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青少年型与晚婴儿型异染性脑白质营养不良的临床病理差异

Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy.

作者信息

Takashima S, Matsui A, Fujii Y, Nakamura H

出版信息

Brain Dev. 1981;3(4):365-74. doi: 10.1016/s0387-7604(81)80065-4.

DOI:10.1016/s0387-7604(81)80065-4
PMID:6119040
Abstract

The autopsy report of the juvenile type of metachromatic leukodystrophy is rare. The clinical and pathological difference between the juvenile and the late infantile type of metachromatic leukodystrophy was described. Loss of myelin sheaths was much less in the brain stem and spinal cord in the juvenile type than in the late infantile type, although that of the roots or cranial and spinal nerves was marked similarly in both types. The relationship of these pathological findings to clinical signs and laboratory data was discussed.

摘要

青少年型异染性脑白质营养不良的尸检报告很少见。本文描述了青少年型和晚婴儿型异染性脑白质营养不良在临床和病理上的差异。青少年型患者脑干和脊髓的髓鞘脱失程度远低于晚婴儿型,尽管两种类型神经根、颅神经及脊神经的髓鞘脱失均较明显。文中还讨论了这些病理表现与临床症状及实验室数据之间的关系。

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Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy.青少年型与晚婴儿型异染性脑白质营养不良的临床病理差异
Brain Dev. 1981;3(4):365-74. doi: 10.1016/s0387-7604(81)80065-4.
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A 5-year-old male child with late infantile metachromatic leukodystrophy: a case report.一名患有晚发性婴儿型异染性脑白质营养不良的5岁男童:病例报告。
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Juvenile metachromatic leukodystrophy. Clinical, biochemical, and neuropathologic studies in nine new cases.青少年异染性脑白质营养不良。9例新病例的临床、生化及神经病理学研究。
Arch Neurol. 1980 Jan;37(1):42-6. doi: 10.1001/archneur.1980.00500500072011.
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Arylsulfatases A and B in leukocytes: a comparative statistical study of late infantile and juvenile forms of metachromatic leukodystrophy and controls.白细胞中的芳基硫酸酯酶A和B:晚期婴儿型和青少年型异染性脑白质营养不良及对照的比较统计研究
Biomedicine. 1980 Feb;33(1):2-4.
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Bone marrow transplantation in metachromatic leukodystrophy.异染性脑白质营养不良的骨髓移植
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Metachromatic leukodystrophy: clinical and enzymatic parameters.异染性脑白质营养不良:临床及酶学参数
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Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy.在芳基硫酸酯酶A缺乏的小鼠的髓鞘形成细胞中增加硫苷脂合成会导致脱髓鞘和神经症状,类似于人类异染性脑白质营养不良。
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[Metachromatic leukodystrophy. Late juvenile form. Review of 6 cases].[异染性脑白质营养不良。晚发型青少年型。6例病例回顾]
An Esp Pediatr. 1983 Jul;19(1):41-8.
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[Metachromatic leukodystrophy: report of 2 cases with histochemistry of nerves and muscles].[异染性脑白质营养不良:2例神经与肌肉组织化学报告]
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[Juvenile form of metachromatic leukodystrophy. Clinical variant of sulfatidosis].[少年型异染性脑白质营养不良。硫脂沉积症的临床变异型]
Pediatrie. 1978 Oct-Nov;3(7):629-36.

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