THALAMIC DEGENERATION IN X-CHROMOSOME-LINKED COPPER MALABSORPTION

被引:10
作者
IWATA, M [1 ]
HIRANO, A [1 ]
FRENCH, JH [1 ]
机构
[1] MONTEFIORE HOSP & MED CTR,DEPT PEDIAT NEUROL,BRONX,NY 10467
关键词
D O I
10.1002/ana.410050409
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Thalamic degeneration was present in 5 autopsied cases of X‐chromosome–linked copper malabsorption (X‐cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuronal depopulation. The thalamic nuclei that send axons to the agranular cortices were less often and less severely involved. The thalamic afferent system was intact except for degeneration of the red nucleus. Cerebral cortical lesions varied from case to case and usually were less marked than thalamic neuronal changes. Copyright © 1979 American Neurological Association
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页码:359 / 366
页数:8
相关论文
共 22 条
  • [1] AGULAR MJ, 1966, J NEUROPATHOL EXP NE, V25, P507
  • [2] Cowan W.M., 1970, CONT RES METHODS NEU, P217
  • [3] DANKS DM, 1972, PEDIATRICS, V50, P188
  • [4] Dewulf A, 1971, ANATOMY NORMAL HUMAN
  • [5] CEREBRAL MALFORMATION ASSOCIATED WITH METABOLIC DISORDER - A REPORT OF 2 CASES
    ERDOHAZI, M
    BARNES, ND
    ROBINSON, MJ
    LAKE, BD
    [J]. ACTA NEUROPATHOLOGICA, 1976, 36 (04) : 315 - 325
  • [6] FRENCH JH, HDB CLIN NEUROLOGY
  • [7] TRICHOPOLIODYSTROPHY .2. PATHOLOGICAL CHANGES IN SKELETAL-MUSCLE AND NERVOUS-SYSTEM
    GHATAK, NR
    FRENCH, JH
    HIRANO, A
    POON, TP
    [J]. ARCHIVES OF NEUROLOGY, 1972, 26 (01) : 60 - &
  • [8] LAFORAS DISEASE - REPORT OF A CASE IN A SENEGALESE PATIENT
    GIRARD, PL
    ESCOUROLLE, R
    DUMAS, M
    PAPY, JJ
    IWATA, M
    [J]. JOURNAL OF THE NEUROLOGICAL SCIENCES, 1975, 25 (04) : 507 - 527
  • [9] FINE-STRUCTURE OF CEREBELLAR CORTEX IN MENKES KINKY-HAIR DISEASE - X-CHROMOSOME-LINKED COPPER MALABSORPTION
    HIRANO, A
    LLENA, JF
    FRENCH, JH
    GHATAK, NR
    [J]. ARCHIVES OF NEUROLOGY, 1977, 34 (01) : 52 - 56
  • [10] IWATA M, 1973, CONTRIBUTION ETUDE M