Joseph R. Archer, BS
Medical Student
Tulane University School of Medicine
New Orleans, Louisiana, United States
Colby M. V. Shores, n/a
Undergraduate Research Assistant
University of Pittsburgh
Penfield, New York, United States
Gabriela Figueiredo Pucci, MD
Neurology Resident
University of Pittsburgh Medical Center
Pittsburgh, Pennsylvania, United States
Brendan McNeish, MD
Assistant Professor
University of Pittsburgh
PIttsburgh, Pennsylvania, United States
A 76-year-old woman was admitted with acute progressive bilateral ptosis and leg weakness. Over the previous month she completed 2 cycles of nivolumab and ipilimumab (ICIs) for metastatic melanoma. Admission labs showed creatinine kinase levels of 9256 units/L. Electrodiagnostic studies demonstrated an irritable myopathy and labs revealed an Anti-Striated Muscle Antibody. Treatment included high dose intravenous corticosteroids and plasmapheresis.
Ten days later, the patient was switched to oral corticosteroids with improved ptosis but was complicated by hematemesis the next day. A Duotube was placed for risk of aspiration. Three days later she presented with abdominal pain and CT identified a bowel perforation with subsequent septic shock. The perforation was repaired, and she remained on ventilation with intravenous antibiotics and continuous renal replacement therapy due to declining kidney function. Two days later necrotic cecum was removed. Difficulty coming off ventilation four days later led to family discussions and elective terminal extubation.
Discussions: ICIs are increasingly being prescribed for advanced cancers but are associated with autoimmune adverse events. Despite some ICI associated adverse events being associated with a positive prognosis, neurological adverse events are linked to increased risk of disability and death. Anti-Striated Muscle Antibody has been reported with ICI associated myasthenia gravis but has never been reported in an isolated ICI myopathy. This case demonstrates the importance of early recognition of disease onset, treatment, components of multisystemic impairment, appropriate serologic testing, and need of interdisciplinary rehabilitation during neuromuscular adverse events associated with ICIs.
Conclusions: Neuromuscular and rehabilitation providers should be aware of the disability and possibility of death in ICI associated myopathy and potentially in cases with seropositvity to anti-striated muscle antibody.
