MEDICAL-TREATMENT OF RASMUSSENS-SYNDROME (CHRONIC ENCEPHALITIS AND EPILEPSY) - EFFECT OF HIGH-DOSE STEROIDS OR IMMUNOGLOBULINS IN 19 PATIENTS

被引:158
作者
HART, YM
CORTEZ, M
ANDERMANN, F
HWANG, P
FISH, DR
DULAC, O
SILVER, K
FEJERMAN, N
CROSS, H
SHERWIN, A
CARABALLO, R
机构
[1] MONTREAL NEUROL HOSP & INST, MONTREAL H3A 2B4, PQ, CANADA
[2] HOSP SICK CHILDREN, TORONTO M5G 1X8, ON, CANADA
[3] UCL NATL HOSP NEUROL & NEUROSURG, LONDON WC1N 3BG, ENGLAND
[4] HOP ST VINCENT DE PAUL, F-75674 PARIS, FRANCE
[5] MONTREAL CHILDRENS HOSP, MONTREAL H3H 1P3, PQ, CANADA
[6] HOSP JUAN GARRAHAN, BUENOS AIRES, ARGENTINA
[7] HOSP SICK CHILDREN, LONDON WC1N 3JH, ENGLAND
关键词
D O I
10.1212/WNL.44.6.1030
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We treated 19 patients with Rasmussen's syndrome (chronic encephalitis and epilepsy)-a rare progressive disorder of unknown etiology causing focal epilepsy, hemiparesis, and intellectual deterioration-with intravenous immunoglobulins, high-dose steroids, or both, to control seizures and improve the end point of the disease. Ten of 17 patients receiving steroids, and eight of nine patients receiving immunoglobulins, had some reduction of seizure frequency in the short term. Improvement in hemiparesis was slight. The effect of these drugs in ameliorating the end point of the disease in the long term remains unknown, and further multicenter studies with standardized protocols are warranted.
引用
收藏
页码:1030 / 1036
页数:7
相关论文
共 28 条
  • [1] CHRONIC ENCEPHALITIS, EPILEPSY, AND CEREBROVASCULAR IMMUNE-COMPLEX DEPOSITS
    ANDREWS, JM
    THOMPSON, JA
    PYSHER, TJ
    WALKER, ML
    HAMMOND, ME
    [J]. ANNALS OF NEUROLOGY, 1990, 28 (01) : 88 - 90
  • [2] HIGH-DOSE GAMMA-GLOBULIN FOR INTRACTABLE CHILDHOOD EPILEPSY
    ARIIZUMI, M
    SHIIHARA, H
    HIBIO, S
    RYO, S
    BABA, K
    OGAWA, K
    SUZUKI, Y
    MOMOKI, T
    [J]. LANCET, 1983, 2 (8342) : 162 - 163
  • [3] Asher DM, 1991, CHRONIC ENCEPHALITIS, P147
  • [4] EFFECTS OF GAMMA-GLOBULIN THERAPY IN SEVERELY EPILEPTIC CHILDREN
    BEDINI, R
    DEFEO, MR
    ORANO, A
    ROCCHI, L
    [J]. EPILEPSIA, 1985, 26 (01) : 98 - 102
  • [5] THE PROGNOSIS AND LONG-TERM COURSE OF VIRAL ENCEPHALITIS
    BERLIT, P
    [J]. JOURNAL OF NEUROIMMUNOLOGY, 1988, 20 (2-3) : 117 - 125
  • [6] INFANTILE SPASMS - COMPARATIVE TRIAL OF NITRAZEPAM AND CORTICOTROPIN
    DREIFUSS, F
    FARWELL, J
    HOLMES, G
    JOSEPH, C
    LOCKMAN, L
    MADSEN, JA
    MINARCIK, CJ
    ROTHNER, AD
    SHEWMON, DA
    [J]. ARCHIVES OF NEUROLOGY, 1986, 43 (11) : 1107 - 1110
  • [7] Dulac O., 1992, Epilepsia, V33, P128
  • [8] DULAC O, 1991, CHRONIC ENCEPHALITIS, P193
  • [9] TREATMENT OF INFANTILE SPASMS WITH INTRAVENOUS GAMMA-GLOBULINS
    ECHENNE, B
    DULAC, O
    PARAYRECHANEZ, MJ
    CHIRON, C
    TAILLEBOIS, L
    COGNOT, C
    ANDARY, M
    CLOT, J
    BALDYMOULINIER, M
    [J]. BRAIN & DEVELOPMENT, 1991, 13 (05) : 313 - 319
  • [10] IMMUNOLOGICAL STUDIES IN FOCAL EPILEPSY
    EEGOLOFSSON, O
    OSTERLAND, CK
    GUTTMANN, RD
    ANDERMANN, F
    PRCHAL, JF
    ANDERMANN, E
    JANJUA, NA
    [J]. ACTA NEUROLOGICA SCANDINAVICA, 1988, 78 (05): : 358 - 368