Summary
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.
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Beal, J. A.: Morphogenesis of the Hirano body in neurons of the squirrel monkey dorsal horn. J. Neurocytol.7, 395–403 (1978)
Berenberg, R. A., Pellock, J. M., Dimauro, S., Schotland, D. L., Bonilla, E., Eastwood, A., Hays, A., Vicale, C. T., Behrens, M., Chutorian, A., Rowland, L. P.: Lumping or splitting? “Ophthalmoplegia-plus” or Kearns-Sayre syndrome? Ann. Neurol.1, 37–54 (1977)
Cavanagh, J. B., Blakemore, W. F., Kyu, M. H.: Fibrillary accumulations in oligodendroglial processes of rats subjected to portocaval anastomosis. J. Neurol. Sci.14, 143–152 (1971)
Cullen, R. F., Daroff, R. B., Popoff, N.: Early onset external ophthalmoplegia and retinopathy: a distinct clinical and neuropathologic syndrome. Neurology (Minneap.)23, 406–408 (1973)
Daroff, R. B., Solitare, G. B., Pincus, J. H., Glaser, G. H.: Spongiform encephalopathy with chronic progressive external ophthalmoplegia: central ophthalmoplegia mimicking ocular myopathy. Neurology (Minneap.)16, 161–169 (1966)
Fisher, E. R., Gonzalez, A. R., Khurana, R. C., Danowski, T. S.: Unique, concentrically laminated, membranous inclusions in myofibers. Am. J. Clin. Pathol.58, 239–244 (1972)
Field, E. J., Mathews, J. D., Raine, C. S.: Electron microscopic observations on the cerebellar cortex in Kuru. J. Neurol. Sci.8, 209–224 (1969)
Gessaga, E. C., Anzil, A. P.: Rod-shaped filamentous inclusions and other ultrastructural features in a cerebellar astrocytoma. Acta Neuropathol. (Berl.)33, 119–127 (1975)
Gonatas, N. K.: A generalized disorder of nervous system, skeletal muscle, and heart resembling Refsum's disease and Hurler's syndrome: II. Ultrastructure. Am. J. Med.42, 169–178 (1967)
Hadfield, M. G., Martinez, A. J., Gilmartin, R. C.: Progressive multifocal leukoencephalopathy with paramyxovirus-like structures, Hirano bodies and neurofibrillary tangles. Acta Neuropathol. (Berl.)27, 277–288 (1974)
Hirano, A., Malamud, N., Elizan, T. S., Kurland, L. T.: Amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam. Arch. Neurol.15, 35–51 (1966)
Hirano, A., Dembitzer, H. M., Kurland L. T., Zimmermann, H. M.: The fine structure of some intraganglionic alterations. Neurofibrillary tangles, granulovacuolar bodies and ‘rok-like’ structures as seen in Guam amyotrophic lateral sclerosis and parkinsonism-dementia complex. J. Neuropathol. Exp. Neurol.27, 167–182 (1968)
Hirano, A., Dembitzer, H. M.: Eosinophilic rod-like structures in myelinated fibers of hamster spinal roots. Neuropathol. Appl. Neurobiol.2, 225–232 (1976)
Jager, B. V., Fred, H. L., Butler R. B., Carnes, W. H.: Occurrence of retinal pigmentation, ophthalmoplegia, ataxia, deafness, and heart block. Am. J. Med.29, 888–893 (1960)
Kearns, T. P., Sayre, G. P.: Retinitis pigmentosa, external ophthalmoplegia, and complete heart block. Arch. Ophthalmol.60, 280–289 (1958)
Luft, R., Ikkos, D., Palmieri, G., ERnster, L., Afzelius, B.: A case of severe hypermetabolism of non thyroid origin with a defect in the maintenance of mitochondrial respiratory control: A correlated clinical, biochemical, and morphological study. J. Clin. Invest.41, 1776–1804 (1962)
Ogata, J., Budzilovich, G. N., Cravioto, H.: A study of rod-like structures (Hirano body) in 240 normal and pathological brains. Acta Neuropathol. (Berl.)21, 61–67 (1972)
Olson, W., Engel, W. K., Walsh, G. O., Einangler, R.: Oculocraniosomatic neuromuscular disease with “ragged red” fibers. Arch. Neurol.26, 193–211 (1972)
Schochet, S. S., Jr., McCormick, W. F.: Ultrastructure of Hirano bodies. Acta Neuropathol. (Berl.)21, 50–60 (1972)
Toga, M., Bérard-Badier, M., Gambarelli, D., Pinsard, N., Hassoun, J.: Un cas de dystrophie neuroaxonal infantile ou maladie de Seitelberger. III. Étude ultrastructurale du muscle strié. Acta Neuropathol. (Berl.)18, 327–341 (1971)
Tomonaga, M.: Ultrastructure of Hirano bodies. Acta Neuropathol. (Berl.)28, 365–366 (1974)
Yagishita, S., Kimura, S.: Infantile neuroaxonal dystrophy (Seitelberger's Disease). Histological and electron microscopical study of two cases. Acta Neuropathol. (Berl.)29, 115–126 (1974)
Yamamura, Y., Atsumi, T., Sato, T., Iwata, I.: Progressive external ophthalmoplegia. An autopsy case pathologically characterised by spinocerebellar degeneration with polyneuropathy. Adv. Neurol. Sci. (Tokyo)19, 46–48 (1975)
Zintz, R., Villiger, W.: Elektronmikroskopische Befunde bei 3 Fällen von chronisch progressiver okulärer Muskeldystrophie. Ophthalmologica153, 439–459 (1967)
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Atsumi, T., Yamamura, Y., Sato, T. et al. Hirano bodies in the axon of peripheral nerves in a case with progressive external ophthalmoplegia with multisystemic involvements. Acta Neuropathol 49, 95–100 (1980). https://doi.org/10.1007/BF00690748
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DOI: https://doi.org/10.1007/BF00690748