Summary
A case of essential degeneration of the thalamus is reported. The patient was a 43-year-old Japanese male, who, a few weeks after mild head trauma, suffered from forgetfulness, psychomotor slowing, and Korsakoff's syndrome. Four to five months later, there were optical hallucinations and delirium and he died 9 months after the onset of symptoms.
Neuropathological examination revealed symmetrical thalamic degeneration, whose distribution corresponded to phylogenetically younger subunits of the thalamus. In addition, there was olivovermian degeneration.
These findings are identical to those of eleven cases hitherto reported. Five of these were Japanese, including the present one.
The syndrome thalamic degeneration may now be classified as a special type of “system degeneration”.
Zusammenfassung
Es wird über einen Fall von essentieller Systemdegeneration des Thalamus berichtet. Ein 43jähriger Japaner zeigte nach leichtem Kopfstoß eine langsam progrediente Vergeßlichkeit; nach 2 Monaten eindeutiges Korsakoff-Syndrom, 4–5 Monate nach dem Initialsymptom optische Halluzinationen, nächtliche Delirien und starke Abmagerung. Der Tod trat 9 Monate nach dem Kopftrauma ein.
Neuropathologisch ergab sich eine symmetrische Thalamusdegeneration, deren Intensität und topographische Verteilung mit der phylogenetisch jüngeren Kerngruppe des Thalamus übereinstimmen. Als Nebenbefund fand sich eine olivovermale Degeneration.
Der neuropathologische Hauptbefund ist weitgehend identisch mit den bisher berichteten 11 Fällen; 5 davon betreffen Japaner, einschließlich unseres Falles.
Das Syndrom „Thalamusdegeneration“ kann als besondere Form einer „Systematrophie“ klassifiziert werden.
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References
Behrman S, Mandybur T, McMenemey WH (1962) Un cas de maladie de Creutzfeldt-Jakob à la suite d'un traumatisme cérébrale. Rev Neurol 107:453–459
Conrad K, Ule G (1951) Ein Fall von Korsakow-Psychose mit anatomischem Befunde und klinischen Betrachtungen. Dtsch Z Nervenheilk 165:430–445
Daniels AC, Chokrverty S, Barron KD (1969) Thalamic degeneration, dementia, and seizures. Inappropriate ADH secretion associated with bronchogenic carcinoma. Arch Neurol (Chic) 21:15–24
Dewulf A (1971) Anatomy of the normal human thalamus. Topometry and standarized nomenclature. Elsevier, Amsterdam London New York
Finlayson MH, Snider S, Oliva LA, Gault MH (1973) Cerebral and pontine myelinolysis. Two cases with fluid and electrolyte inbalance and hypotension. J neurol Sci 18:399–409
Gamper E (1928) Zur Frage der polioencephalitis haemorrhagica der chronischen Alkoholiker. Anatomische Befunde bei alkoholischen Korsakow und ihre Beziehungen zum klinischen Bild. Dtsch Z Nervenheilk 102:122–129
Garcin R, Brion S, Khochneviss AA (1963) Le syndrome de Creutzfeldt-Jakob et les syndromes cortico-striées du présenium (à l'occasion de 5 observations anatomocliniques. Rev Neurol 109:419–441
Glees P, Griffith HB (1952) Bilateral destruction of the hippocampus (Cornu ammonis) in a case of dementia. Monatsschr Psychiatr 123:193–204
Grahmann H, Ule G (1957) Beitrag zur Kenntnis der chronischen cerebralen Krankheitsbilder bei Boxern (Dementia pugilistica und traumatische Boxer-Encephalopathie). Psychiatr Neurol (Basel) 134:261–283
Grünthal E (1942) Über thalamische Demenz. Monatsschr Psychiatr 106:114–128
Khochneviss AA (1962) Contribution à l'etude du syndrome de Creutzfeldt-Jakob et des syndromes cortico-striées du présenium (à l'occasion de cinq observations anatomocliniques). Thése Méd, Paris (cited from Garcin et al.)
Martin JJ (1970) Contribution à l'étude de l'anatomie du thalamus et de sa pathologie au cours des maladies dégénératives dites abiotrophiques. Acta Neurol Belg 70:1–211
Martin JJ (1970) Sémiologie et neuropathologie thalamique humaines. Acta Neurol Belg 70:771–794
Martin JJ (1975) Thalamic degenerations. In: Vinken PJ, Bruyn GW (eds) Handb. Clin. Neurol. 21, System disorders and atrophies, part I. North-Holland Publ. Co., Amsterdam, pp 587–604
McMenemey WH, Grant HC, Behrman S (1965) Two examples of “presenile dementia” (Pick's disease and Stern-Gracin syndrome) with a history of trauma. Arch Psychiatr Z Neurol 207:128–140
Oda M, Yoshida T, Shiraki H, Yokoyama T (1965) An autopsy case of the systemic degeneration of the bilateral thalamic nuclei associated with olivocerebellar atrophy. Psychiatr Neurol Jpn (Seishin-Shinkeigaku Zasshi) 67:67–82
Oda M, Yamamoto T, Abe H, Shikiba S (1972) An autopsy case of thalamic, nigro-pallidal and olivo-ponto-cerebellar degeneration. A contribution to understanding of the system degeneration of the CNS. Adv Neurol Sci (Shinkei Kenkyu no Shinpo) (Tokyo) 16:514–520
Oda M, Yoshimura T, Okumura A (1973) Degeneration of the central nervous system enhanced in the thalamus. Adv Neurol Sci (Shinkei Kenkyu no Shinpo) (Tokyo) 17:238–255
Ogasawara S (1973) Two cases of Creutzfeldt-Jakob disease. Adv Neurol Sci (Shinkei Kenkyu no Shinpo) (Tokyo) 17:402 (Abstr.)
Orthner H (1957) Pathologische Anatomie der vom Hypothalamus ausgelösten Bewußtseinsstörungen. 1st International Congress of Neurological Science. Joint Meetings, Brüssel 1957. Volume de la Deuxieme Journée Commune, 77–96
Papez JW (1937) A proposed mechanism of emotion. Arch Neurol Psychiatr (Chic) 38:725–743
Pilz P, Erhart P (1981) Thalamic degeneration. Acta Neuropathol (Berl), Suppl VII:362–364
Schulman S (1957) Bilateral symmetrical degeneration of the thalamus. A clinico-pathological study. J Neuropathol Exp Neurol 16:446–470
Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogenous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10:333–359
Stern K (1939) Severe dementia associated with bilateral symmetrical degeneration of the thalamus. Brain 62:157–171
Tsuchiya M, Hada H, Akimoto Y, Iwasaki Y, Sato T (1970) Chronic carbon disulfide poisoning or systemic thalamic degeneration? Adv Neurol Sci (Shinkei Kenkyu no Shinpo) (Tokyo) 14:416–417 (Abstr.) (cited by Oda et al., 1972, as the second case)
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Dedicated to Dr. Shikoh Takeya on his 77th birthday, 13th October 1981
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Hori, A., Ikeda, K., Kosaka, K. et al. System degeneration of the thalamus. Arch Psychiatr Nervenkr 231, 71–80 (1981). https://doi.org/10.1007/BF00342831
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DOI: https://doi.org/10.1007/BF00342831