Hirano bodies in the axon of peripheral nerves in a case with progressive external ophthalmoplegia with multisystemic involvements

Inclusions, ultrastructurally identical with Hirano bodies which were previously believed to be limited to the central nervous system (CNS), were found both within peripheral myelinated nerve axons and within terminal axons of neuromuscular junctions in the ocular muscles of an autopsied woman who had suffered from progressive external ophthalmoplegia with multisystemic involvements. Electron micrographs showed the inclusions to consist of beaded filaments or lattice-like structures with filamentous elements continuing onto neurofilaments in the axon. The corelation of these new pathological findings in peripheral nerve axons and ophthalmoplegia is discussed.