Table 2. Clinical, Radiological, and Serological Characteristics of Patients.
| Characteristic | No. | Autoimmune disease, No. | Inflammatory CNS event, No. | Median (range), y | Autoantibody, No. | Neuroimaging, No./total | CSF OCB, No./total | CSF pleocytosis, No./total | Biopsy, No. | TNF inhibitor exposure, No. (%) | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Age at inflammatory CNS event onset | Disease duration at inflammatory CNS event onset | ||||||||||
| Demyelinating CNS events | |||||||||||
| Demyelination | 48 | RA = 24; CD = 11; P/PA = 9; UC = 3; AS = 2 | RRMS = 20; CIS = 17; PPMS = 4; RIS = 3; AQP4-IgG NMOSD = 3; MOG-IgG TM = 1 | 12 (6-18) | AQP4-IgG NMOSD = 3; MOG-IgG = 1 | MRI, features of demyelination of brain or spinal cord = 48/48 | 20/41 | 10/41 | NA | 33 (69) | |
| Optic neuritis | 8 | CD = 4; RA = 2; P/PA = 3 | UON = 6; BON = 2; (AQP4-IgG NMOSD = 1) | 60 (53-62) | 15 (8-27) | AQP4-IgG NMOSD = 1 | MRI = 3/8 with UON; 0/8 with demyelination of brain | 0/3 | 0/3 | NA | 6 (75) |
| Nondemyelinating CNS events | |||||||||||
| Meningitis | 23 | RA = 13; P/PA = 5; CD = 3; UC = 2 | Aseptic (CSF only) = 8; leptomeningitis = 9; pachymeningitis = 6 | 68 (47-78) | 10 (4-29) | NA | MRI, leptomeningitis = 9/23; pachymeningitis = 6/23 (all aseptic had negative result on MRI) | 1/13 | 18/23 (All aseptic had pleocytosis) | Leptomeningeal = 3 (leptomeningeal inflammation); pachymeningeal = 2 (inflammation of the dura) | Aseptic = 3 (38); lepto-pachymeningitis = 9 (60) |
| Meningoencephalitis | 4 | RA = 2; P/PA = 1; AS = 1 | Meningoencephalitis = 4; cerebellitis with meningeal = 1 | 61 (56-65) | 4 (2-8) | GFAP-IgG = 2 | MRI, abnormal leptomeningitis and parenchyma = 4/4 | 3/3 | 4/4 | Brain (temporal, cerebellar, frontal) = 3 (leptomeningeal and parenchymal acute and chronic inflammation) | 3 (75) |
| Encephalitis | 7 | CD = 4; AS = 2; RA = 1 | Limbic = 3; brainstem = 1; frontal lobe = 1; multifocal = 1; cerebellitis = 1 | 45 (42-46) | 9 (5-19) | Limbic encephalitis = 2 (patient 1: serum GAD65 = 0.09; patient 2: serum acetylcholine receptor modulating antibody = 34% loss; GAD65 antibody = 0.35 nmol/L; striational antibody = 1:240) | MRI, abnormal brain parenchyma = 7/7 | 1/5 | 6/7 | Brain (frontal) = 1 (microglial activation and chronic inflammation) | 3 (43) |
| Neurosarcoidosis | 8 | RA = 4; CD = 3; AS = 1 | Leptomeningeal = 3; pachymeningeal = 3; cavernous sinus = 1; myelopathy = 1 | 59 (54-64) | 16 (13-16) | NA | MRI, abnormal brain or spinal cord = 8/8 | 1/6 | 6/8 | Brain = 4; pulmonary = 1; lymph node = 2; spinal cord = 1 (granulomatous disease) | 7 (88) |
| CNS vasculitis | 8 | CD = 3; RA = 2; P/PA = 2; UC = 1 | CNS vasculitis = 8 | 51 (44-65) | 5 (3-9) | NA | MRI, radiological features of vasculitis = 6/8; DSA = 6/7 features of vasculitis | 0/5 | 6/7 | Brain = 2 (perivascular chronic inflammation, hemorrhage and small vein microthrombi | 0 |
Abbreviations: AQP4-IgG NMOSD, aquaporin-4-IgG–positive neuromyelitis optica spectrum disorder; AS, ankylosing spondylitis; BON, bilateral optic neuritis; CD, Crohn disease; CIS, clinically isolated syndrome; CNS, central nervous system; CSF, cerebrospinal fluid; DSA, digital subtraction angiogram; GAD, glutamic acid decarboxylase; GFAP, glial fibrillary acidic protein; MOG-IgG, myelin oligodendrocyte glycoprotein-IgG–associated disorder; MRI, magnetic resonance imaging; NA, not applicable; OCB, oligoclonal bands; PPMS, primary progressive multiple sclerosis; P/PA, psoriasis and psoriatic arthritis; RA, rheumatoid arthritis; RIS, radiologically isolated syndrome; RRMS, relapsing-remitting multiple sclerosis; TM, transverse myelitis; UC, ulcerative colitis; UON, unilateral optic neuritis.