Abstract
ObjectiveHuntington’s disease is a rare, genetic, neurodegenerative disease characterized by a triad of cognitive, behavioral, and motor symptoms. The condition gradually results in increasing disability, loss of independence, and ultimately death. Our objective was to use United States claims data (which offer valuable insight into the natural history of disease) to compare the prevalent comorbidities of people with Huntington’s disease against matched controls with Parkinson’s disease or with no major neurodegenerative diseases (general population controls). We also assess medication use in people with Huntington’s disease.MethodsThis was a retrospective, observational study using data from the IBM MarketScan® Databases. Cases and controls were matched 1:1, and comorbidities were analyzed in each group during 2017. Medications were also assessed in the Huntington’s disease cohort. Eligible cases had ≥ 2 diagnostic codes for Huntington’s disease; controls had ≥ 2 codes for Parkinson’s disease (with no record of Huntington’s disease), or, for general population controls, no record of Huntington’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, or dementia.ResultsA total of 587 matched individuals were assessed in each cohort. Depression and anxiety were more common in Huntington’s disease versus Parkinson’s disease (odds ratios: 1.51 and 1.16, respectively). Other conditions more common in Huntington’s disease included dementia, communication/speech problems, dysphagia, and falls. The use of antidepressant (59.9%) and antipsychotic (39.5%) medications was frequent among Huntington’s disease cases.InterpretationThese data highlight the prevalence of psychiatric, cognitive, communication, swallowing, and mobility problems in people with Huntington’s disease, underscoring the need for holistic expert care of these individuals.
Highlights
Huntington’s disease (HD) is a rare, autosomal-dominant neurodegenerative disease that manifests as a triad of cognitive, behavioral, and motor symptoms
IBM MarketScanâ has previously been used to explore differences in healthcare interventions and direct medical costs among people with HD by disease stage.[30,31]. The aim of this retrospective, matched cohort study was to compare the prevalent comorbidities of people with HD, which may be true features of the disease or coexisting independent conditions, against matched controls – people with Parkinson’s disease (PD) or with no major neurodegenerative diseases (PD, amyotrophic lateral sclerosis [ALS], or dementia) – using US claims data
There was a greater proportion of females among HD cases compared to PD controls (59.5% vs. 44.0%)
Summary
Huntington’s disease (HD) is a rare, autosomal-dominant neurodegenerative disease that manifests as a triad of cognitive, behavioral, and motor symptoms. HD results in increasing disability, loss of independence, and death, with a median survival of 15 years after the onset of motor symptoms.[1] The disease is caused by a cytosine-adenineguanine (CAG) trinucleotide repeat expansion in the HTT gene, resulting in the production of a toxic mutant huntingtin (mHTT) protein.[2,3,4] The neuropathological abnormalities associated with HD appear to be caused by a dominant gain-of-function in mHTT.[5] Reducing the synthesis of this protein targets the primary disease mechanism; there are currently no treatments that can cure or modify HD progression. There is evidence that the prevalence in Western countries has increased over the past 50 years,[10] likely due to increases
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