CONGENITAL RETINAL DYSTROPHIES - A STUDY OF EARLY COGNITIVE AND VISUAL DEVELOPMENT

被引:8
作者
BLACK, MM [1 ]
SONKSEN, PM [1 ]
机构
[1] POOLE GEN HOSP, POOLE BH15 2JB, DORSET, ENGLAND
关键词
D O I
10.1136/adc.67.3.262
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The reported incidence of mental retardation in Leber's congenital amaurosis has varied from 10% to 87%. There has been no review of the estimate since it became possible to delineate an increasing number of diagnostic subcategories. In this study, the visual and cognitive development of 38 children with congenital retinal dystrophies has been followed up prospectively. Children with associated disorders in other systems and those with central nervous system malformations or degenerations were significantly more likely to have learning disability than those without additional medical problems. Most subgroups made little or no visual progress with the exception of the group with associated hypoplasia of the cerebellar vermis. The study highlights the importance of using the specialised techniques now available to delineate fully the visual diagnosis and paediatric perspective because of their relevance to congitive and visual prognosis.
引用
收藏
页码:262 / 265
页数:4
相关论文
共 25 条
  • [1] CHILDHOOD ONSET PERVASIVE DEVELOPMENTAL DISORDER
    BURD, L
    FISHER, W
    KERBESHIAN, J
    [J]. JOURNAL OF CHILD PSYCHOLOGY AND PSYCHIATRY AND ALLIED DISCIPLINES, 1988, 29 (02): : 155 - 163
  • [2] CANTANI A, 1990, ANN GENET-PARIS, V33, P96
  • [3] DEKABAN AS, 1972, DEV MED CHILD NEUROL, V14, P436
  • [4] FIELDER AR, 1991, OPHTHALMOLOGY, V98, P1306
  • [5] TAPETORETINAL DEGENERATION IN THE CEREBRO-HEPATO-RENAL (ZELLWEGERS) SYNDROME
    GARNER, A
    FIELDER, AR
    PRIMAVESI, R
    STEVENS, A
    [J]. BRITISH JOURNAL OF OPHTHALMOLOGY, 1982, 66 (07) : 422 - 431
  • [6] NORRIES DISEASE - A PROSPECTIVE-STUDY OF DEVELOPMENT
    GOODYEAR, HM
    SONKSEN, PM
    MCCONACHIE, H
    [J]. ARCHIVES OF DISEASE IN CHILDHOOD, 1989, 64 (11) : 1587 - 1592
  • [7] JOUBERTS SYNDROME WITH RETINAL DYSPLASIA - NEONATAL TACHYPNEA AS THE CLUE TO A GENETIC BRAIN-EYE MALFORMATION
    KING, MD
    DUDGEON, J
    STEPHENSON, JBP
    [J]. ARCHIVES OF DISEASE IN CHILDHOOD, 1984, 59 (08) : 709 - 718
  • [8] BEHAVIOR CHECKLIST FOR IDENTIFYING SEVERELY HANDICAPPED INDIVIDUALS WITH HIGH-LEVELS OF AUTISTIC BEHAVIOR
    KRUG, DA
    ARICK, J
    ALMOND, P
    [J]. JOURNAL OF CHILD PSYCHOLOGY AND PSYCHIATRY, 1980, 21 (03) : 221 - 229
  • [9] FOLLOW-UP AND DIAGNOSTIC REAPPRAISAL OF 75 PATIENTS WITH LEBERS CONGENITAL AMAUROSIS
    LAMBERT, SR
    KRISS, A
    TAYLOR, D
    COFFEY, R
    PEMBREY, M
    [J]. AMERICAN JOURNAL OF OPHTHALMOLOGY, 1989, 107 (06) : 624 - 631
  • [10] LEBER T, 1989, GRAEFES ARCH CLIN EX, V15, P1