Korean J Intern Med.  2007 Jun;22(2):77-86. 10.3904/kjim.2007.22.2.77.

Comparison of the Clinical Manifestations, Brain MRI and Prognosis between NeuroBehcet's Disease and Neuropsychiatric Lupus

• Cho BS
• Kim HS
• Oh SJ
• Ko HJ
• Yoon CH
• Jung SL
• Min DJ
• Kim WU

• KMID: 1091866
• DOI: http://doi.org/10.3904/kjim.2007.22.2.77

Abstract

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients.
METHODS
Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE.
RESULTS
The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients.
CONCLUSION
NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.

Keyword

Behcet's disease,; Systemic lupus erythematosus; Neurologic involvement; Magnetic resonance image

MeSH Terms

Adult
Behcet Syndrome/complications/*diagnosis/pathology
Brain/*pathology
Female
Humans
Lupus Erythematosus, Systemic/complications/diagnosis/pathology
Lupus Vasculitis, Central Nervous System/complications/*diagnosis/pathology
*Magnetic Resonance Imaging
Male
Prognosis
Retrospective Studies
Risk Factors