Background Huntington’s disease (HD) is a neurodegenerative condition with neuropsychiatric symptoms. Progression of the disease, as well as social attitude to the symptoms and knowledge about the disease may also contribute to the symptoms.
Aim To access the functional and behavioural aspects in genetically confirmed HD patients during follow up.
Methods HD Patients (N = 62) at the National Institute of Mental and Neurosciences, Bengaluru, India were contacted 24 months after testing, to access their functional and behavioural aspects using Unified Huntington’s disease rating scale.
Results Of 62 HD patients contacted, we could get information for only 38 patients. Two patients had died in this period, and 24 patients were unreachable (total patients followed =36; M:F = 61%:39%). About 40% patients were lost to follow-up. The mean duration of illness was 57 months (range of expanded CAG allele is 39–59 repeats) and mean time since testing was 27 months. About 50% showed aggressive behaviour, low self-esteem and irritable behaviour; 33% had sadness, anxiety and suicidal thoughts. Psychotic symptoms, obsessions and compulsions were seen in a few individuals. No behavioural symptoms were seen in 15%, while another 15% had multiple behavioural symptoms. More than 50% were impaired in their domestic chores and 30% are impaired in ability to handle finances, occupation and activities of daily living.
Conclusion and implications Most of the patients contacted on follow-up showed moderate disability in behavioural aspects and most were able to handle the disease at home care. The burden of care for severe neurodegenerative disease falls on the family. Within the first decade of onset of symptoms, many patients had psycho-social disabilities, and moderate impairment. All these subjects were staying with their families. The outcomes of those who were not in contact remains unknown. It thus highlights the urgent need to develop services for these patients, even in resource poor countries like India.
- Unified Huntington’s disease rating scale-UHDRS
- Domestic chores – DC