Background/Purpose: Neurological manifestations in SLE are diverse. Because of its varied manifestations and low prevalence, the ACR has developed nomenclature and case definitions for neuropsychiatric SLE (NPSLE) to facilitate clinical research. Brain MRI has been used for the evaluation of neurologic symptoms. The purpose of this study was to identify characteristic brain MRI findings in NPSLE and to investigate the association between brain MRI findings and NPSLE manifestations.

Methods: All brain MRI cases that received the diagnosis of SLE at three tertiary university-based hospitals from August 2002 to August 2013 were screened. 219 brain MRIs with diagnosis of SLE were screened. All clinical manifestations found by brain MRI were retrospectively assessed and were classified as NPSLE according to the 1999 NPSLE ACR nomenclature and case definitions. In total, 139 brain MRIs in 121 patients with NPSLE from 2002 to 2013 were retrospectively reviewed. The images were evaluated for the presence of white matter hyperintensity (WMH), gray matter hyperintensity(GMH), parenchymal defects, atrophy, enhancement, and the abnormalities in diffusion-weighted image (DWI).  The number, size and location of WMH,GMH and parenchymal defects were evaluated. The NPSLE manifestations of each patient were classified according to the 1999 ACR case definitions for NPSLE syndromes. The associations between MRI findings and manifestations of NPSLE were examined.

Results: In total, 97 MRIs (69.8%) demonstrated abnormalities among the 139 brain MRIs reviewed. There were 164 NP events that encompassed 16 of 19 NP syndromes. The most common MRI abnormalities were WMHs. One or more WMHs were found in 78 MRIs (56.1 %) among the total 139 MRIs. GMHs were observed in 42 MRIs (32.0%). GMHs tended to involve much larger areas than WMHs. Patients with cerebrovascular disease or seizures were more likely to have GMHs than patients with other NP manifestations. 33 MRIs among 42  MRIs which had GMHs also exhibited WMHs. Parenchymal defects were found in 34 MRIs (24.5%). Atrophy was detected in 20 MRIs (14.4%). Brain MRIs were enhanced in 21 of the 122 cases that had undergone enhancement. Patients who had seizures were more likely to demonstrate MRI enhancement than patients with other NP manifestations. DWIs were obtained in 97 MRIs and abnormal DWIs were obtained in 17 MRIs cases. Patients with cerebrovascular disease were more likely to have GMH, parenchymal defects and abnormal DWI than patients with other NP manifestations.

°°	Number of MRIs (%)	Abnormalities of MRI	WMH	GMH	Parenchymal defect	Atrophy	Enhancement
Acute confusion	9 (6.5 %)	6	3	3	3	1	3
Anxiety disorder	1 (0.7 %)	0	0	0	0	0	0
Aseptic meningitis	6 (4.3 %)	4	3	0	1	0	2
Cerebrovascular disease	32 (23.0 %)	32	26	15	15	4	4
Cognitive dysfunction	4 (2.9 %)	3	3	0	1	3	0
Demyelinating disease	2 (1.4 %)	2	2	1	1	0	1
Headache	33 (23.7 % )	17	12	6	2	4	2
Mood disorder	1 (0.7 %)	0	0	0	0	0	0
Movement disorder	4 (2.9 %)	3	3	0	0	0	0
Psychosis	4 (2.9 %)	3	2	0	1	1	1
Seizure	23 (16.6 %)	17	14	12	5	5	7
Autonomic disorder	3 (2.2 %)	0	0	0	0	0	0
Cranial neuropathy	12 (8.6 %)	7	7	2	2	0	0
Mononeuropathy	3 (2.2 %)	1	1	1	1	0	0
Myasthenia gravis	0 (0 %)	0	0	0	0	0	0
Polyneuropathy	2 (1.4 %)	2	2	2	2	2	1
Total	139 (100 %)°°	97	78	42	34	20	21

Conclusion: Diverse brain MRI abnormalities were observed in the brain MRI of patients with NPSLE. In addition to WMHs, which were previously known as SLE findings, we also noted the presence of GMHs, parenchymal defects and abnormal DWI in a substantial portion of SLE patients, particularly in those with cerebrovascular disease or seizure.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mri-in-neuropsychiatric-lupus-correlations-with-the-1999-acr-case-definitions/