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Open Access 16-11-2023 | Huntington's Disease | Guidelines

Symptomatic treatment options for Huntington’s disease (guidelines of the German Neurological Society)

Authors: Carsten Saft, Jean-Marc Burgunder, Matthias Dose, Hans Heinrich Jung, Regina Katzenschlager, Josef Priller, Huu Phuc Nguyen, Kathrin Reetz, Ralf Reilmann, Klaus Seppi, Georg Bernhard Landwehrmeyer

Published in: Neurological Research and Practice | Issue 1/2023

Abstract

Introduction

Ameliorating symptoms and signs of Huntington’s disease (HD) is essential to care but can be challenging and hard to achieve. The pharmacological treatment of motor signs (e.g. chorea) may favorably or unfavorably impact other facets of the disease phenotype (such as mood and cognition). Similarly, pharmacotherapy for behavioral issues may modify the motor phenotype. Sometimes synergistic effects can be achieved. In patients undergoing pragmatic polypharmacological therapy, emerging complaints may stem from the employed medications' side effects, a possibility that needs to be considered. It is recommended to clearly and precisely delineate the targeted signs and symptoms (e.g., chorea, myoclonus, bradykinesia, Parkinsonism, or dystonia). Evidence from randomized controlled trials (RCTs) is limited.

Summary or definition of the topic

Therefore, the guidelines prepared for the German Neurological Society (DGN) for German-speaking countries intentionally extend beyond evidence from RCTs and aim to synthesize evidence from RCTs and recommendations of experienced clinicians.

Recommendations

First-line treatment for chorea is critically discussed, and a preference in prescription practice for using tiapride instead of tetrabenazine is noted. In severe chorea, combining two antidopaminergic drugs with a postsynaptic (e.g., tiapride) and presynaptic mode of action (e.g., tetrabenazine) is discussed as a potentially helpful strategy. Sedative side effects of both classes of compounds can be used to improve sleep if the highest dosage of the day is given at night. Risperidone, in some cases, may ameliorate irritability but also chorea and sleep disorders. Olanzapine can be helpful in the treatment of weight loss and chorea, and quetiapine as a mood stabilizer with an antidepressant effect.

Conclusions

Since most HD patients simultaneously suffer from distinct motor signs and distinct psychiatric/behavioral symptoms, treatment should be individually adapted.

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Title: Symptomatic treatment options for Huntington’s disease (guidelines of the German Neurological Society)
Authors: Carsten Saft
Jean-Marc Burgunder
Matthias Dose
Hans Heinrich Jung
Regina Katzenschlager
Josef Priller
Huu Phuc Nguyen
Kathrin Reetz
Ralf Reilmann
Klaus Seppi
Georg Bernhard Landwehrmeyer
Publication date: 16-11-2023
Publisher: BioMed Central
Keywords: Huntington's Disease
Neurologic Therapy
Antidepressant Drugs
Antidepressant Drugs
Antipsychotics
Antipsychotics
Risperidone
Published in: Neurological Research and Practice / Issue 1/2023
Electronic ISSN: 2524-3489
DOI: https://doi.org/10.1186/s42466-023-00285-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Symptomatic treatment options for Huntington’s disease (guidelines of the German Neurological Society)

Abstract

Introduction

Summary or definition of the topic

Recommendations

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Summary or definition of the topic

Recommendations

Conclusions

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