ACR 1999 NPSLE diagnoses in primary NPSLE patients. Patients can have multiple diagnosis. ACR 1999 diagnoses not found in our patients include aseptic meningitis, demyelinating syndrome, acute confusional state, acute inflammatory demyelinating polyradiculopathy, autonomic disorder, myasthenia gravis, cranial neuropathy, and plexopathy. Data are number (%).
| Diagnosis | Undefined NPSLE, n = 5 | Inflammatory NPSLE, n = 21 | Ischemic NPSLE, n = 12 |
|---|---|---|---|
| Cognitive dysfunction | 2 (40) | 14 (67) | 6 (50) |
| Cerebrovascular disease | 3 (14) | 11 (92) | |
| Infarction | 3 (100) | 10 (91) | |
| Infarction and hemorrhage | 1 (9) | ||
| Headache | 9 (43) | 6 (50) | |
| Intractable | 4 (44) | 3 (50) | |
| Migraine with aura | 4 (44) | 1 (17) | |
| Migraine without aura | 2 (33) | ||
| Tension | 1 (11) | ||
| Mood disorders | 3 (60) | 7 (33) | 3 (25) |
| Major depressive episode | 2 (67) | 3 (43) | 1 (33) |
| Depressive features | 1 (33) | 2 (29) | 2 (67) |
| Mixed (depression and manic) | 2 (29) | ||
| Seizure disorders | 4 (19) | 2 (17) | |
| Generalized | 4 (100) | 2 (100) | |
| Psychosis | 4 (19) | ||
| Anxiety disorder | 1 (5) | ||
| Myelopathy | 1 (5) | ||
| Movement disorder (chorea) | 1 (5) | ||
| Mononeuropathy | 1 (5) | ||
| Multiplex | 1 (100) | ||
| Polyneuropathy | 1 (8) |
ACR: American College of Rheumatology; NPSLE: neuropsychiatric systemic lupus erythematosus.