PERVASIVE NEUROANATOMICAL ABNORMALITIES OF THE BRAIN IN 3 CASES OF RETTS-SYNDROME

被引:137
作者
BAUMAN, ML
KEMPER, TL
ARIN, DM
机构
[1] BOSTON CITY HOSP,DEPT NEUROL,BOSTON,MA 02118
[2] MASSACHUSETTS INST ALLIED HLTH PROFESS,BOSTON,MA
关键词
D O I
10.1212/WNL.45.8.1581
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Rett's syndrome (RS) is a clinically defined disorder that appears to be unique to girls and is characterized by apparent cognitive and motor skill loss early in life. We report our findings in the brains of three girls with RS, which were studied in comparison with age-matched controls by means of gapless serial section. Reduced neuronal cell size and increased cell-packing density were present throughout the cortical and subcortical regions of the brain in all cases without evidence of active degeneration. These observations appear to be consistent with a curtailment of development. Further, the degree of abnormality in each case correlates more closely with the clinical presentation of the patient at the time of death than with the age of the patient or duration of symptoms.
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页码:1581 / 1586
页数:6
相关论文
共 45 条
  • [1] Angevine J, 1961, HUMAN CEREBELLUM ATL
  • [2] [Anonymous], 1967, CEREBELLUM NEURONAL, DOI 10.1007/978-3-662-13147-3
  • [3] ARIN D M, 1991, Neurology, V41, P307
  • [4] ARMSTRONG DD, 1992, BRAIN DEV-JPN, V14, pS89
  • [5] HISTOANATOMIC OBSERVATIONS OF THE BRAIN IN EARLY INFANTILE-AUTISM
    BAUMAN, M
    KEMPER, TL
    [J]. NEUROLOGY, 1985, 35 (06) : 866 - 874
  • [6] BAUMAN ML, 1991, PEDIATRICS, V87, P791
  • [7] BAUMAN ML, 1994, NEUROBIOLOGY AUTISM, P116
  • [8] Modification of Gudden method for study of cerebral localization
    Brodal, A
    [J]. ARCHIVES OF NEUROLOGY AND PSYCHIATRY, 1940, 43 (01): : 46 - 58
  • [9] CROSBY EC, 1962, CORRELATIVE ANATOMY, P260
  • [10] de No RL, 1934, J PSYCHOL NEUROL, V46, P113