A previously well, 8-year-old boy presented to the emergency room with a 2.5-month history of worsening skin rash, fatigue, progressive muscle weakness and recent 2-day history of choking on food and drinks. He has had increasing difficulty negotiating stairs and reported several recent falls. A rheumatic disease was suspected and he was admitted to the hospital for further work-up. Investigations revealed diffuse, symmetrical muscle weakness (most prominent in the proximal muscle groups), a waddling gait, and a positive Gower's sign. Heliotrope rash and Gottron's papules (on hands, elbows, and knees) were present. On nailfold capillaroscopy, capillary dilation and dropout was observed. Video fluoroscopic feeding assessment confirmed clinical dysphagia and parenteral nutrition was initiated. Laboratory investigations revealed elevated serum muscle enzymes and acute phase reactants. Magnetic resonance imaging (MRI), electromyography (EMG), nerve conduction studies (NCS), and muscle biopsy were consistent with myositis. The diagnosis of juvenile dermatomyositis (JDM) was made. On the second day after hospital admission, a physical therapy consult for evaluation and treatment was requested.
What examination signs may be associated with this diagnosis?
What are the most appropriate examination tests?
What are the examination priorities?
What are the most appropriate physical therapy outcome measures for muscle strength and endurance?
Describe a physical therapy plan of care based on each stage of the disease.?
Identify referrals to other medical team members.
- DYSPHAGIA: Difficulty (or inability) to swallow
- GOWER'S SIGN: Physical sign indicating proximal lower limb weakness; describes when a patient flexes his trunk, places his hands on his knees and moves them up his legs in order to extend the trunk
- GOTTRON'S PAPULES: Erythematous, maculopapular rash most commonly found on extensor surfaces of the hands (especially proximal interphalangeal joints), as well as elbows, knees and less often, medial malleoli; erythematous rash without papules in this distribution is known as Gottron's sign
- HELIOTROPE RASH: Violet-colored rash of (primarily) the upper eyelids
- NAILFOLD CAPILLAROSCOPY: Non-invasive test in which skin capillaries at the base of the fingernail (nailfold) are examined under a microscope to evaluate vascular abnormalities; test assists in diagnosis, classification, and prediction of clinical outcome in connective tissue disorders
- VIDEOFLUOROSCOPY FEEDING STUDY: Also known as dysphagia barium swallow; test to determine oral and pharyngeal swallowing difficulties that uses videotaping, barium, and x-ray while a patient swallows varying consistencies of food and liquids
Identify the signs and symptoms of JDM.
Identify valid and reliable outcome tools to measure muscle strength and endurance in this population.
Recognize the complications of JDM.
Understand the medical treatments for JDM and their relevance to physical therapy management.
Design an appropriate physical therapy program for a child with JDM in the hospital.
PT considerations during management of the child with newly diagnosed juvenile dermatomyositis: