Summary
A 30-year-old man had had chronic progressive wasting and weakness of muscles for 17 years. A muscle biopsy 5 years prior to death revealed myopathic changes with rimmed vacuoles and intranuclear inclusions which corresponded to “inclusion body myositis”. At autopsy, intranuclear inclusions were observed in neurons, oligodendroglia, and inparenchymal cells of the adrenal medulla. Ultrastructurally, the inclusions in muscles, nervous tissue, and adrenal medulla were identical and consisted of abnormal tubulolinear structures measuring 10–20 nm in diameter.
Similar inclusions have been reported in muscles with “inclusion body myositis” and in the nervous system with “neuronal intranuclear hyaline inclusion disease”, respectively. Absence of clinical symptoms related to the CNS and adrenal gland, and well-preserved parenchymal cells in these organs of our patient suggest a benign nature of the disease except in the muscular system. Attempts to isolate a virus from the brain were fruitless. This patient may serve to connect both diseases in muscles and the nervous system, and to disclose the etilogy of these inclusions.
Similar content being viewed by others
References
Carpenter S, Karpati G, Heller I, Eisen A (1978) Inclusion body myositis: A distinct variety of idiopathic inflammatory myopathy. Neurology (Minneap) 28:8–17
Chou S-M (1968) Myxovirus-like structures and accompanying nuclear changes in chronic polymyositis. Arch Pathol 86:649–658
Danon MJ, Reyes MG, Perurena OH, Masdeu JC, Manaligod JR (1982) Inclusion body myositis: A corticosteroid-resistant idiopathic inflammatory myopathy. Arch Neurol 39:760–764
Dubowitz V, Brooke MH (1973) Muscle biopsy: A modern approach. Saunders, London Philadelphia Toronto
Fukuhara N, Kumamoto T, Tsubaki T (1980) Rimmed vacuoles. Acta Neuropathol (Berl) 51:229–235
Hughes JH, Esiri MM (1975) Ultrastructural studies in human polymyositis. J Neurol Sci 25:347–360
Janota I (1979) Case report. Widespread intranuclear neuronal corpuscles (Marinesco bodies) associated with familial spinal degeneration with cranial and peripheral nerve involvement. Neuropathol Appl Neurobiol 5:311–317
Jerusalem F, Baumgartner G, Wyler R (1972) Virus-ähnliche Einschlüsse bei chronischen neuromuskulären Prozessen: Elektronemikroskopische Biopsiebefunde von 2 Fällen. Arch Psychiatr Nervenkr 215:148–166
Ketelsen UP, Beckmann R, Zimmermann H, Sauer M (1977) Inclusion body myositis: A ‘slow virus’ infection of skeletal musculature? Klin Wochenschr 55:1063–1066
Michaud J, Gilbert JJ (1981) Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a new case with light and electron microscopic studies. Acta Neuropathol (Berl) 54:113–119
Oteruelo FT (1976) Intranuclear inclusions in a myopathy of late onset. Virchows Arch [Cell Pathol] 20:319–324
Russell WC, Newman C, Williamson DH (1975) A simple cytochemical technique for demonstration of DNA in cells infected with mycoplasmas and viruses. Nature 253:461–462
Sato T, Walker DL, Peters HA, Reese HH, Chou S-M (1971) Chronic polymyositis and myxovirus-like inclusions. Electron microscopic and viral studies. Arch Neurol 24:409–418
Sung JH (1980) Light-, fluorescence-, and electron-microscopic features of neuronal intranuclear hyaline inclusions associated with multisystem atrophy. Acta Neuropathol (Berl) 50:115–120
Sung JH, Ramirez-Lassepas M, Mastri AR, Larkin SM (1980) An unusual degenerative disorder of neurons associated with a novel intranuclear hyaline inclusion (neuronal intranuclear hyaline inclusion disease). A clinico-pathological study of a case. J Neuropathol Exp Neurol 39:107–130
Yunis EJ, Samaha FJ (1971) Inclusion body myositis. Lab Invest 25:240–248
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tateishi, J., Nagara, H., Ohta, M. et al. Intranuclear inclusions in muscle, nervous tissue, and adrenal gland. Acta Neuropathol 63, 24–32 (1984). https://doi.org/10.1007/BF00688467
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00688467