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两姐妹患视神经脊髓炎(德维克综合征)。

Neuromyelitis optica (Devic's syndrome) in two sisters.

作者信息

Ch'ien L T, Medeiros M O, Belluomini J J, Lemmi H, Whitaker J N

出版信息

Clin Electroencephalogr. 1982 Jan;13(1):36-9. doi: 10.1177/155005948201300104.

DOI:10.1177/155005948201300104
PMID:7067151
Abstract

Neuromyelitis optica (Devic's syndrome) typically occurs in a sporadic pattern. We studied the disorder in two sisters, ages 6 and 10 years, who presented with sudden loss of vision at age 3 years, followed 5 months later by a transverse myelopathy. The older girl had only light perception in either eye, persistent paraparesis and progressive scoliosis; her sister had a visual acuity of 20/200 in both eyes and mild spinal cord dysfunction. Results of electrodiagnostic tests correlated well with the severity of clinical neurologic deficits and provided objective estimates of the extent of tissue damage in both the optic nerves and the spinal cord. The presence of Devic's syndrome in these two sisters could be coincidental; however, the possibility of a shared genetic factor(s) should be considered.

摘要

视神经脊髓炎(德维克综合征)通常呈散发性出现。我们研究了两名分别为6岁和10岁的姐妹所患的这种疾病,她们在3岁时突然失明,5个月后出现横贯性脊髓病。年长的女孩双眼仅有光感,存在持续性双下肢轻瘫和进行性脊柱侧弯;她的妹妹双眼视力为20/200,伴有轻度脊髓功能障碍。电诊断测试结果与临床神经功能缺损的严重程度密切相关,并对视神经和脊髓的组织损伤程度提供了客观评估。这两名姐妹患德维克综合征可能是巧合;然而,应考虑存在共同遗传因素的可能性。

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J Clin Neurol. 2011 Sep;7(3):115-27. doi: 10.3988/jcn.2011.7.3.115. Epub 2011 Sep 29.
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Familial neuromyelitis optica.家族性视神经脊髓炎。
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