Neuropsychiatric Burden in Huntington’s Disease

Paoli, Ricardo Augusto; Botturi, A.; Ciammola, Andrea; Silani, Vincenzo; Prunas, Cecilia; Lucchiari, Claudio; Zugno, Elisa; Caletti, Elisabetta

doi:10.3390/brainsci7060067

Cited by 114 publications

(96 citation statements)

References 92 publications

(157 reference statements)

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“…The lifetime prevalence of psychiatric morbidity among HD patients varies between 33 and 76% as per various reports, with depression, apathy, irritability, aggressiveness, impulsiveness, obsessions, and compulsions being commonly reported [19,24]. Nearly onethird patients in our sample reported non-motor symptoms at onset, and majority (91.4%) had psychiatric and behavioral problems, of varying severity, during the course of the disease.…”

Section: Discussionsupporting

confidence: 58%

“…This pessimism, the lack of services, and the socio-economic factors (expenses involved in treatment of a person who was anyway 'destined' to die) perhaps contributed in no small part to the stark difference. The estimated suicide rate in HD patients is 4-6 times higher than in the general population [19]. We too observed a high prevalence of suicidal tendencies (22%) in our sample, which is more than that was reported in other HD studies [20,21] and that in the general Indian population (0.01%) [22].…”

Section: Discussioncontrasting

confidence: 55%

See 1 more Smart Citation

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

et al. 2020

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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.

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Section: Discussionsupporting

confidence: 58%

Section: Discussioncontrasting

confidence: 55%

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

et al. 2020

View full text Add to dashboard Cite

show abstract

“…The estimated suicide rate in HD patients is 4-6 times higher than in the general population (19). We too observed a high prevalence of suicidal tendencies (22%) in our sample, which is more than that reported in other studies in HD patients (20,21) and that in the general Indian population (0.01%) (22).…”

Section: Discussioncontrasting

confidence: 58%

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

Ratna

Kamble

Sowmya

et al. 2019

Preprint

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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 & UHDRS TFC scoring. RESULTS: Of the 144 patients, 25% were untraceable, and another 17 (11.8%) had already died. Mean age at and duration of illness at the time of death, were 53 years and 7 years respectively, which is much shorter than in Europe and USA. The patients who could be contacted (n=81) were assessed for symptomatic 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 vast 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 many 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 & other psychiatric symptoms, suboptimal survival durations and functional outcomes highlighting the need for holistic care for the majority who appear to be amenable for interventions.

show abstract

“…The estimated suicide rate in HD patients is 4-6 times higher than in the general population [19]. We too observed a high prevalence of suicidal tendencies (22%) in our sample, which is more than that was reported in other HD studies [20,21] and that in the general Indian population (0.01%) [22].…”

Section: Discussioncontrasting

confidence: 53%

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

Ratna

Kamble

Sowmya

et al. 2020

Preprint

View full text Add to dashboard Cite

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.

show abstract

Neuropsychiatric Burden in Huntington’s Disease

Cited by 114 publications

References 92 publications

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

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

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

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

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