The prevalence and healthcare utilization of rare neurological diseases in Hong Kong: 2014-2018

Abstract

Background: Rare neurological diseases (RND) constitute a large proportion of rare diseases (RD). However, there has not been any study dedicated to the elucidation of prevalence and healthcare utilization pattern of RND and its subtypes. There is also a lack of understanding into the state of pharmacological research in RND. Methods: A list of ICD-10 codes corresponding to RNDs was compiled with adaptation from the Orphanet Classification of Rare Diseases, and classified into rare epilepsy, movementrelated, neurocutaneous, neuroimmune, neurometabolic and neurodegenerative, neuromuscular and other RNDs. Using the Clinical Data Analysis and Reporting System (CDARS), which holds public hospital healthcare records of Hong Kong in an anonymous manner, we calculated the prevalence and healthcare utilization of patients RNDs between 2014 and 2018. The list of RNDs was also used to look up pharmacological research related to RNDs within the International Clinical Trials Registry Platform (ICTRP) between 2009-2018. Results: The prevalence of RND in Hong Kong is 3.6 in 1000, without major difference between paediatric and adult population. Patients with RNDs have frequent emergency department, inpatient and outpatient healthcare utilization, with average annual cost per patient estimated at HKD 175404 (USD 22488) excluding pharmacological costs. Different categories of RND also showed different patterns of healthcare utilization. On the other hand, there were only 677 RND-related pharmacological trials in the past decade, and no such trial was found for up to 78% of RNDs. Only 41 (6%) of the trials were conducted within Chinese speaking communities. Discussion: This is the first comprehensive review on the prevalence, healthcare utilization, and state of pharmacological research in RNDs. It shows high healthcare utilization rate amongst patients with RND in the Emergency Department, inpatient and outpatient settings. It also shows a wide research gap in many of the RNDs, particularly within Chinese speaking communities. We call for better attention and tailored healthcare for these patients.