OBJECTIVES: Huntington’s disease (HD) is a neurodegenerative disorder manifested with a triad of signs and symptoms, including motor, cognitive, and behavioral features. Data on the epidemiology and burden of HD in Israel are scarce. This analysis assessed the epidemiology, patient characteristics, and treatment patterns of HD patients in Israel.

METHODS: This was a retrospective analysis using a nationwide health plan database (Maccabi Healthcare Services), which represents 25% of the Israeli population. The study included adult members alive between 2013-2018 with HD (ICD-9: 333.4) diagnosis based on either: neurologist diagnosis, hospital-discharge report, medicine preauthorization or "chronic patient" diagnosis, or ≥2 HD diagnoses by any other healthcare professional any time prior to 2019. Period prevalence incidence and mortality rates for 2016-2018 were assessed. For incidence, a 1-year minimum enrollment was required. Therapy initiation with tetrabenazine, benzodiazepines, and amantadine, as well as proportions of patients initiating antipsychotics were assessed.

RESULTS: A total of 106 patients were diagnosed. Mean age of diagnosis was 49 years (SD=14), 57.5% were female, and the largest percentage of patients (48%) were of medium socioeconomic status. Mean period prevalence and annual incidence of HD for 2016-2018 were 4.45 per 100,000 people and 0.24 per 100,000 people, respectively. Mean annual mortality rate was 0.18 per 100,000 people and median survival time was 13 years after diagnosis (95% CI: 11-15). Most patients (62%) visited a movement-disorder clinic at least once, and 31% had ≥1 fracture after HD diagnosis. Furthermore, 67%, 65%, and 11% of patients initiated tetrabenazine, benzodiazepines, and amantadine, respectively. 25% and 45% of patients initiated first and second generation antipsychotics, respectively.

CONCLUSIONS: The prevalence of HD in Israel is lower than northern and western Europe, however, similar to Mediterranean and southern European countries. Further real-world research is needed to assess the healthcare resource utilization and costs implication of the disease.