Summary
An autopsy case of multiple system degeneration is characterized by the following; (1) progressive dementia and abnormal sleep patterns, followed by Parkinsonian symptoms with terminal akinetic mutism: (2) severe symmetrical degeneration in the thalamus, particularly nucl. medialis thalami, the reticular formation of the brain stem, also the pallidonigral, pallido-Luysian and dentato-rubral systems. As far as we know, there is no case in the literature, of combined system degeneration, which shows such a wide anatomical range of lesions as the present one. Clinico-pathological correlation between dementia and degeneration of the thalamus, and between abnormal sleep-consciousness mechanism and degeneration of the reticular formation are discussed. The extrapyramidal symptoms are discussed from the clinico-pathological aspect. In addition, peculiar eosinophilic bodies are described, which were most frequently found in the putamen.
Similar content being viewed by others
References
Bénard, L. R., Grossiord, A., Gruner, J., Hoppeler, A.: Sur une forme particulière de maladie familiale du système nerveux, apparentée aux maladies de Friedreich et de Charcot-Marie. Deux observations anatomo-cliniques. Rev. neurol86, 800–818 (1952)
Bricolo, A.: Neurosurgical exploration and neurological pathology as a means for investigating human sleep semiology and mechanisms. In: The experimental study of human sleep; Methodological problems, (Ed. G. C. Lairy, and P. Salzarulo), pp. 51–79. Amsterdam-Oxford-New York: Elesvier 1975
Dewulf, A.: Anatomy of the normal human thalamus. Topometry and standardized nomenclature. Amsterdam-London-New York: Elsevier 1971
Garcin, R., Brion, S., Khochneviss, A. A.: Le syndrome de Creutzfeldt-Jakob et les syndromes cortico-striés du présénium (á l'occasion de 5 observations anatomocliniques). Rev. neurol.109, 419–441 (1963)
Grünthal, E.: Über thalamische Demenz. Mschr. Psychiat. Neurol.106, 114–128 (1942)
Hirano, A., Dembitzer, H. M., Kurland, L. T., Zimmerman, H. M.: The fine structure of some intraganglionic alterations. J. Neuropath. exp. Neurol.27, 167–182 (1968)
Hori, A., Matsushita, M., Ishii, T., Mori, T., Iizuka, R., Murofushi, K., Shinohara, S.: A case of symmetrical thalamus degeneration. 16th Congress of Jap. Neuropath. Soc., Yonago 1975
Jellinger, K.: Degenerations and exogeneous lesions of the pallidum and striatum. In: Handbook of clinical neurology. Vol. 6, (Ed. P. J. Vinken and G. W. Bruyn), pp. 632–693. Amsterdam: North Holland 1968
Kaiya, H., Mehraein, P., Yoshimura, T.: Pallidonigrale und thalamische Degeneration. Beitrag zur thalamischen Demenz. Arch. Psychiat. Nervenkr.219, 323–330 (1974)
Kobayashi, H., Kosaka, K., Hoshino, T., Shibayama, H., Iwase, S.: An autopsy case of the characteristic degeneration of the dentate nucleus with choreic movement and psychiatric symptoms. Clin. Neurol.15, 724–730 (1975)
Martin, J. J.: Thalamic degenerations. In: Handbook of clinical neurology. Vol. 21, (Ed. P. J. Vinken and G. W. Bruyn), pp. 587–604. Amsterdam: North Holland 1975
Martin, J. P.: Hemichorea of sudden onset (Hemiballismus). In: The basal ganglia and posture, (Ed. J. P. Martin), pp. 79–91. London: Pitman Medical 1967
Matsushita, M., Kosaka, K., Hori, A., Oyanagi, S., Fukajima, O.: An autopsy case of hereditary degeneration of the dentate nucleus and the external segment of the globus pallidus. 17th Congress of Jap. Neuropath. Soc., Niigata 1976
Neumann, M. A.: Combined degeneration of globus pallidus and dentase nucleus and their projections. Neurology (Minneap.)9, 430–438 (1959)
Oda, M., Yoshimura, T., Shiraki, H., Yokoyama, T.: An autopsy case of the systemic degeneration of the bilateral thalamic nuclei associated with olivo-cerebellar atrophy. Psychiatr. Neurol. jap.67, 67–82 (1965)
Oda, M., Yamamoto, T., Abe, H., Shikiba, S.: An autopsy case of thalamic, nigropallidal and olivo-ponto-cerebellar degeneration. A contribution to understading to the system degeneration of the CNS. Advanc. Neurol. Sci.16, 514–520 (1972)
Oda, M., Yoshimura, T., Okumura, A.: Degeneration of the central nervous system enhanced in the thalamus. Advanc. Neurol. Sci.17, 238–255 (1973)
Ogata, J., Budzilovich, G. N., Cravioto, H.: A study of rod-like structures (Hirano bodies) in 240 normal and pathological brains. Acta neuropath. (Berl.)21, 61–67 (1972)
Oyanagi, S., Tanaka, M., Naito, H., Shirakawa, K., Saito, K., Nakamura, N., Ohama, E.: A neuropathological study of 8 autopsy cases of degenerative type of myoclonus epilepsy-With special reference to latent combination of degeneration of the pallido-luysian system. Advanc. Neurol. Sci.20, 410–424 (1976)
Schochet, S. S., Jr., Lampert, P. W., Earle, K. M.: Alexander's disease. A case report with electron microscopic observations. Neurology (Minneap.)18, 543–549 (1968)
Schulman, S.: Bilateral symmetrical degeneration of the thalamus. A clinicopathological study. J. Neuropath. exp. Neurol.16, 446–470 (1957)
Shiraki, H.: The neuropathology of the cerebellar disease group—With special reference to the clinical features. Advanc. Neurol. Sci.17, 941–985 (1973)
Smith, J. K., Gonda, V. E., Malamud, N.: Unusual form of cerebellar ataxia. Combined dentatorubral and pallido-Luysian degeneration. Neurology (Minneap.)8, 205–214 (1958)
Stern, K.: Severe dementia associated with bilateral symmetrical degeneration of the thalamus. Brain62, 157–171 (1939)
Takahashi, K., Sakamoto, K., Takezaki, H., Hanawa, S., Machii, Y., Hori, A., Matsushita, M., Kosaka, K.: Two cases of mother and her daughter of degenerative disease with the severest changes in the dentate nucleus. 17th Congress of Jap. Neuropath. Soc., Niigata 1976
Tanaka, Y., Murofushi, K., Ando, S., Hayashi, T., Suwa, K.: Two cases of siblings with degeneration of the dentato-rubral and pallido-luysian systems. 17th Congress of Jap. Neuropath. Soc., Niigata 1976
Titica, J., Van Bogaert, L.: Heredo-degenerative hemiballismus. A contribution to the question of primary atrophy of the corpus Luysii. Brain69, 251–263 (1946)
Tsuchiya, M., Hada, H., Akimoto, Y., Iwasaki, Y., Sato, T.: Chronic carbon disulfide poisoning? or systemic thalamic degeneration? 10th Congress of Jap. Neuropath. Soc., Fukuoka 1969.
Van Bogaert, L.: Aspects cliniques et pathologiques des atrophies pallidalles et pallido-luysiennes progressives. J. Neurol. Neurosurg. Psychiat.9, 125–157 (1946)
Verhaart, W. J. C.: An unclassified degenerative disease of the central nervous system. Arch. Neurol. Psychiat. (Chic.)44, 1262–1270 (1940)
Verhaart, W. J. C.: Degeneration of the brain stem reticular formation, other parts of the brain stem and the cerebellum. An example of heterogeneous systemic degeneration of the central nervous system. J. Neuropath. exp. Neurol.17, 382–391 (1958)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kosaka, K., Oyanagi, S., Matsushita, M. et al. Multiple system degeneration involving thalamus, reticular formation, pallido-nigral, pallido-Luysian and dentato-rubral systems. Acta Neuropathol 39, 89–95 (1977). https://doi.org/10.1007/BF00690390
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00690390