Summary
Argentophilic intracytoplasmic glial inclusions were recently reported in olivo-ponto-cerebellar atrophy (OPCA). We examined the brains of 3 cases of OPCA [2 with striato-nigral degeneration (SND) and 1 without SND], 1 case of pure autonomic failure (PAF) without pathology of OPCA or SND, as well as 36 controls including 2 cases of Holmes' type cerebellar cortical atrophy and 2 cases of Joseph's disease. Although the inclusions were tubulin-positive, the immunoreactivity was different from that of the dendrites. Electron microscopically, the microtubular structures composing the inclusion were fuzzy with granular material. These findings may indicate that the microtubules composing the inclusions are modified. Inclusion-bearing cells appeared to be oligodendrocytes while many of them had larger and lighter nuclei than those of normal-looking oligodendrocytes without the inclusions. The inclusions were widely distributed in a characteristic fashion beyond the typical lesions of OPCA, SND and PAF. The distribution pattern was essentially the same in the case of PAF and 3 cases of OPCA irrespective of the presence or absence of OPCA or SND lesions. In contrast, argentophilic inclusions were not observed in other types of spinocerebellar degeneration, in Holmes' type cerebellar cortical atrophy or in Joseph's disease. It is suggested, in line with other studies, that the inclusion may be specific to OPCA and related disorders which include PAF and a useful marker to distinguish OPCA from other neurodegenerative diseases.
Similar content being viewed by others
References
Arima K, Inose T, Akaishi T, Mukoyama M, Suhara T (1989) Intracytoplasmic argentophilic inclusions in multiple system atrophy (Oppenheimer) (in Japanese). Abstracts of the 30th Japanese Congress of Neuropathology. Japanese Society of Neuropathology, Kyoto, p 93
Bannister R (1988) Introduction and classification. In: Bannister R (ed) Autonomic failure, 2nd edn. Oxford University Press, Oxford, pp 1–20
Binder LI, Frankfurter A, Rebhun LI (1985) The distribution of tau in mammalian nervous system. J Cell Biol 101:1371–1378
Graham JC, Oppenheimer DR (1969) Orthostatic hypotension in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry 32:28–34
Hershko A, Leshinsky E, Ganoth D, Heller H (1984) ATP-dependent degradation of ubiquitin-protein conjugates. Proc Natl Acad Sci USA 81:1619–1623
Iwabuchi K, Kosaka K, Haga C, Tsuchiya K, Amano N, Itoh K, Yagishita S, Mizutani Y (1991) Study of argyrophilic inclusions of multiple atrophy (Oppenheimer). Brain Nerve 43:561–568
Kato S, Nakamura H (1990) Cytoplasmic argyrophilic inclusions in neurons of pontine nuclei in patients with olivopontocerebellar atrophy: immunohistochemical and ultrastructural studies. Acta Neuropathol 79:584–594
Ksiezak-Reding H, Dickson DW, Davis P, Yen S-HC (1987) Recognition of tau epitopes by anti-neurofilament antibodies that bind to Alzheimer's neurofibrillary tangles. Proc Natl Acad Sci USA 84:3410–3414
Lee S, Park YD, Yen S-HC, Ksiezak-Reding H, Goldman JE, Dickson DW (1989) A study of infantile motor neuron disease with neurofilament and ubiquitin immunohistochemistry. Neuropediatrics 20:107–111
Mannen T, Kwak S, Inoue K (1985) Neurotransmitter alterations in a brain of “pure” progressive autonomic failure. In: Mannen T (ed) Annual report of the research committee of CNS degenerative disease. The Ministry of Health and Welfare of Japan, Tokyo, pp 112–116
Nakazato Y, Yamazaki H, Hirato J, Ishida Y, Yamaguchi H (1990) Oligodendroglial microtubular tangle in olivopontocerebellar atrophy. J Neuropathol Exp Neurol 49:521–530
Papp MI, Kahn JE, Lantos PL (1989) Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome). J Neurol Sci 94:79–100
Sobue G, Hashizume Y, Mitsuma T, Takahashi A (1987) Size-dependent myelinated fiber loss in the corticospinal tract in Shy-Drager syndrome and amyotrophic lateral sclerosis. Neurology 37:529–532
Yamada T, McGeer PL (1990) Oligodendroglial microtubular masses: an abnormality observed in some human neurodegenerative diseases. Neurosci Lett 120:163–166
Yamamoto T, Hirano A (1986) A comparative study of modified Bielschowsky, Bodian and thioflavin S stains on Alzheimer's neurofibrillary tangles. Neuropathol Appl Neurobiol 12:3–9
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mochizuki, A., Mizusawa, H., Ohkoshi, N. et al. Argentophilic intracytoplasmic inclusions in multiple system atrophy. J Neurol 239, 311–316 (1992). https://doi.org/10.1007/BF00867586
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00867586