Table 2.

Recommended Evaluations Following Initial Diagnosis in Individuals with IBMPFD

System/ConcernEvaluation
Muscle Assessment of muscle strength, muscle wasting, & tendon reflexes. EMG &/or muscle biopsy may be necessary.
Cardiac Baseline echocardiogram & EKG
Lungs Baseline pulmonary function studies
Bone Blood alkaline phosphatase, urine pyridinoline studies, & bone scan studies followed by skeletal x-ray to evaluate distribution & severity of Paget disease of bone
Neurologic Baseline neuropsychological studies of behavior & mental status
Other Consultation w/clinical geneticist &/or genetic counselor

From: Inclusion Body Myopathy with Paget Disease of Bone and/or Frontotemporal Dementia

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