Reappraisal of Rasmussen's syndrome with special emphasis on treatment with high doses of steroids.
Eight patients with Rasmussen's syndrome and epilepsia partialis continua were treated with high doses of steroids, including pulses of methylprednisolone and prednisone in decreasing doses. Three patients exhibited clinical, radiological, or histological evidence of bilateral involvement. Epilepsy and focal deficit decreased within six months in seven patients. Only five patients, in whom steroid treatment had begun less than 15 months after the onset of epilepsia partialis continua, experienced a lasting effect although they had periodic episodes of transient relapse. Treatment with high doses of steroids seems advisable during the first year after onset of epilepsia partialis continua, before hemiplegia has developed and in cases with bilateral involvement.
Neuropediatric Department Hospital Saint Vincent de Paul, Paris, France.
D
D
Chinchilla
O
O
Dulac
O
O
Robain
P
P
Plouin
G
G
Ponsot
J F
JF
Pinel
D
D
Graber
D000293:Adolescent; D017668:Age of Onset; D001706:Biopsy; D001921:Brain / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003711:Demyelinating Diseases; D004828:Epilepsies, Partial / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D005625:Frontal Lobe / Q000503:physiopathology; D006429:Hemiplegia; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D009778:Occipital Lobe / Q000503:physiopathology / Q000648:ultrastructure
D008775:Methylprednisolone