Table 3

Features in the 14 patients with central nervous system and/or cranial nerve involvement

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