Summary
We examined the Onufrowicz nucleus (Onuf's nucleus) of ten sporadic amyotrophic lateral sclerosis (ALS) patients with light and electron microscopic and immunohistochemical methods. Neurons in the Onuf's nucleus of ALS patients were better preserved than those in anterior horn cells. However, some showed morphological changes in the nucleus, namely, central chromatolytic changes, Bunina bodies, ubiquitin-positive filaments and spheroids. The Onuf's neurons of ALS patients showed more argentophilia than those of non-ALS patients. Electron microscopic observations revealed that neurofilaments were relatively more numerous in the Onuf's neurons of ALS patients. Bunina bodies and degenerated neurites were also seen in the Onuf's nucleus. In conclusion, the Onuf's nucleus in sporadic ALS patients showed some morphological changes similar to those noted in anterior horn cells.
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References
Bunina TL (1962) On intracellular inclusions in familial amyotrophic lateral sclerosis. Korsakov J Neuropathol Psychiatry 62: 1293–1299
Carpenter S (1986) Proxomal axonal enlargement in motor neuron disease. Neurology 18: 841–851
Hattori T, Hirayama K, Yasuda K, Shimazaki J (1983) Disturbance of micturition in amyotrophic lateral sclerosis. Clin Neurol (Tokyo) 23: 224–227
Hirano A, Inoue E (1979) Early pathological changes of amyotrophic lateral sclerosis. Electron microscopic study of chromatolysis, spheroids and Bunina bodies. Neurol Med (Tokyo) 13: 148–160
Hirano A, Donnenfeld H, Sasaki S, Nakano I (1984) Fine structural observation of neurofilamentous changes in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43: 461–470
Hirano A, Nakano I, Kurland LT, Mulder DW, Holley PW, Saccomanno G (1984) Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43: 471–480
Iwata M, Hirano A (1977) A method for the macroscopic identification of human lumbo-sacral spinal cord levels. Neurol Med (Tokyo) 7: 126–131
Iwata M, Hirano A (1978) Sparing of the Onufrowicz nucleus in sacral anterior horn lesions. Ann Neurol 4: 245–249
Konishi A, Sato M, Mizuno N, Itoh K, Nomura S, Sugimoto T (1978) An electron microscope study of the areas of the Onuf's nucleus in the cat. Brain Res 156: 333–338
Kojima H, Furuta Y, Fujita M, Fujioka Y, Nagashima K (1989) Onuf's motoneuron is resistant to poliovirus. J Neurol Sci 93: 85–92
Kuzuhara S, Kanazawa I, Nakanishi T (1980) Topographic localization of the Onuf's nuclear neurons innervating the rectal and vesical striated sphincter muscles: a retrograde fluorescent double labeling in cat and dog. Neurosci Lett 16: 125–130
Leigh PN, Anderton BH, Dodson A, Gallo J-M, Swash M, Power DM (1988) Ubiquitin deposits in anterior horn cells in motor neurone disease. Neurosci Lett 93: 197–203
Mannen T (1988) Amyotrophic lateral scleosis. In: Toyokura Y (ed) Intractable neurological disorders. University of Tokyo Press, Tokyo, pp 45–57
Mannen T, Iwata M, Toyokura Y, Nagashima K (1977) Preservation of a certain motoneuron group of the sacral cord in amyotrophic lateral sclerosis: its clinical significance. J Neurol Neurosurg Psychiatry 40: 464–469
Mannen T, Iwata M, Toyokura Y, Nagashima K (1982) The Onuf's nucleus and the external anal sphincter muscles in amyotrophic lateral sclerosis and Shy-Drager syndrome. Acta Neuropathol (Berl) 58: 255–260
Matsumoto S, Hirano A, Goto S (1990) Ubiquitin-immunoreactive filamentous inclusions in anterior horn cells of Guamanian and non-Guamanian amyotrophic lateral sclerosis. Acta Neuropathol 80: 233–238
Murayama S, Mori H, Ihara Y, Bouldin TW, Suzuki K, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis. Ann Neurol 27: 137–148
Nakamura H, Fujii Y, Yorita T, Takahashi K, Agari M (1978) Ultrastructural changes of the anterior horn of the sacral cord in amyotrophic lateral slerosis with special reference to the group X of Onuf. In: Tsubaki T (ed) Annual report of the Reserch Committie of motor neuron disease (1977). The Ministry of Health and Welfare of Japan, Tokyo, pp 90–92
Okamoto K, Hirai S, Shoji M, Harigaya Y, Fukuda T (1991) Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis. Acta Neuropathol 81:349–353
Onufrowicz B (1899) Notes on the arrangement and function of the cell groups in the sacral region of the spinal cord. J Nerv Ment Dis 26: 498–504
Onufrowicz B (1900) On the arrangement and function of the cell group in the sacral region of the spinal cord in man. Arch Neurol Psychopathol 3: 387–412
Sato M, Mizuno N, Konishi A (1980) Localization of motoneurons innervating perineal muscles: a HRP study in cat. Brain Res 140: 149–154
Sung JH (1979) Autonomic neurons affected by lipid storage in the spinal cord in Fabry's disease: distribution of autonomic neurons in the sacral cord. J Neuropathol Exp Neurol 38: 87–98
Sung JH, Mastri AE (1980) Spinal autonomic neurons in Werdnig-Hoffmann disease, mannosidosis, and Hurler's syndrome: distribution of autonomic neurons in the sacral spinal cord. J Nueropathol Exp Neurol 39: 441–451
Tomonaga M (1980) Selective appearance of Bunina bodies in amyotrophic lateral sclerosis. J Neurol 223:259–267
Yase Y, Kihira T, Yoshida S, Uebayashi Y, Tanaka S, Yoshimasu F (1987) An ALS case with conglomerate inclusions in Onuf's nuclei — Morphological, morphometric and metal analytical study of the 2nd sacral cord. In: Nakanishi T (ed) Annual report of the Reserch Committie of CNS Degenerative Disease (1986). The Ministry of Health and Welfare of Japan. Tokyo, pp 62–66
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Okamoto, K., Hirai, S., Ishiguro, K. et al. Light and electron microscopic and immunohistochemical observations of the Onuf's nucleus of amyotrophic lateral sclerosis. Acta Neuropathol 81, 610–614 (1991). https://doi.org/10.1007/BF00296370
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DOI: https://doi.org/10.1007/BF00296370