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Objective: Pain has not been considered to be a major part of the symptom manifestation of Duchenne muscular dystrophy (DMD). However, a growing number of studies indicated that chronic pain is a common symptom for patients with neuromuscular disease. Previous studies on pain in DMD are studies of a broad group of diseases called neuromuscular disease, not a single disease group of DMD. Most of previous researches focused on pain intensity and under-assessed regarding other pain domain such as pain quality. However, it is important to classify the pain as nociceptive or neuropathic pain to provide adequate treatment. The aim of this study was to evaluate comprehensive characteristics of pain with single-disease group of DMD. Method: We studied characteristics of pain and stiffness in patients with DMD using a self-reported questionnaire validated. The questionnaire packet included pain frequency (Likert scale), pain duration (Likert scale), painDETECT Questionnaire (PD-Q), Brief Pain Inventory-Short Form, and Upper Limb Short Questionnaire. To detect neuropathic component of pain, we used PD-Q as a reliable screening tool with high sensitivity and specificity. Thirty-five patients with DMD completed a comprehensive evaluation including a detailed interview and structured questionnaire. Results: The majority of patients with DMD (age 15.49±5.21 years) were non-ambulatory(77.1%) and they have contracture(82.9%). More than two-third of the patients received steroid therapy(82.9%) and used orthotics such as spinal brace, sit device, and ankle-foot orthosis(71.4%). Nineteen of thirty-five subjects(54%) reported pain at least several times per year. Moderate to severe pain intensity (Neumeric rating scale≥4) was observed in 7 of 35 subjects on current intensity (20%), 13 of 35 subjects on worst pain intensity over the last four weeks(37.1%), and 10 of 35 subjects on average pain intensity over the last four weeks(28.6%), respectively. Pain was reported as negatively affecting many aspects of quality of life including general activity(31.4%), mood(34.3%), social relationship(20%), and sleep(26.7%) on patients with DMD. The Pain in the legs and feet(57.1%) was most commonly reported and followed by shoulders and chest(11.4%), pelvic region(5.7%), lower back(5.7%), and abdomen(5.7%). Most of subjects with pain reported pain for relatively short period of time, less than an hour. According to PD-Q, most of patients have nociceptive pain than neuropathic pain. Thirteen of thirty-five subjects reported stiffness(37%). The most frequent site was legs and feet(25.7%), followed by arms and hands(8.6)%, and pelvic region(5.7%). The presence of stiffness and the site of stiffness are not completely consistent with the pain. Conclusion: The results of this study suggest that pain is clinically significant and could affect quality of life in patients with DMD. These data also support the necessity for interventions to ameliorate pain in this patients group. |
