BACKGROUND: Myotonic dystrophy type 2/proximal myotonic myopathy (DM2/PROMM) is an autosomal dominant multisystem disorder. Musculoskeletal pain is one of its frequent symptoms but also occurs in other chronic noninflammatory muscle disorders (OMD). OBJECTIVES: To characterize the phenotype of DM2/PROMM-associated musculoskeletal pain and to test whether it shows features distinct from OMD. SETTING: Outpatient clinic for patients with neuromuscular disorders, university hospital. PATIENTS: Twenty-four patients with DM2/PROMM (12 women and 12 men; median age, 57 years) and 24 age- and sex-matched patients with OMD consecutively recruited during a 3-year period were examined for musculoskeletal pain. METHODS: Standardized pain assessment; McGill Pain Questionnaire; depression score; and quantification of pain thresholds to blunt pressure on limb muscles with analgometer. RESULTS: Unlike patients with OMD who have musculoskeletal pain, patients with DM2/PROMM distinguished a wide spectrum of coexisting pain types. The major pain type in patients with DM2/PROMM was exercise-related, temperature-modulated, and palpation-induced, whereas, cramps were rare. In 8 of the patients with DM2/PROMM and in 3 of the patients with OMD, musculoskeletal pain was the most disabling symptom. CONCLUSION: Besides many similarities, DM2/PROMM-associated musculoskeletal pain shows features distinct from OMD.
BACKGROUND:Myotonic dystrophy type 2/proximal myotonic myopathy (DM2/PROMM) is an autosomal dominant multisystem disorder. Musculoskeletal pain is one of its frequent symptoms but also occurs in other chronic noninflammatory muscle disorders (OMD). OBJECTIVES: To characterize the phenotype of DM2/PROMM-associated musculoskeletal pain and to test whether it shows features distinct from OMD. SETTING:Outpatient clinic for patients with neuromuscular disorders, university hospital. PATIENTS: Twenty-four patients with DM2/PROMM (12 women and 12 men; median age, 57 years) and 24 age- and sex-matched patients with OMD consecutively recruited during a 3-year period were examined for musculoskeletal pain. METHODS: Standardized pain assessment; McGill Pain Questionnaire; depression score; and quantification of pain thresholds to blunt pressure on limb muscles with analgometer. RESULTS: Unlike patients with OMD who have musculoskeletal pain, patients with DM2/PROMM distinguished a wide spectrum of coexisting pain types. The major pain type in patients with DM2/PROMM was exercise-related, temperature-modulated, and palpation-induced, whereas, cramps were rare. In 8 of the patients with DM2/PROMM and in 3 of the patients with OMD, musculoskeletal pain was the most disabling symptom. CONCLUSION: Besides many similarities, DM2/PROMM-associated musculoskeletal pain shows features distinct from OMD.
Authors: James E Hilbert; Tetsuo Ashizawa; John W Day; Elizabeth A Luebbe; William B Martens; Michael P McDermott; Rabi Tawil; Charles A Thornton; Richard T Moxley Journal: J Neurol Date: 2013-06-27 Impact factor: 4.849
Authors: Jordi Miró; Katherine A Raichle; Gregory T Carter; Sarah A O'Brien; Richard T Abresch; Craig M McDonald; Mark P Jensen Journal: Am J Hosp Palliat Care Date: 2009-05-04 Impact factor: 2.500