Experience with immunomodulatory treatments in Rasmussen's encephalitis.
The authors investigated immunomodulatory treatments in 15 patients with Rasmussen encephalitis (RE) (14 with childhood and one with adolescent onset RE). Positive time-limited responses were obtained in 11 patients using variable combinations of corticosteroids, apheresis, and high-dose IV immunoglobulins. Although surgical exclusion of the affected hemisphere is the only treatment that halts disease progression, immunomodulation can be considered when early surgery is not feasible, in late-onset patients with slower disease progression, and in the few cases of bilateral disease.
Granata
T
T
Division of Child Neurology, Istituto Nazionale Neurologico C. Besta, Milan, Italy. granata@istituto-besta.it
Fusco
L
L
Gobbi
G
G
Freri
E
E
Ragona
F
F
Broggi
G
G
Mantegazza
R
R
Giordano
L
L
Villani
F
F
Capovilla
G
G
Vigevano
F
F
Bernardina
B Dalla
BD
Spreafico
R
R
Antozzi
C
C
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D001781:Blood Component Removal; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D018450:Disease Progression; D004660:Encephalitis / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy*; D017036:Epilepsia Partialis Continua / Q000209:etiology; D005260:Female; D038421:Hemispherectomy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction / Q000379:methods; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide