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血浆置换成功治疗皮肤神经综合征。

Successful treatment of dermato-neuro syndrome with plasmapheresis.

机构信息

Department of Internal Medicine, Providence-Providence Park Hospital, Southfield, Michigan, USA

Department of Internal Medicine, Providence-Providence Park Hospital, Southfield, Michigan, USA.

出版信息

BMJ Case Rep. 2020 Dec 29;13(12):e237170. doi: 10.1136/bcr-2020-237170.

Abstract

Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of dermato-neuro syndrome in a 71-year-old man with a known history of scleromyxoedema. The patient initially presented with encephalopathy which quickly progressed to generalised tonic-clonic seizures and coma. While his presentation fits with other, although rare, cases of dermato-neuro syndrome, it is imperative to rule out lethal, more common causes of altered mentation. Due to the rarity and difficulty in diagnosis of dermato-neuro syndrome, there is a significant debate regarding the optimal management as there are no standardised treatment protocols. In our case, the patient was successfully treated with plasmapheresis resulting in improved neurologic function.

摘要

精神状态改变可能有很多原因,从紧急颅内病变到更隐匿的全身性中毒性病因都有可能。我们报告了一例罕见的皮神经综合征病例,患者为 71 岁男性,既往有硬肿性黏液水肿病史。该患者最初表现为脑病,很快进展为全面强直阵挛发作和昏迷。虽然他的表现符合其他罕见的皮神经综合征病例,但必须排除更常见的致命性精神状态改变的原因。由于皮神经综合征的罕见性和诊断难度,关于最佳治疗方法存在很大争议,因为目前尚无标准化的治疗方案。在我们的病例中,患者成功接受了血浆置换治疗,神经功能得到改善。

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本文引用的文献

1
Acute Encephalitic Syndrome Induced by Scleromyxedema.硬肿性皮病所致急性脑炎综合征。
Am J Med Sci. 2020 Aug;360(2):192-195. doi: 10.1016/j.amjms.2020.05.018. Epub 2020 May 20.
2
Scleromyxedema treatment: a systematic review and update.硬肿性黏液水肿的治疗:系统评价与更新。
Int J Dermatol. 2020 Oct;59(10):1191-1201. doi: 10.1111/ijd.14888. Epub 2020 May 2.
3
Dermato-neuro syndrome in a case of scleromyxedema.硬皮病黏液水肿一例中的皮肤神经综合征
Eur J Rheumatol. 2015 Dec;2(4):160-162. doi: 10.5152/eurjrheum.2015.0116. Epub 2015 Apr 22.
6
Dermato-neuro syndrome associated with scleromyxedema.与硬化性黏液水肿相关的皮肤神经综合征
Indian J Dermatol Venereol Leprol. 2015 Sep-Oct;81(5):519-21. doi: 10.4103/0378-6323.162344.
7
Does dermatoneuro syndrome have a viral aetiology?皮肤神经综合征有病毒病因吗?
Clin Exp Dermatol. 2016 Jan;41(1):53-6. doi: 10.1111/ced.12698. Epub 2015 Jul 14.

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