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[多重耐药性发作性睡病]

[Multidrug resistant narcolepsy].

作者信息

González-García A M, Morán-Sánchez M, Sánchez-Serrano A R, Vizcaya-Gaona J A, El Berdei-Montero Y, Morán-Sánchez J C

机构信息

Hospital Universitario de Salamanca, Salamanca, España.

Hospital Universitario La Paz, Madrid, España.

出版信息

Rev Neurol. 2023 Jul 28;77(s01):S13-S16. doi: 10.33588/rn.77s01.2023198.

Abstract

INTRODUCTION

Narcolepsy type 1 is a focal degenerative disease of the hypothalamus that selectively affects orexin (hypocretin)-producing neurons. It presents multiple clinical manifestations, both in wakefulness and in sleep. The symptoms are often so disruptive that they cause enormous suffering and impair patients' quality of life. Although a non-pharmacological approach is sometimes sufficient, the vast majority of patients need medication for adequate clinical management.

CASE REPORT

A male who, at 43 years of age, began to present acutely with excessive daytime sleepiness and episodes of cataplexy. After a thorough examination, he was diagnosed with narcolepsy type 1. Throughout the course of the disease, he was prescribed antidepressants, neurostimulants and sodium oxybate, in monotherapy or in combination. The response to pharmacological treatment was insufficient and accompanied by numerous side effects. Following the introduction of pitolisant, there was a marked improvement in his symptoms and a reduction in the dose of the other drugs and their adverse effects was achieved.

CONCLUSION

A number of measures are now available to address the cardinal symptoms of the disease, although there are still cases that are resistant to anti-narcoleptic treatment. Drugs with mechanisms of action that act upon receptors in the histaminergic system can be very useful in these cases.

摘要

引言

1型发作性睡病是一种下丘脑局灶性退行性疾病,选择性地影响产生食欲素(下丘脑泌素)的神经元。它在清醒和睡眠状态下均有多种临床表现。这些症状往往极具破坏性,会给患者带来巨大痛苦并损害其生活质量。尽管有时非药物治疗方法就足够了,但绝大多数患者需要药物治疗以进行充分的临床管理。

病例报告

一名43岁男性开始急性出现白天过度嗜睡和猝倒发作。经过全面检查,他被诊断为1型发作性睡病。在疾病过程中,他接受了抗抑郁药、神经兴奋剂和羟丁酸钠的单一疗法或联合治疗。药物治疗的反应不足且伴有许多副作用。使用匹托品后,他的症状有明显改善,其他药物的剂量减少,且副作用也有所减轻。

结论

现在有多种措施可用于解决该疾病的主要症状,尽管仍有一些病例对抗发作性睡病治疗有抗性。作用于组胺能系统受体的具有特定作用机制的药物在这些病例中可能非常有用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b66e/10831720/94303728ac8e/RN-77-S13-g001.jpg

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