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亚历山大病:一份报告及重新评估

Alexander's disease: a report and reappraisal.

作者信息

Russo L S, Aron A, Anderson P J

出版信息

Neurology. 1976 Jul;26(7):607-14. doi: 10.1212/wnl.26.7.607.

DOI:10.1212/wnl.26.7.607
PMID:180453
Abstract

A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination. This may be the first report of a case with significant neuronal changes and inflammation within the brain stem in this disease. The clinical picture of this entity is varied and age-dependent. Alexander's disease may be a motor system specific entity secondary to astrocytic dysfunction.

摘要

一名患有亚历山大病幼年起病症状的男孩,临床表现为假性延髓麻痹、共济失调和痉挛性截瘫。中枢神经系统的病理检查显示弥漫性存在罗森塔尔纤维和斑片状脱髓鞘。这可能是该疾病中首例脑干内存在显著神经元变化和炎症的病例报告。该疾病的临床表现多样且与年龄相关。亚历山大病可能是继发于星形细胞功能障碍的运动系统特异性疾病。

相似文献

1
Alexander's disease: a report and reappraisal.亚历山大病:一份报告及重新评估
Neurology. 1976 Jul;26(7):607-14. doi: 10.1212/wnl.26.7.607.
2
Alexander's disease. A disease of astrocytes.亚历山大病。一种星形胶质细胞疾病。
Brain. 1985 Jun;108 ( Pt 2):367-85. doi: 10.1093/brain/108.2.367.
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Prominent white matter cavitation in an infant with Alexander's disease.患有亚历山大病的婴儿出现显著的白质空洞形成。
Clin Neuropathol. 1994 Jan-Feb;13(1):31-8.
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Identification of GFAP gene mutation in hereditary adult-onset Alexander's disease.遗传性成人型亚历山大病中胶质纤维酸性蛋白(GFAP)基因突变的鉴定。
Ann Neurol. 2002 Dec;52(6):779-85. doi: 10.1002/ana.10375.
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Alexander's disease in an adult: clinicopathologic study of a case and review of the literature.
Clin Neuropathol. 1982;1(3):106-12.
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Bulbar palsy with Rosenthal fiber formation in the medulla of a 15-year-old girl. Localized form of Alexander's disease?
Neuropediatrics. 1981 Nov;12(4):382-91. doi: 10.1055/s-2008-1059669.
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Progressive parkinsonism associated with Rosenthal fibers: senile-onset Alexander's disease?与罗森塔尔纤维相关的进行性帕金森病:老年起病的亚历山大病?
Neurology. 1992 Sep;42(9):1733-5. doi: 10.1212/wnl.42.9.1733.
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The corticofugal projection on the brain stem and spinal cord in man.
Psychiatr Neurol Neurochir. 1969 Jan-Feb;72(1):121-8.
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Hereditary adult-onset Alexander's disease with palatal myoclonus, spastic paraparesis, and cerebellar ataxia.伴有腭肌阵挛、痉挛性截瘫和小脑共济失调的遗传性成人起病型亚历山大病
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Diffuse Rosenthal fiber formation in the adult: a report of four cases.成人弥漫性罗森塔尔纤维形成:4例报告
J Neuropathol Exp Neurol. 1973 Jul;32(3):424-36. doi: 10.1097/00005072-197307000-00007.

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