200 Section - Multisegmental Focal Myositis: Tackling Orofacial, Respiratory, and Mobility Challenges

Case Diagnosis: Focal myositis is a rare inflammatory myopathy usually limited to a single muscle group. Multisegmental involvement, particularly affecting adductor muscles, diaphragm, and masseter, is exceptional and generates complex functional compromise. This case highlights the pivotal role of Physical and Rehabilitation Medicine (PRM) in  ventilatory management, masticatory rehabilitation, and motor reconditioning.

Case Description:

A 46-year-old man, diagnosed with focal myositis in 2013 after left thigh swelling, elevated CK, and reduced muscle strength. MRI revealed diffuse edema and thickening of the posteromedial thigh muscles, predominantly the left adductor magnus. Muscle biopsy confirmed inflammatory myositis, and EMG showed myopathic changes with necrosis. High-dose prednisolone was initiated, followed by azathioprine and IVIG in 2014, resulting in partial improvement.

During follow-up, he developed fatigue, exertional dyspnea, morning headaches, and non-restorative sleep. Pulmonary function tests showed restrictive syndrome (FVC 58%), diaphragmatic ultrasound revealed pillar atrophy, and EMG confirmed denervation. He was enrolled in a PRM-guided NIV program with nightly BiPAP, improving symptoms; discontinuation in 2016 led to deterioration, requiring re-titration. By 2017, left masseter atrophy and masticatory dysfunction emerged, necessitating targeted orofacial rehabilitation. He now maintains regular 3-month NIV follow-ups, with 7 hours of nightly use, stable ventilation, and no apnea or desaturation events.

Discussions:

PRM was pivotal in managing this patient’s multisegmental myositis. NIV titration and interface optimization stabilized respiratory function, while orofacial therapy improved chewing and quality of life. Lower-limb rehabilitation preserved mobility, enabling return to federated padel. This case highlights the value of coordinated, multimodal rehabilitation in rare myopathies, showing that early and ongoing PRM intervention can prevent functional decline, enhance daily performance, and maintain long-term independence.

Conclusions:

This case demonstrates the role of PRM in managing complex multisegmental focal myositis. Through coordinated ventilatory management, treatment of masticatory dysfunction, and motor rehabilitation, PRM was fundamental in maintaining respiratory stability, functional capacity, and quality of life.