Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington’s disease

Ratna, N., Kamble, N. L., Venkatesh, S. D. , Purushottam, M., Pal, P. K. and Jain, S. (2020) Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington’s disease. BMC Neurology, 20, 87. (doi: 10.1186/s12883-020-01671-x) (PMID:32164608) (PMCID:PMC7068943)

Abstract

Background: Huntington’s disease (HD), an inherited, often late-onset, neurodegenerative disorder, is considered to be a rare, orphan disease. Research into its genetic correlates and services for those affected are inadequate in most low-middle income countries, including India. The apparent ‘incurability’ often deters symptomatic and rehabilitative care, resulting in poor quality of life and sub-optimal outcomes. There are no studies assessing disease burden and outcomes from India. Methods: We attempted to evaluate individuals diagnosed to have HD at our tertiary-care center between 2013 and 2016 for clinical symptoms, functionality, mortality, follow up status through a structured interview, clinical data from medical records and UHDRS-TFC scoring. Results: Of the 144 patients, 25% were untraceable, and another 17 (11.8%) had already died. Mean age at death and duration of illness at the time of death, were 53 years and 7 years respectively, perhaps due to suicides and other comorbidities at an early age. The patients who could be contacted (n = 81) were assessed for morbidity and total functional capacity (TFC). Mean CAG repeat length and TFC score were 44.2 and 7.5 respectively. Most individuals (66%) were in TFC stage I and II and could perhaps benefit from several interventions. The TFC score correlated inversely with duration of illness (p < 0.0001). The majority were being taken care of at home, irrespective of the physical and mental disability. There was a high prevalence of psychiatric morbidity (91%) including suicidal tendency (22%). Three of the 17 who died had committed suicide, and several other families reported suicidal history in other family members. Only about half the patients (57%) maintained a regular clinical follow-up. Conclusions: This study demonstrates the poor follow-up rates, significant suicidality and other psychiatric symptoms, sub-optimal survival durations and functional outcomes highlighting the need for holistic care for the majority who appear to be amenable to interventions.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Venkatesh, Dr Sowmya
• Authors: Ratna, N., Kamble, N. L., Venkatesh, S. D., Purushottam, M., Pal, P. K., and Jain, S.
• College/School: College of Medical Veterinary and Life Sciences > School of Molecular Biosciences
• Journal Name: BMC Neurology
• Publisher: BioMed Central
• ISSN: 1471-2377
• ISSN (Online): 1471-2377
• Copyright Holders: Copyright © 2020 The Author(s)
• First Published: First published in BMC Neurology 20:87
• Publisher Policy: Reproduced under a Creative Commons license