Objective: Primary objective:- 

1) To study the prevalence of non-motor manifestations in Huntington disease.

2) To study the correlation between non motor symptoms and Quality of life

Secondary objectives:-

1)To study difference between clinical profile of patients with motor and non-motor onset in Huntington disease.

Background: Non-motor symptoms appear early in Huntington’s disease (HD) and frequently go unrecognized or misdiagnosed. This study aimed to investigate the frequency of non-motor symptoms and assess the quality of life in patients with genetically confirmed HD.

Method: We prospectively enrolled patients with genetically confirmed HD from a single movement disorder clinic at a teaching hospital in Northern India. Motor symptoms were measured using a Quantified Neurological Examination, while non-motor symptoms were evaluated with several scales, including The Montreal Cognitive Assessment (MoCA), Apathy Evaluation Scale (clinician version), The Hospital Anxiety and Depression Scale (HADS), and The Pittsburgh Sleep Quality Index (PSQI). Quality of life (QOL) was assessed using the SF-36 questionnaire (SF36V2).

Results: During a six-month period, we recruited 15 patients, comprising 9 males (60%) and 6 females (40%). The mean (SD) age at onset was 45.64 (18), and the mean duration of symptoms was 8.3 years (6.5).Demographic and clinical details are provided in Table 1. Seven patients (47%) exhibited non-motor symptoms prior to motor symptoms at onset. All participants displayed behavioral disturbances and cognitive impairments upon recruitment. The frequency and severity scores of most non-motor symptoms are presented in Table 2. Apathy (93%) and executive dysfunction (86%) were the most reported non-motor manifestations. Patients with non-motor symptom onset had a QOL score of 56.48 (21.3), while those with motor symptom onset had a score of 51.16 (21.16).

Conclusion: Non-motor symptoms are frequent in HD and often precede the onset of motor symptoms in about half of the cohort. Patients with both non-motor and motor symptom onset show similar reductions in quality of life.

Demographic and clinical details of HD cohort

Non motor manifestations of HD cohort

References: 1) Elizabeth et al. Prevalent Nonmotor Symptoms Associated With Huntington Disease. Neurology. 2021 May 11;96(19):875-6.
2) Aldaz et al. Non-motor symptoms in Huntington’s disease: a comparative study with Parkinson’s disease. J Neurol. 2019;266(6):1340-50.
3) Hussain Z et al. “Clinical profile of genetically proven Huntington’s disease patients from Eastern India.” Annals of Indian Academy of Neurology. 2020 Jan 1;23(2):195-5.
4) Mestre TA et al. Rating scales for behavioral symptoms in Huntington’s disease: Critique and recommendations. Movement Disorders. 2016 Oct;31(10):1466-78

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MDS Abstracts - https://www.mdsabstracts.org/abstract/burden-of-non-motor-manifestations-in-huntingtons-disease-patients-a-cross-sectional-study-in-a-teaching-hospital-in-india/