Neuroleptic malignant syndrome in Huntington disease.

Huntington disease dopamine neuroleptic malignant syndrome neuroleptics tetrabenazine

Journal

European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311

Informations de publication

Date de publication:
23 Oct 2024
Historique:
revised: 11 07 2024
received: 08 04 2024
accepted: 30 07 2024
medline: 24 10 2024
pubmed: 24 10 2024
entrez: 24 10 2024
Statut: aheadofprint

Résumé

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. 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. 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. 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.

Sections du résumé

BACKGROUND AND PURPOSE OBJECTIVE
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 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 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 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.

Identifiants

pubmed: 39444167
doi: 10.1111/ene.16442
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e16442

Informations de copyright

© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

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Auteurs

Antonio Funcis (A)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.

Beatrice Ravera (B)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.

Paola Zinzi (P)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.
Institute of Neurology, Policlinic A. Gemelli Foundation, Rome, Italy.

Marcella Solito (M)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.
Institute of Neurology, Policlinic A. Gemelli Foundation, Rome, Italy.

Martina Petracca (M)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.
Institute of Neurology, Policlinic A. Gemelli Foundation, Rome, Italy.

Paolo Calabresi (P)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.
Institute of Neurology, Policlinic A. Gemelli Foundation, Rome, Italy.

Anna Rita Bentivoglio (AR)

Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy.
Institute of Neurology, Policlinic A. Gemelli Foundation, Rome, Italy.

Classifications MeSH