Epidemiology and health care utilization of patients suffering from Huntington’s disease in Germany: real world evidence based on German claims data

Abstract

Background: Huntington's disease (HD) is a rare, genetic, neurodegenerative and ultimately fatal disease with no cure or progression-delaying treatment currently available. HD is characterized by a triad of cognitive, behavioural and motor symptoms. Evidence on epidemiology and management of HD is limited, especially for Germany. This study aims to estimate the incidence and prevalence of HD and analyze the current routine care based on German claims data. Methods: The source of data was a sample of the Institute for Applied Health Research Berlin (InGef) Research Database, comprising data of approximately four million insured persons from approximately 70 German statutory health insurances. The study was conducted in a retrospective cross-sectional design using 2015 and 2016 as a two-year observation period. At least two outpatient or inpatient ICD-10 codes for HD (ICD-10: G10) during the study period were required for case identification. Patients were considered incident if no HD diagnoses in the 4 years prior to the year of case identification were documented. Information on outpatient drug dispensations, medical aids and remedies were considered to describe the current treatment situation of HD patients. Results: A 2-year incidence of 1.8 per 100,000 persons (95%-Confidence interval (CI): 1.4-2.4) and a 2-year period prevalence of 9.3 per 100,000 persons (95%-CI: 8.3-10.4) was observed. The prevalence of HD increased with advancing age, peaking at 60-69 years (16.8 per 100,000 persons; 95%-CI: 13.4-21.0) and decreasing afterwards. The most frequently observed comorbidities and disease-associated symptoms in HD patients were depression (42.9%), dementia (37.7%), urinary incontinence (32.5%), extrapyramidal and movement disorders (30.5%), dysphagia (28.6%) and disorders of the lipoprotein metabolism (28.2%). The most common medications in HD patients were antipsychotics (66.9%), followed by antidepressants (45.1%). Anticonvulsants (16.6%), opioids (14.6%) and hypnotics (9.7%) were observed less frequently. Physical therapy was the most often used medical aid in HD patients (46.4%). Nursing services and speech therapy were used by 27.9 and 22.7% of HD patients, respectively, whereas use of psychotherapy was rare (3.2%). Conclusions: Based on a representative sample, this study provides new insights into the epidemiology and routine care of HD patients in Germany, and thus, may serve as a starting point for further research.

Identification of contributing genes of Huntington’s disease by machine learning

“…Huntington’s disease (HD) is an inherited disorder that results in neurodegeneration characterized by chorea, loss of coordination, cognitive decline, depression, and psychosis [ 1 ]. The prevalence of HD is 13.7/100,000 in North America [ 2 ] and 16.8/100,000 for the elderly in Western Europe [ 3 ]. The neurodegeneration of HD is featured by a general shrinkage of the brain, especially the medium spiny neurons (MSNs) of the striatum [ 4 ].…”

Incidence of Huntington disease in a northeastern Spanish region: A 13-year retrospective study at tertiary care centre

“…In other European countries, the incidence rates calculated were the following, all of them per 100.000 person-years: 0.3 in the province of Ferrara, northern Italy (1990-2009) [18]; 1.8 in Germany (2015-2016) [19]; 0.14 in Iceland (1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007) [20]; the minimum incidence rate was 0.22, being able to reach 0.44 in Greece (1995Greece ( -2008 [21]; 0.25 in Cyprus (1984-2015) [22]; and 0,72 in United Kingdom (1990-2010) [23]. So, the incidence of HD varies from 0.14 to 1.8 per 100.000 person-years in Europe.…”

Incidence of Huntington disease in a northeastern Spanish region: A 13-year retrospective study at tertiary care centre

“…In other European countries, the incidence rates calculated were the following, all of them per 100 000 person-years: 0.3 in the province of Ferrara, northern Italy (1990Italy ( -2009 [17]; 1.8 in Germany (2015-2016) [18]; 0.14 in Iceland (1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007) [19]; the minimum incidence rate was 0.22, being able to reach 0.44 in Greece (1995Greece ( -2008 [20]; 0.25 in Cyprus (1984-2015) [21]; and 0.72 in United Kingdom (1990-2010) [22]. So, the incidence of HD varies from 0.14 to 1.8 per 100 000 person-years in Europe.…”