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Devic's syndrome as initial presentation of systemic lupus erythematosus
Shabana Karim1, Vikas Majithia
1Division of Rheumatology, Department of Medicine, University of Mississippi Medical Center, Jackson, Mississippi 39216, USA. skarim@medicine.umsmed.edu
The American Journal of the Medical Sciences
|September 12, 2009
Summary
Neuromyelitis optica (NMO), a CNS inflammatory disease, can present alongside autoimmune disorders like lupus. This case highlights diagnostic challenges and management considerations for NMO and coexisting conditions.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Devic's syndrome, or neuromyelitis optica (NMO), is a central nervous system inflammatory demyelinating disease.
- NMO is characterized by optic neuritis, myelitis, and NMO-IgG antibody seropositivity.
Observation:
- A pregnant patient presented with progressive weakness, diplopia, and headache.
- Physical exam revealed neurological deficits, a discoid rash, and laboratory findings suggestive of autoimmune disease.
- MRI confirmed CNS lesions, and NMO antibodies were positive, leading to a diagnosis of NMO with probable SLE.
Findings:
- The patient received pulse IV solumedrol and plasmapheresis, showing clinical improvement.
- Fetal bradycardia developed during maternal treatment and was managed with IV dexamethasone.
- The case underscores the diagnostic complexities when NMO coexists with systemic autoimmune disorders.
Implications:
- The relationship between NMO and autoimmune diseases like SLE remains debated.
- Distinguishing NMO as an independent condition versus a complication of systemic disease is crucial for management.
- Further case studies are needed to clarify diagnostic and therapeutic strategies for these complex cases.
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