Table 2

Clinical features and response to treatment with biological therapies in 81 published cases

Biological agentPapersTypeDisorderPrevious treatmentReason for changeOutcomeFollow-up
TNFa antagonistLicata277 CRBrainS, cycloRelapseResolved<1 y
InfliximabRibi278 CRBrainS, CyA, thalid, cycloRelapseImproved2y
Sarwar279 CRBrainSfirst lineResolved1y
Fujikawa280 CRBrainstemS, cyclo, MTXRelapseImprovedns
Borhani Haghighi281 CRBrainstem, cordS, cycloRelapseDeteriorated (progression)2y
Kikuchi282 5 patientsBrain, brainstemS, MTXProgressionStabilised1y
All progressiveCSF IL-6 reduced
Pipitone283 8 patientsBrainstemS, cycloRelapseResolved1y
BrainstemSfirst lineResolved1y
BrainS, CyA, MTXRelapseResolved3y
Brain, brainstemS, Aza, cycloRelapseImproved<1 y
Brainstem, ONS, CyARelapseImproved<1 y
BrainS, CyARelapseImproved3y
BrainstemS, CyAfirst lineResolved1y
BrainstemS, CyAfirst lineResolved<1 y
Abalos-Medina284 CRBrainS, MTXRelapseResolved1y
Matsui285 2 patientsbrainstemS, MTXProgressionImproved2y
Brain, brainstemS, MTXProgressionPartial response2y
BorhaniHaghighi286 3 patientsbrainstemS, cycloRelapseDeteriorated (progression)ns
Brain, brainstemS, cycloNo responseImproved2y
Kadowaki*, 287 CRBrainstemS, cyclofirst lineImproved1y
Fasano288 CRBrainstemS, MTXRelapseImprovedns
Giardini289 5 patientsBrainS, Aza, MTX, CyAfirst lineImprovedns
“cerebral vasculitis”S, CyARelapseResolved1y
“cerebral vasculitis”S, CyARelapseResolved1y
“cerebral vasculitis”S, Aza ChlRelapseResolved1y
“cerebral vasculitis”S, Aza, CyARelapseResolved1y
Coulter96 CR“cerebral vasculitis”S, cycloRelapseResolved1y
Kuroda290 CRCordSfirst lineResolved<1 y
Kanemaru291 CRCordS, MTXProgressionImproved<1 y
Li292 CRBrain, brainstemS, CyAfirst lineResolved2y
di Filippo293 CRBrainstemS, Azafirst lineResolved4y
Satuka* 294 CRBrainSfirst lineResolved3y
Uygunoglu98 CRCordS, cycloProgressionImprovedns
Hibi234 3 patientsCordS, cyclofirst lineImproved3y
Desbois295 13 patientsBrainstem progressiveSRelapseStabilised1y
BrainS, cyclo, Aza, MMFProgressionImproved4y
BrainS, Aza, cycloImproved1y
BrainS, Aza CyAResolved1y
Cord, brain, brainstemS, Aza, Cyclo, MMFResolved<1 y
BrainstemS, AzaImproved2y
BrainS, AzaImproved1y
BrainS, Aza, cyclo, ChlImproved>10 y
BrainstemS, cycloImproved2y
BrainS, cyclo, ChlResolved6y
Cord, brainstemSNo response1y
BrainstemS, AzaResolved<1 y
BrainstemS, cycloImproved1y
Zeydan253 15 patientsBrain, brainstemS, Aza, cycloImproved<1 y
Hamada* 235 CRRelapsing diseaseNsfour improved1y
Baresic296 CRProgressive brainstemS, MTXProgressionPartial responsens
Brainstem, ONS, Aza, CyARelapseImproved2y
AdalimumabBelzenegui297 CR
Lecesse259 CRCord, brainS, Aza, cyclo, IFXs/e IFXImproved3y
Robinson298 CRBrainstemS, CyA, IFXRelapseImproved<11 y
Korkmaz260 CRBrainstemS, MMFRelapseResolved1y
Desbois295 4 patientsBrainstemS, cyclo, AzaRelapseImproved<1 y
BrainstemS, cyclo, Aza, INFResolved2y
BrainS, AzaImproved<1 y
CordS, AzaImproved2y
CordS, Aza INFImproved4y
CRBrainstemS, Aza, CyA, thalid, IFXRelapseImproved3y
?7 patientsno datano data
IL-6 antagonist
TocilizumabShapiro301 CR
Urbeniak302 CRBrainstemS, Aza, MTX, MMF, IFXs/e IFXResolved<1 y
Addimanda303 3 patientsCordS, Aza, IFXRelapseImproved1y
ON, brain, cordS, IFX, cycloUnresponsiveImproved1y
ON, brain, cordS, Aza, cyclo, INFRelapseStabilised1y
Brain, brainstem, cordS, CyA, IFXRelapseStabilised1y
IL-1 antagonist
AnakinraVitale304 ON
CanakinumabEmmi305 CRS, Azafirst lineImprovedns
Gevokizumabone case ? same as Vitale
IL-12 / −17 / −23 antagonistno reports
CD20 antagonist
Kidd306 CRS, cycloRelapseImprovedns
Jade307 CRCordS, Aza, MMF, IFXRelapseResolved3y
CRBrainstemS, col, cyclo, IFXRelapseResolved3y
Tumefactive brain
CD52 antagonist
AlemtuzumabLockwood308 12 patients
nsS, Aza, CyA, cyclo, MMFnsPartial responsens
SNo response
S, CyAPartial response
S, cycloResolved
S, AzaNo response
S, Aza col, thalidNo response
S, CyANo data
S, Aza MTXNo response
S, Aza CyAGood response
S, CyA, thalidNo response
Janus kinase inhibitorsno reports
  • *Data derived from abstract alone.

  • Aza, azathioprine; Chl, chlorambucil; col, colchicine; CR, case report; CyA, cyclosporine A; cyclo, cyclophosphamide; IFX, infliximab; INF, interferon α2a; MMF, mycophenolate; MTX, methotrexate; ns, not stated; S, corticosteroids; thalid, thalidomide.