Neuroleptic malignant syndrome in Huntington disease

IF 4.5 2区医学 Q1 CLINICAL NEUROLOGY

European Journal of Neurology Pub Date : 2024-10-23 DOI:10.1111/ene.16442

Antonio Funcis, Beatrice Ravera, Paola Zinzi, Marcella Solito, Martina Petracca, Paolo Calabresi, Anna Rita Bentivoglio

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Abstract

Background and Purpose

Despite the wide use of dopamine receptor blocking agents (DRBAs) in Huntington disease (HD), neuroleptic malignant syndrome (NMS) is rarely described in this population. The aim of this study was to assess NMS prevalence in a large cohort of HD patients and explore the main associated risk factors.

Methods

In 2023, an HD patient was admitted to our neurology department due to NMS. Starting from the case description, we performed a narrative review of the literature of NMS cases in HD, reviewed data from the fifth dataset of the Enroll-HD (a longitudinal, observational, global study of families with HD) study (PDS5) selecting HD patients treated with DRBAs and/or tetrabenazine (TBZ) who presented at least one of the core symptoms of NMS (rigidity and hyperthermia), and collected data to investigate prevalence of NMS and identify risk factors.

Results

In the Enroll-HD PDS5 dataset, we identified 5108 of 11,569 HD patients who were undergoing DRBA and/or TBZ treatment. Only one patient, a Caucasian man of 46 years, undergoing clozapine and valproate treatment, had a registered diagnosis of NMS.

Conclusions

NMS in HD patients is seldom described. This could be due to an underestimation of this condition. There are no available objective NMS diagnostic criteria at present, and the existence of atypical forms of NMS further complicates diagnosis. Advanced disease stage, rigid–akinetic phenotype, abrupt therapy changes, polytherapy, and dehydration are key risk factors, most of which are preventable through awareness and caution in managing medications in the HD population.

Abstract Image

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亨廷顿病的神经性恶性综合征。

背景和目的：尽管亨廷顿病（HD）广泛使用多巴胺受体阻滞剂（DRBAs），但神经性恶性综合征（NMS）在这一人群中却很少见。本研究的目的是评估NMS在一大批HD患者中的患病率，并探索主要的相关风险因素：2023 年，一名 HD 患者因 NMS 被送入我们的神经内科。从病例描述开始，我们对有关 HD NMS 病例的文献进行了叙事性回顾，回顾了 Enroll-HD（一项针对 HD 患者家庭的纵向、观察性、全球性研究）研究（PDS5）的第五个数据集的数据，这些数据集选择了接受 DRBAs 和/或四苯嗪（TBZ）治疗、至少出现一种 NMS 核心症状（僵直和高热）的 HD 患者，并收集数据以调查 NMS 的患病率并确定风险因素：在 Enroll-HD PDS5 数据集中，我们发现 11,569 名 HD 患者中有 5108 人正在接受 DRBA 和/或 TBZ 治疗。只有一名正在接受氯氮平和丙戊酸钠治疗的 46 岁白种男性患者登记诊断为 NMS：HD患者的NMS很少被描述。结论：HD 患者的 NMS 很少被描述，这可能是由于低估了这一病症。目前尚无客观的 NMS 诊断标准，而非典型 NMS 的存在又使诊断更加复杂。疾病晚期、僵化-激酶表型、突然改变治疗方案、多疗程治疗和脱水是主要的风险因素，其中大部分都是可以通过对 HD 患者的认识和谨慎用药来预防的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

European Journal of Neurology 医学-临床神经学

CiteScore

9.70

自引率

2.00%

发文量

418

审稿时长

1 months

期刊介绍： The European Journal of Neurology is the official journal of the European Academy of Neurology and covers all areas of clinical and basic research in neurology, including pre-clinical research of immediate translational value for new potential treatments. Emphasis is placed on major diseases of large clinical and socio-economic importance (dementia, stroke, epilepsy, headache, multiple sclerosis, movement disorders, and infectious diseases).