Features in the 14 patients with central nervous system and/or cranial nerve involvement
| Patient | Sex/age (years) | Symptoms and treatment |
|---|---|---|
| 1 | M/71 | Involvement of the right fifth cranial nerve for 14 years despite pharmacological treatment. |
| 2 | M/47 | Involvement of both fifth cranial nerves and of the optic nerve |
| 3 | F/80 | Involvement of the right fifth cranial nerve that resolved with gabapentin treatment |
| 4 | M/64 | Cerebellar syndrome with cerebral MRI showing persistent multiple hyperintensities despite glucocorticoids, cyclophosphamide, mycofenolate mofetil and rituximab |
| 5 | M/53 | Urinary retention suggesting transverse myelitis; uninformative spinal-cord MRI; resolution with cyclophosphamide therapy |
| 6 | F/35 | C4-C5 myelitis, normal cerebrospinal fluid, improvement with pulsed glucocorticoids, but persistent lower-limb dysesthesia |
| 7 | M/63 | D6-D8 myelitis with proprioceptive ataxia and lower-limb paresis; improvement with glucocorticoids and cyclophosphamide but residual abnormalities |
| 8 | F/49 | C6-C7 myelitis with right lower-limb paresis confirmed by EMG and MRI; partial regression with glucocorticoids and cyclophosphamide |
| 9 | F/60 | Right hemiparesis, left homonymous hemianopsia, dizziness, multiple hyperintensities by cerebral MRI, no embolic heart disease |
| 10 | F/61 | Headaches, acute-phase reactant elevation, hemiparesis, cerebellar involvement, multiple MRI lesions, irreversible sequelae complicated with vascular dementia despite treatment with glucocorticoids and cyclophosphamide |
| 11 | F/57 | Two episodes of stroke (cerebellar and optic artery) and sudden deafness with no cardiovascular risk factor |
| 12 | F/52 | Lymphocytic meningitis at diagnosis with no detectable infectious and other autoimmune cause; resolution after glucocorticoid therapy for 2 years; no relapse |
| 13 | F/66 | Involvement of the second and third branches of the left fifth cranial nerve, treated with clonazepam |
| 14 | F/51 | Dizziness, vertigo, sudden deafness, optic neuritis with multiple cerebral MRI lesions; glucocorticoids and antimalarial drugs; persistent dizziness but no relapse |
EMG, electromyography.