Skip to main content
SpringerLink
Log in

Familiäre Striatumdegeneration

Familial striatal degeneration

  • Published:
Archiv für Psychiatrie und Nervenkrankheiten Aims and scope Submit manuscript

Summary

An extrapyramidal disorder occurring in three generations of a family (only males) is described. The clinical features were progressive dementia and extrapyramidal signs without choreiform hyperkinesia. The youngest patient (onset of disease at the age of 22 years) showed tremor, rigidity, ataxia, convulsions, and myoclonus. The neuropathologic findings were characterized by isolated symmetrical degeneration of the corpus striatum and diffuse cortical atrophy without affecting other cerebrospinal neuronal systems. The clinical features of this familial disorder and its relation to other types of familial striatal degeneration and to the juvenile form of Huntington's chorea are discussed.

Zusammenfassung

Bericht über ein extrapyramidales Krankheitsbild mit Befall von 3 Generationen einer Familie (Großvater, Vater, Sohn), klinisch gekennzeichnet durch organische Demenz ohne choreiforme Hyperkinesen mit beim jüngsten Probanden (Krankheitsbeginn 22. Lebensjahr) vorherrschendem Tremor, Rigor, Ataxie sowie Krampfanfällen mit Myoklonien. Neuropathologisch fand sich eine symmetrische Striatumdegeneration und diffuse Großhirnrindenatrophie ohne sonstige zentralnervöse Systemdegenerationen. Die klinischen Besonderheiten dieses Krankheitsbildes und seine Beziehungen zu anderen Formen familiärer Striatumdegeneration sowie zur juvenilen Form der Chorea Huntington werden erörtert.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Literatur

  • Barrows, H. S., Cooper, W. C.: Rigidity as a disease form of Huntington's chorea. Bull. Los Angeles Neurol. Soc. 28, 144–147 (1963)

    Google Scholar 

  • Bruyn, G. W.: Huntington's chorea. Historical, clinical and laboratory synapsis. In: Handbook of clinical neurology, P. J. Vinken, G. W. Bruyn (eds.), Vol. 6, pp. 298–378. Amsterdam: North Holland Publ. Comp. 1968

    Google Scholar 

  • Byers, R. K., Gilles, F. H., Fung, C.: Huntington's chorea in children. Neuropathologic study of four cases. Neurology (Minneap.) 23, 561–569 (1973)

    Google Scholar 

  • Carlier, G., Reznik, M., Franck, G., Husquinet, H.: Etude anatomoclinique d'une forme infantile de la maladie de Huntington. Acta Neurol. Belg. 74, 36–63 (1974)

    Google Scholar 

  • Erdohazi, M., Marshall, P.: Striatal degeneration in childhood. Arch. Dis. Childh. 54, 85–91 (1979)

    Google Scholar 

  • Fau, R., Chateau, R., Tommasi, M., Groslambert, R., Garrel, S., Perret, J.: Etude anatomoclinique d'une forme rigide et myoclonique de maladie de Huntington familiale. Rev. Neurol. 124, 353–366 (1971)

    Google Scholar 

  • Gilbert, J.: Dyssynergia cerebellaris myoclonica. In: Handbook of clinical neurology, P. J. Vinken, G. W. Bruyn (eds.), Vol. 21, pp. 509–518. Amsterdam: North Holland Publ. Comp. 1975

    Google Scholar 

  • Goebel, H. H., Heipertz, R., Scholz, W., Iqbal, K., Tellez-Nagel, I.: Juvenile Huntington chorea: clinical, ultrastructural and biochemical studies. Neurology (Minneap.) 28, 23–31 (1978)

    Google Scholar 

  • Hawke, W. A., Donohue, W. L.: Bilateral symmetrical necrosis of the corpora striata. Report of a fatal case and referende to a possible syndrome of the corpora striata. J. Nerv. Ment. Dis. 113, 20–39 (1950)

    Google Scholar 

  • Jellinger, K., Kothbauer, P.: Das Chorea Syndrom. In: Neurologie in Klinik und Praxis, H. Hopf, H. Schliack, K. Poeck (eds.), Bd. 1. Stuttgart: G. Thieme 1979 (in Druck)

    Google Scholar 

  • Jervis, G. A.: Huntington's chorea in children. Arch. Neurol. (Chic.) 9, 244–257 (1963)

    Google Scholar 

  • Marinesco, G., Draganesco, S.: Contribution anatomoclinique á l'étude du syndrome de Foerster. Encéphale 24, 685–699 (1929)

    Google Scholar 

  • Miyoshi, K., Matusoka, T., Mizushima, S.: Familial holotopistic striatal necrosis. Acta Neuropath. (Berl.) 13, 240–249 (1969)

    Google Scholar 

  • Peretti, J.: Über hereditäre choreatische Bewegungsstörungen. Berl. Klin. Wschr. 22, 824–825, (1885)

    Google Scholar 

  • Petit, H.: La maladie de Huntington. C. R. Congrés de Psychiatrie et de Neurologie de Langue Française, LXVIIe Session: Bruxelles 1969, 2, 901–1078. Paris: Masson & Cie 1969

  • Roessmann, U., Schwartz, J. F.: Familial striatal degeneration. Arch. Neurol. (Chic.) 29, 314–317 (1973)

    Google Scholar 

  • Takahata, N., Ito, K., Yoshimura, Y., Nishihori, K., Suzuki, H.: Familial chorea and myoclonus epilepsy. Neurology (Minneap.) 28, 913–919 (1978)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Ludwig-Boltzmann-Institut für klin. Neurobiologie, Krankenhaus Wien-Lainz, Wolkersbergenstrasse 1, A-1130, Wien, Österreich

    K. Jellinger, W. Danielczyk, P. Kothbauer & D. Seemann

Authors
  1. K. Jellinger
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. W. Danielczyk
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. P. Kothbauer
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. D. Seemann
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Jellinger, K., Danielczyk, W., Kothbauer, P. et al. Familiäre Striatumdegeneration. Arch. Psychiat. Nervenkr. 227, 261–269 (1979). https://doi.org/10.1007/BF00367395

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00367395

Key words

Schlüsselwörter

Search

Navigation