Humans ; Lupus Vasculitis, Central Nervous System/pathology* ; Magnetic Resonance Imaging/methods* ; Diffusion Tensor Imaging/methods* ; Patients ; Lupus Erythematosus, Systemic/pathology* ; Brain/pathology*

BACKGROUNDConventional magnetic resonance imaging (MRI) is the preferred neuroimaging method in the evaluation of neuropsychiatric systemic lupus erythematosus (NPSLE). The purpose of this study was to investigate the association between clinical and immunological features with MRI abnormalities in female patients with NPSLE, to screen for the value of conventional MRI in NPSLE.

METHODSA total of 59 female NPSLE patients with conventional MRI examinations were enrolled in this retrospective study. All patients were classified into different groups according to MRI abnormalities. Both clinical and immunological features were compared between MRI abnormal and normal groups. One-way analysis of variance was used to compare the systemic lupus erythematosus disease activity index (SLEDAI) score for MRI abnormalities. Multivariate logistic regression analysis investigated the correlation between immunological features, neuropsychiatric manifestations, and MRI abnormalities.

RESULTSThirty-six NPSLE patients (61%) showed a variety of MRI abnormalities. There were statistically significant differences in SLEDAI scores (P < 0.001), incidence of neurologic disorders (P = 0.001), levels of 24-h proteinuria (P = 0.001) and immunoglobulin M (P = 0.004), and incidence of acute confusional state (P = 0.002), cerebrovascular disease (P = 0.004), and seizure disorder (P = 0.028) between MRI abnormal and normal groups. In the MRI abnormal group, SLEDAI scores for cerebral atrophy (CA), cortex involvement, and restricted diffusion (RD) were much higher than in the MRI normal group (P < 0.001, P = 0.002, P = 0.038, respectively). Statistically significant positive correlations between seizure disorder and cortex involvement (odds ratio [OR] = 14.90; 95% confidence interval [CI], 1.50-151.70; P = 0.023) and cerebrovascular disease and infratentorial involvement (OR = 10.00; 95% CI, 1.70-60.00; P = 0.012) were found.

CONCLUSIONSMRI abnormalities in NPSLE, especially CA, cortex involvement, and RD might be markers of high systemic lupus erythematosus activity. Some MRI abnormalities might correspond to neuropsychiatric manifestations and might be helpful in understanding the pathophysiology of NPSLE.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Lupus Erythematosus, Systemic ; immunology ; pathology ; Lupus Vasculitis, Central Nervous System ; immunology ; pathology ; Magnetic Resonance Imaging ; Middle Aged ; Retrospective Studies

Pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying Iesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. Pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in Korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. Headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache.
Adult ; Case Report ; Female ; Headache/etiology/pathology ; Human ; Lupus Vasculitis, Central Nervous System/*complications/pathology ; Magnetic Resonance Imaging ; Pseudotumor Cerebri/*etiology/pathology ; Recurrence

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.
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