Dependent and paranoid personality patterns in myotonic dystrophy type 1

Area of Science:

• Neurology
• Psychiatry
• Genetics

Background:

• Myotonic dystrophy type 1 (DM1) is a multisystem disorder with variable clinical presentations.
• Personality patterns in DM1 patients are not well-characterized, yet may influence disease management and quality of life.

Purpose of the Study:

• To determine the frequency and types of personality patterns in DM1 patients.
• To correlate personality traits with clinical data and genetic factors.
• To assess the impact of personality patterns on quality of life (QoL) in DM1.

Main Methods:

• A cross-sectional study involving 62 DM1 patients.
• Utilized the Muscular Impairment Rating Scale, Raven's Standard Progressive Matrices (RSPM), Millon Multiaxial Clinical Inventory I (MMCI), SF-36, and Individualized Neuromuscular Quality of Life (INQoL) questionnaires.
• Clinical interviews were conducted to assess personality impairment.

Main Results:

• Over 75% of patients had at least one pathological personality trait, with 58.1% showing clinically significant impairment.
• Dependent (51.6%) and paranoid (38.7%) personality patterns were most prevalent.
• Paranoid traits correlated with lower education, lower RSPM scores, and higher CTG repeat counts; paranoid personality was associated with worse QoL.

Conclusions:

• Clinically significant personality impairment is common in DM1, with dependent and paranoid patterns being most frequent.
• Paranoid personality traits significantly reduce QoL in DM1 patients.
• Identifying and addressing paranoid personality traits may offer new avenues for symptomatic therapy in DM1.