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

• DOI: 10.21203/rs.2.10368/v2
• Publication Date: 2019-11-21T19:37:26Z
• Authors: Nikhil Ratna; Nitish L Kamble; Sowmya D V; Meera Purushottam; Pramod K Pal; Sanjeev Jain
• Keywords: Medical record

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’ deters symptomatic rehabilitative care, resulting poor quality of life sub-optimal outcomes. There no studies assessing burden outcomes from METHODS: We attempted evaluate individuals diagnosed have HD at our tertiary-care center between 2013 2016 clinical symptoms, functionality, mortality, follow up status through structured interview, data medical records & UHDRS TFC scoring. RESULTS: Of the 144 patients, 25% were untraceable, another 17 (11.8%) had already died. Mean age death duration illness time death, 53 years 7 respectively, perhaps due suicides other comorbidities early agewhich much shorter than Europe USA. patients who could contacted (n=81) assessed morbid symptoms total functional capacity (TFC). CAG repeat length score 44.2 7.5 respectively. Most (66%) stage I II benefit several interventions. correlated inversely with (p<0.0001). vast majority being taken care home, irrespective physical mental disability. was high prevalence psychiatric morbidity (91%) suicidal tendency (22%). Three died committed suicide, many families reported history family members. Only about half (57%) maintained regular follow-up. CONCLUSIONS: This study demonstrates follow-up rates, significant suicidality suboptimal survival durations highlighting need holistic appear amenable