Pain in adult myotonic dystrophy type 1: relation to function and gender

Abstract

Background Pain is prevalent in myotonic dystrophy 1 (DM1). This study investigated whether CTG repeat size, disease duration, BMI and motor and psychological function were related to pain in adult patients with DM1, and if there were gender differences regarding intensity and location of pain. Method Cross-sectional design. Pain was investigated in 50 genetically confirmed DM1 patients by combining clinical assessment and self-reports of pain intensity and locations. Pain scoring results were related to CTG size, disease duration, muscle strength, walking capacity measured by 6-min walk test, activity of daily life by Katz ADL Index, respiratory function by Forced Vital Capacity and BMI. In addition, the degree of reported pain was related to Quality of life measured by WHOQOL-BREF; fatigue was measured by Fatigue severity scale; psychological functions were measured by Beck Depression Inventory, Beck Anxiety Inventory, IQ and Autism spectrum Quotient. Results Pain was reported in 84% of the patients and was significantly correlated with CTG size (r = 0.28 p = 0.050), disease duration (r = 0.38 p = 0.007), quality of life (r = − 0.37 p = 0.009), fatigue (r = 0.33 p = 0.02) and forced vital capacity (r = − 0.51, p = 0.005). Significant gender differences, with higher scores for females, were documented. In male subjects the number of pain locations was significantly correlated with quality of life and the autism quotient. In females, pain intensity was significantly correlated with activity, respiratory function and BMI. Conclusions Pain in DM1 was prevalent, with a strong association to lung function and other aspects of the disease. Significant gender differences were present for pain intensity and number of pain locations. How pain was related to other symptoms differed between male and female subjects. Our findings highlight the importance of assessments of pain in DM1 patients.

Retrospective Observational Study on the Characteristics of Pain and Associated Factors of Breakthrough Pain in Advanced Cancer Patients

“…Pain was considered to have strong influence on ADL difficulties [ 35 ]. Interestingly, we found the severe background pain was associated with better activity of daily life, which was contradictory to some conclusions that pain intensity was significantly negatively correlated with ADL [ 36 , 37 ]. The possible interpretation was patients with better ADL more easily triggered the pain due to more activity.…”

Gameterapia na marcha e equilíbrio na distrofia miotônica tipo 1: relato de caso

“…Solbakken et al 4 mostra áreas onde os pacientes sentem dores, o que reafirma essa queixa apresentada pela paciente. Outro achado foi a melhora da força muscular em MMII, corroborando com outro estudo que focava na força muscular de pacientes com DM1, e que aplicaram carga de 60% de 1RM.…”

“…Foi visto em seu estudo que pacientes que receberam maior atenção e explicação do caso, apresentaram melhora nos problemas psicológicos e déficits cognitivos, até na depressão, sendo assim, um dos motivos para a melhora no bem-estar e na saúde mental. Outro sintoma clínico encontrado foi dor na região torácica relatada pela paciente em algumas sessões de atendimento.Solbakken et al4 mostra áreas onde os pacientes sentem dores, o que reafirma essa queixa apresentada pela paciente. Outro achado foi a melhora da força muscular em MMII, corroborando com outro estudo que focava na força muscular de pacientes com DM1, e que aplicaram carga de 60% de 1RM.…”

New developments in myotonic dystrophies from a multisystemic perspective

“…The myotonia is prominent, further impairing hands’ function and myalgia is frequently reported. Chronic musculoskeletal pain is prominent in both DM1 and DM2 patients and probably results from peripheral mechanisms within the muscle and central sensitization [13,28 ▪ ,29 ▪ ]. In DM2, muscle weakness is proximal and axial (neck flexors, hip flexors and knee extensors) without the tendency to generalize to distal muscles.…”