The pathologic hallmark of OPMD on muscle biopsy is filamentous intranuclear inclusions composed of the misfolded polyalanine expanded PABPN1 protein [which is similar to other trinucleotide repeat diseases such as Huntington’s disease (HD)], though aggregates of dysmorphic mitochondria have also been observed (11), which may be the mechanism by which the LPS is preferentially affected. The gene discussed is PABPN1; the disease is Huntington disease.