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非多发性硬化相关的典型和非典型白质疾病:我们在印度一家三级医疗中心过去两年中对儿童和成人的经验。

Non-multiple-Sclerosis-Related Typical and Atypical White Matter Disorders: Our Experience in the Last 2 Years in Both Children and Adults From a Tertiary Care Center in India.

作者信息

Chandra Sadanandavalli Retnaswami, Ramanujam Chakravarthula Nitin, Kalya Vyasaraj Kishore, Christopher Rita, Padmanabha Hansashree, Jagadish Annapureddy, Arshad Faheem, Gohel Abhishek

机构信息

Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

Department of Neurochemistry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

出版信息

J Pediatr Neurosci. 2019 Jan-Mar;14(1):20-29. doi: 10.4103/jpn.JPN_37_19.

Abstract

INTRODUCTION

White matter signal changes are common in wide spectrum of disorders other than primary demyelinating diseases. Knowledge into their characteristics is of great relevance as treatment options are available in most cases, if diagnosed early.

PATIENT AND METHODS

Patients both children and adults who showed radiological evidences of leukoencephalopathy which was not due to primary demyelinating disorders were evaluated in detail.

RESULTS

There were a total of 55 patients in the last 2 years. 58% patients were <10 years, 16% were <20years, 9% were <30 years and the rest of the patients were 40 and above. Commonest condition was ALD, followed by SSPE and Unclassified group. There were 3 cases each of MLD, Krabbe's, Vanishing White Matter Disease and Hypomyelination.

DISCUSSION AND CONCLUSION

White matter disorders belong to a wide spectrum of illnesses which varies from degeneration to a spectrum of other disorders. Correlating the clinical, radiological and other laboratory data are mandatory for proper diagnosis. Those who belong to older age with shorter duration and uncharacterized radiological features suffered from acquired treatable causes.

摘要

引言

白质信号改变在原发性脱髓鞘疾病以外的广泛疾病谱中很常见。了解其特征具有重要意义,因为如果早期诊断,大多数情况下都有可用的治疗选择。

患者与方法

对表现出非原发性脱髓鞘疾病所致白质脑病影像学证据的儿童和成人患者进行了详细评估。

结果

在过去两年中共有55例患者。58%的患者年龄小于10岁,16%小于20岁,9%小于30岁,其余患者年龄在40岁及以上。最常见的疾病是肾上腺脑白质营养不良(ALD),其次是亚急性硬化性全脑炎(SSPE)和未分类组。各有3例异染性脑白质营养不良(MLD)、克拉伯病、消失性白质病和髓鞘形成低下。

讨论与结论

白质疾病属于从变性到其他一系列疾病的广泛疾病谱。为了正确诊断,必须将临床、影像学和其他实验室数据相关联。那些年龄较大、病程较短且影像学特征不典型的患者患有可治疗的后天性病因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/afa0/6601113/d635382e8c07/JPN-14-20-g001.jpg

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