Table 1.
Clinical background of the patients with NBD
| Symptom | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Patient no. | Age at NBD onset | Sex | Oral ulcer | Genital ulcer | Uveitis | Skin lesion | Other symptoms | Neurological symptom | NBD onset after BD diagnosis | Brain MRI | Therapy for NBD |
| 1 | 16 | F | (+) | (+) | (−) | (+) | Arthritis Thrombophlebitis | Meningitis | Same time | WNL | PSL 60 mg/day, mino 200 mg/day |
| 2 | 49 | F | (+) | (−) | (+) | (+) | Myelopathy | 1 year later | WNL | mPSL pulse×2 | |
| 3 | 40 | M | (+) | (−) | (+) | (+) | Arthritis | Optic neuritis | 1 year later | WNL | PSL 30 mg/day, MTX 5 mg/week |
| 4 | 49 | M | (+) | (+) | (+) | (+) | Colon ulcer | Meningitis | 9 years later | WNL | COL 1.5 mg/day, PSL 30 mg/day |
| 5 | 44 | F | (+) | (+) | (+) | (+) | Optic neuritis | 6 years later | WNL | PSL (maximum dose): unknown, COL 1.5 mg/day, MTX 4 mg/week | |
| 6 | 60 | F | (+) | (+) | (−) | (+) | Arthritis | Meningitis | 24 years later | WNL | PSL 20 mg/day, COL 1.0 mg/day |
| 7 | 34 | M | (+) | (+) | (+) | (+) | Meningitis, headache | 14 years later | WNL | PSL (maximum dose): unknown, COL 1.0 mg/day | |
| 8 | 49 | M | (+) | (−) | (+) | (+) | Arthritis | Left hemiparesis | 7 years later | Unknown | mPSL pulse, IVCY, MTX 8 mg/w, COL 1.5 mg/day |
| 9 | 49 | M | (+) | (−) | (+) | (+) | Arthritis | Meningitis, dizziness | 17 years later | Old infarction in cerebellum | mPSL pulse, MTX 4 mg/week |
| 10 | 24 | M | (+) | (−) | (+) | (+) | Colon ulcer | Meningitis | 2 months later | High intensity in the medulla | mPSL pulse |
| 11 | 49 | F | (+) | (+) | (+) | (+) | Colon ulcer | Dementia | 8 months later | High intensity in the white matter | PSL 60 mg/day, MTX 4 mg/week |
| 12 | 31 | F | (+) | (+) | (−) | (+) | Arthritis | Psychosis, rt sensory distuebance | 17 years later | High intensity in the white matter, rt thalamus, pons, Lt cerebellum | mPSL pulse, COL 1.0 mg/day |
| 13 | 67 | M | (+) | (+) | (+) | (+) | Colon ulcer, Thrombophlebitis | Psychosis, meningitis | 42 years later | WNL | PSL 30 mg/day, COL 1.0 mg/day |
| 14 | 67 | F | (+) | (+) | (+) | (+) | Arthritis | Confusion, dementia | 33 years later | WNL | PSL 45 mg/day |
| 15 | 30 | M | (+) | (+) | (+) | (+) | Arthritis | Pcychosis | 8 years later | WNL | PSL 60 mg/day, COL 1.0 mg/day |
| 16 | 56 | M | (+) | (+) | (+) | (+) | Colon ulcer, Thrombophlebitis | Psychosis, meningitis | 35 years later | WNL | PSL (maximum dose); unknown, COL 1.0 mg/day |
| 17 | 35 | M | (+) | (−) | (+) | (+) | Dementia, psychosis | 1 year later | WNL | PSL 60 mg/day, MTX 4 mg/week, COL 1.0 mg/day | |
| 18 | 30 | M | (+) | (+) | (+) | (+) | Dementia, psychosis | 6 years later | High intensity in the white matter | PSL 50 mg/day, MTX 4 mg/week, COL 1.5 mg/day | |
NBD, Neuro-Behçet's disease; PSL, prednisolone; COL, colchicine; MTX, methotrexate; mino, minocycline; IVCY, intravenous cyclophosphamide pulse therapy; rt, right; Lt, left.