To describe the clinical spectrum of patients diagnosed with 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody associated necrotizing myopathy.

HMGCR antibody associated necrotizing myopathy is often associated with the need for a multimodal, aggressive management, as well as its association with the use of statin class of medications.

Retrospective chart review of patients diagnosed with HMGCR antibody associated necrotizing myopathy at a single, outpatient neurology clinic between 2018 and 2020.

Four patients were diagnosed as having HMGCR antibody associated necrotizing myopathy. Patient 1 presented with painless quadriparesis. Patient 2 presented with progressive, bilateral, painful, upper and lower extremity weakness. Patient 3 presented with painless bilateral lower extremity weakness. Patient 4 presented with isolated elevated creatine kinase levels. All 4 patients underwent serological testing, and were found to have HMGCR antibodies. All 4 patients underwent needle electromyograms, which demonstrated irritable  proximal myopathy. The three symptomatic patients had a history of exposure to statins, while  the fourth asymptomatic patient, did not have have a history of exposure to statins. The three symptomatic patients were treated with intravenous steroids, with significant improvement of symptoms, as well as reduction in creative kinase levels

HMGCR antibody associated necrotizing myopathy, can have a variable clinical presentation, including one where the patient is clinically asymptomatic. Furthermore, intravenous steroids can be used effectively to manage patients with this disease.