-double-stranded, antiphospholipid antibodies, blood count, ESR and CRP, lumbar puncture and MRI of her spine and brain.
Results
Workup revealed strong ANA positivity, NMO-IgG antibody (antibody to aquaporin 4 IgG (AQP4-IgG)) was positive and MRI of the spine demonstrated extensive hyperintense T2 signal abnormality throughout the spinal cord.
Conclusions
This case is further evidence that they can be co-existing conditions and although demyelination can be related to SLE activity it can sometimes be difficult to distinguish NPSLE from NMOSD. Early recognition of NMOSD in patients with autoimmune rheumatic diseases presenting with a CNS event is key.The therapeutic implications of these relationships are important because they can influence the timing and selection of immunosuppressive therapy.