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Negative symptoms in Huntington’s disease

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Abstract

Background

Apathy is the commonest psychiatric manifestation in Huntington’s disease (HD). We investigated negative psychiatric symptoms—as determined by the Scale for the Assessment of Negative Psychiatric Symptoms (SANS)—in early and intermediate HD patients, hypothesizing that such symptoms would be prominent and constitute a more comprehensive and clinically relevant assessment than apathy alone. We also assessed relations between negative symptoms and disease stage, mood, motor, and cognitive disturbances.

Methods

Thirty-five stage 1 and twenty-nine stage 2 consecutive adult HD outpatients were administered SANS; the Scale for the Assessment of Positive Psychiatric Symptoms (SAPS); the motor section of the Unified Huntington’s Disease Rating Scale (UHDRS); Total Functional Capacity (TFC); and instruments to assess cognition, anxiety, and depression.

Results

The groups had similar age, education, and CAG length. Scores on the Hamilton depression and anxiety scales, and SAPS were similar. Negative symptoms were pervasive in the entire series. Illness duration, UHDRS, TFC, cognition, and SANS scores were significantly worse in stage 2. Mini Mental State Examination (MMSE) and SAPS scores were significantly (multiple regression) associated with SANS score, while Hamilton depression and UHDRS scores were not. SANS score was also associated with stage after removing the cognition-related domains of alogia and attention.

Conclusions

Negative symptoms are pervasive in HD but more severe in stage 2. The associations of SANS with MMSE and SAPS suggest impaired cognition and thinking as important in generating negative symptoms. SANS appears useful for revealing a wide range of negative symptoms in HD.

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Acknowledgements

We thank Don Ward for help with the English language, and Professor G. Pezzoli for support and useful suggestions.

Funding

Dr. Dominga Paridi was in part supported by funds from the Euro-HD Registry project. No other author received specific funding for this study.

Author information

Author notes

  1. Paola Soliveri & Francesca Del Sorbo

    Present address: Parkinson Institute, ASST Gaetano Pini-CTO, Via Bignami 1, 20126, Milan, Italy

  2. Dominga Paridi

    Present address: Fondazione Grigioni per il Morbo di Parkinson, Via Bignami 1, 20126, Milan, Italy

  3. Floriano Girotti

    Present address: Department of Neurology and Laboratory of Neuroscience, San Luca IRCCS Auxological Institute, Piazzale Brescia 20, 20149, Milan, Italy

Authors and Affiliations

  1. Parkinson and Movement Disorders Unit, IRCCS Foundation Carlo Besta Neurological Institute, Via Celoria 11, 20133, Milan, Italy

    Paola Soliveri, Dominga Paridi, Francesca Del Sorbo & Floriano Girotti

  2. Department of Research and Clinical Development, Scientific Directorate, IRCCS Foundation Carlo Besta Neurological Institute, Via Celoria 11, 20133, Milan, Italy

    Irene Tramacere

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  1. Paola Soliveri
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Correspondence to Paola Soliveri.

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Ethical approval

The study was approved by the Besta ethical committee (EC Number 7/04, 2014). All participants provided written informed consent.

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Drs. Soliveri and Girotti are co-senior authors

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Soliveri, P., Paridi, D., Del Sorbo, F. et al. Negative symptoms in Huntington’s disease. Neurol Sci 43, 3695–3701 (2022). https://doi.org/10.1007/s10072-021-05787-x

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