OBJECTIVES

: Myotonic Disorders (MD) are genetic diseases encompassing two forms: Myotonic Dystrophy which is a common and severe multi-system disorder affecting various organs including the skeletal, smooth and cardiac muscles and and Non-Dystrophic myotonias with no overt progressive muscle weakness and no systemic features. With regards to upcoming innovative therapies; there is a lack of data in literature on the patient path and the management of the disease in hospital. We performed an analysis on the French DRG-based information system (PMSI) which aimed to describe patients characteristics and comorbidities, the reasons for hospital admission and mortality.

METHODS

: Hospital stays were extracted from the PMSI database, from 2012 to 2016, using the ICD-10 diagnosis codes G71.1 (Myotonic Disorders). A descriptive analysis of stays and an exploratory analysis of death during hospitalisation were performed.

RESULTS

: During the 5 years period, 6666 Patients with MD were identified. The mean age was 42.9 years (sd = 19.1), and 50.4% were females. Numerous hospital admissions occurred including 6312 for MD and 11258 with MD. The average Length of Stay (LOS) was 1.53 and 6.24 days in patients hospitalized for and with MD, respectively. Main reasons for hospitalisations were examination and polysomnography. The most frequent medical procedure was measurment of the respiratory muscles by pression at mouth (34.3%). During hospitalization, 540 indivuduals died, mainly patients aged 50-70 years (60%). Death was most likely due to respiratory defects and cardiac conduction disorders. Besides, other neurologic conditions were frequently coded during hospitalisation in MD patients.

CONCLUSIONS

: These preliminary results highlight the main characteristcs, comorbididties, reasons for hospitalisation, and LOS in MD patients. Hospital admission frequency and mortality appear to be high in this disease population. Further analyses are needed to better identify the patient pathway in individuals with MD.