Summary
In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contain RNA-Protein compounds. Electron microscopically, they consist of randomly interwoven tubules with granular endoplasmic reticulums and free ribosomes in the margin. Each tubule measures 90–150 Å in diameter and shows no distinct periodic constiction. Amorphous substances as well as ribosome granules are scattered and associated with those tubules. The inclusion-bearing cells are usually swollen and chromatolytic and have a large hydropic nucleus, suggesting a close relation between the development of the inclusion and chromatolysis. Clinically, a rapid progress of the symptoms (total duration: about 12 months) and conspicuous disturbances of the autonomic nerve, such as sinus tachycardia and bladder sphincter dysfunction, should be noticed.
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Berry, R. G., Chambers, R. A., Duckett, S.: Clinicopathological study of juvenile ALS. Neurology (Minneap.)19, 312 (1969)
Bogaert, L. van: La sclérose latérale amyotrophique et la paralysie bulbaire progressive chez l'enfant. Rev. Neurol.1, 180–192 (1952)
Cognazzo, A., Martin, L.: A sporadic case of juvenile ALS. Semiquantitative and histoenzymatical study of the denervated muscles. Eur. Neurol.3, 211–230 (1970)
Hirano, A., Kurland, L. T., Sayre, G. P.: Familial ALS: a subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells. Arch. Neurol.16, 232–243 (1967)
Kuroda, S., Hayahara, T., Tateishi, J.: An autopsy case of spastic tetraparesis, showing Alzheimer's neurofibrillary changes with peculiar localization. 17. Congress of Jap. Neuropathol. Society, Niigata 1976
Mannen, T., Iwata, M., Toyokura, Y., Nagashima, K.: Pathology of anterior horn of the sacral cord in cases of ALS and its clinical significance. Neurol. Med. (Shinkeinaika)3, 169–175 (1975)
Mendell, J. R., Markesbery, W. R.: Neuronal intracytoplasmic hyaline inclusions. J. Neuropathol. Exp. Neurol.30, 233–239 (1971)
Nelson, J. S., Prensky, A. L.: Sporadic juvenile ALS. A clinicopathological study of a case with neuronal cytoplasmic inclusions containing RNA. Arch. Neurol.27, 300–306 (1972)
Nishigaki, S., Takahashi, A., Matsuoka, Y.: Ein Obduktionsfall der sporadischen juvenilen ALS. Clin. Neurol. (Tokyo)11, 500–506 (1971)
Norman, M. G.: Hyaline (colloid) cytoplasmic inclusions in motoneurones in association with familial micrencephaly, retardation and seizures. J. Neurol. Sci.23, 63–70 (1974)
Orthner, H., Becker, P. E., Müller, D.: Recessiv erbliche ALS mit “Lafora-körperchen”. Arch. Psychiatr. Nervenkr.217, 387–412 (1973)
Roessmann, U., McFarland, D. E.: Hyaline cytoplasmic inclusions in motor neurons. J. Neuropathol. Exp. Neurol30, 551–556 (1971)
Schochet, S. S., Hardman, J. M., Ladewig, P. P., Earle, K. M.: Intraneuronal conglomerates in sporadic motor neuron disease. A light and electron microscopic study. Arch. Neurol.20, 548–553 (1969)
Sun, C. N., Araoz, C., Lucas, G., Morgan, P. N., White, H. J.: ALS. Inclusion bodies in a case of the classic sporadic form. Ann. Clin. Lab. Sci5, 38–44 (1975)
Takahashi, K., Nakamura, H., Okada, E.: Hereditary ALS. Arch. Neurol.27, 292–299 (1975)
Tellez-Nagel, I., Wiśniewski, H. W.: Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch. Neurol.29, 324–327 (1973)
Tomonaga, M., Saito, M., Yoshimura, M., Shimada, H., Mochizuki, H., Yamanouchi, H., Togi, H.: Neuronal inclusions (Bunina body) in a case of ALS. 18. Congress of Jap. Neuropathol. Soc., Matsumoto 1977
Tsujihata, M., Taguchi, H., Oku, Y., Takamori, M., Terao, H.: A sporadic case of juvenile ALS. Clin. Neurol. (Tokyo)18, 82–87 (1978)
Wohlfart, G., Swank, R. L.: Pathology of ALS. Fiber analysis of the ventral roots and pyramidal tracts of the spinal cord. Arch. Neurol. Psychiat. (Chicago)46, 783–799 (1941)
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Oda, M., Akagawa, N., Tabuchi, Y. et al. A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions. Acta Neuropathol 44, 211–216 (1978). https://doi.org/10.1007/BF00691069
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DOI: https://doi.org/10.1007/BF00691069