Neuromuscular Case Vignettes

Case 8: Differential diagnosis

Deterioration of weakness despite the discontinuation of atorvastatin argues against statin myotoxicity. However, autoimmune  / necrotizing myopathy may develop in patients exposed to statins, manifesting by progressive weakness and persistet elevation of CK despite discontinuation of statin.  Seropositivity for autoantibodies targeting 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), the pharmacologic target of statins, is now commercially available to assess for statin induced autoimmune myopathy1. Celiac disease could result in proximal weakness and pain in association with rickets and vitamin D deficiency, mainly in children 2. The presence of monoclonal gammopathy raises the possibility of amyloid myopathy and late onset nemaline rod myopathy. The finding of mixed myopathic and neurogenic findings in the EMG is non-specific, but suggests chronic myositis, inclusion body myositis and nemaline rod myopathy. A muscle biopsy (quadriceps) was done as the next diagnostic step.

References:

1.         Mohassel P, Mammen AL. Statin-associated autoimmune myopathy and anti-HMGCR autoantibodies. Muscle & nerve 2013;48:477-483.

2.         Suthar R, Sankhyan N, Thapa BR, Singhi P. Proximal Myopathy: A Rare Presentation of Celiac Disease. Journal of child neurology 2013;28:1485-1488.