Comparison of the clinical manifestations, brain MRI and prognosis between neuroBeçhet's disease and neuropsychiatric lupus.
BACKGROUND
Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBeçhet 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.


CONCLUSIONS
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.
Cho
Byung-Sik
BS
Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
Kim
Hyun-Sook
HS
Oh
Su-Jin
SJ
Koh
Hyeok-Jae
HJ
Yoon
Chong-Hyun
CH
Jung
So-Lyung
SL
Min
Do-June
DJ
Kim
Wan-Uk
WU
D000328:Adult; D001528:Behcet Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D020945:Lupus Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors