ETIOLOGY OF INTELLECTUAL IMPAIRMENT IN DUCHENNE MUSCULAR-DYSTROPHY

被引:29
作者
ALQUDAH, AA
KOBAYASHI, J
CHUANG, S
DENNIS, M
RAY, P
机构
[1] Department of Pediatrics, Jordan University Hospital, Amman
[2] Division of Neurology, The Hospital for Sick Children, Toronto, Ont.
[3] Department of Radiology, The Hospital for Sick Children, Toronto, Ont.
[4] Department of Psychology, The Hospital for Sick Children, Toronto, Ont.
[5] Department of Genetics, The Hospital for Sick Children, Toronto, Ont.
关键词
D O I
10.1016/0887-8994(90)90081-B
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The precise etiology of intellectual impairment in Duchenne muscular dystrophy (DMD) is unknown. Histopathologic and computed tomographic studies have revealed structural brain changes; however, to our knowledge, no cranial magnetic resonance imaging (MRI) studies have been performed on DMD patients to further delineate these structural changes. We prospectively studied 4 DMD patients by cranial MRI, DNA deletion analysis, clinical evaluation, and intelligence testing. There was no significant correlation between verbal intelligence scores and MRI findings, DNA deletion, or the clinical severity of the disease. These first MRI studies of DMD did not reveal any significant anatomic brain alteration, other than mild atrophy in 2 patients. We believe these results must be considered when investigating the etiology of intellectual impairment in DMD in future studies with larger patient samples. © 1990.
引用
收藏
页码:57 / 59
页数:3
相关论文
共 19 条
  • [1] Brett, Lake, Neuromuscular disorders, Pediatric neurology, pp. 56-64, (1983)
  • [2] Dubowitz, Mental retardation in Duchenne muscular dystrophy, Pathogenesis of human muscular dystrophies, 404, (1977)
  • [3] Lindlof, Kaariainen, Vanommen, De La Chapelle, Microdeletions in patients with X-linked muscular dystrophy: Molecular-clinical correlations, Clin Genet, 33, pp. 131-193, (1988)
  • [4] Rosman, Kakulas, Mental deficiency associated with muscular dystrophy. A neuropathological study, Brain, 89, pp. 769-788, (1966)
  • [5] Rosman, The cerebral defect and myopathy in Duchenne muscular dystrophy: Comparative clinicopathological study, Neurology, 20, pp. 324-335, (1970)
  • [6] Jagadha, Becker, Brain morphology in Duchenne muscular dystrophy: A Golgi study, Pediatr Neurol, 4, pp. 87-92, (1988)
  • [7] Yoshioka, Okuno, Honda, Nakano, Central nervous system involvement in progressive muscular dystrophy, Arch Dis Child, 55, pp. 589-594, (1980)
  • [8] Brooke, Griggs, Mendell, Fenichel, Shumate, Pellegrino, Clinical trial in Duchenne dystrophy—the design of the protocol, Muscle Nerve, 4, pp. 186-197, (1981)
  • [9] Wechsler, Wechsler Intelligence Scale for Children-Revised, (1974)
  • [10] Wechsler, Wechsler Intelligence Scale-Revised, (1981)