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- Title
Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington's disease.
- Authors
Ratna, Nikhil; Kamble, Nitish L.; Venkatesh, Sowmya D.; Purushottam, Meera; Pal, Pramod K.; Jain, Sanjeev
- Abstract
<bold>Background: </bold>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.<bold>Methods: </bold>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.<bold>Results: </bold>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.<bold>Conclusions: </bold>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.
- Subjects
INDIA; HUNTINGTON disease; DISABILITIES; DISEASES; FAMILY history (Medicine); NEURODEGENERATION; INTELLECTUAL disabilities
- Publication
BMC Neurology, 2020, Vol 20, Issue 1, p1
- ISSN
1471-2377
- Publication type
journal article
- DOI
10.1186/s12883-020-01671-x