Neurology BulletinNeurology Bulletin1027-48982304-3067Eco-Vector8750010.17816/nb87500Research ArticleClinical and MRT analysis of different variants in the Konovalov-Wilson’s diseaseZalyalovaZ. A.info@eco-vector.comBogdanovE. iinfo@eco-vector.comKazan State Medical University20062002XXXIV1-25101011202110112021Copyright © 2002, Zalyalova Z.A., Bogdanov E.i.2002<p>Seven patients with KonovalovWilsons disease are presented and also materials concerning clinical and MRT analysis of the disease different variants are given. Multiple neurologic manifestations correlate with characteristic MRT picture hypointensive in T1-images and hyperintensive in T2-images and there are foci in basal ganglia, thalamus and dentate nucleus in the form of giant panda mouth, ventricle dilatation, diffuse, cerebral and cerebella atrophy. In akineticorigid syndrome absence of changes or slight ventricle dilatation are characteristic, in dystonia bilateral hull damage; in intention tremor and dysmetria atrophic or focal cerebellar changes.</p>Bekhterev neurology archiveНеврологический архив Бехтерева[Коновалов Н.В. Гепато-церебральная дистрофия. — М., 1960.][Лекарь П.Г., Макарова В.А. Гепато-церебральная дистрофия. — Л., 1984.][Alanen A., Komu М., Penttinen М. et al. // Br. J. Radiol. — 1999. - Vol. 72(860). — P.749—756.][Brewer G.J., Yuzbasiyan-Gurkan V. // Medicine. — 1991. — Vol. 71. —P. 139—64.][Dening T.R. // Int. J. Psychiatr. Med. — 1991. — Vol. 21. — P.135—48.][Donfrid M., Jankovic G., Strahinja R. et al. // Hepatogastroenterology. — 1998. — Vol.45(23). — P.1774— 1776.][Firneisz G., Szalay F., Halasz P. et al. // Acta Neurol. Scand. — 2000. — Vol. 101(4). — P. 286—288.][Garnier H., Diederich N., Pilloy W. et al. // Rev. Neurol. — 1997. - Vol. 153(2). — P. 124—128. (Medline, abstact).][Gow P.J., Peacock S.E., Chapman R.W. // J. Gastroenterol. Hepatol. — 2001. — Vol. 16(6). — P. 699—701.][Hitoshi A., Ivata M., Yoshikawa K. // J. Neurol. Neurosurg. Psychiatry. — 1991. — Vol. 54. — P. 624—626.][Horoupian D.S., Sternlieb I., Scheinberg I.H. // Clin. Neuropathol. — 1988. — Vbl.7. — P. 62—67.][Huang C.C., Chu N.S. // Eur. Neurol. — 1998. — Vol. 39(1). — P.32—37.][Imai N., Nozaki H., Miyata K. et al. // No To Shinkei. — 1999. — Medline, Abstract.][Jones E.A., Weissenborn K. // J. Neurol. Neurosurg. and Psych. — 1997. — Vol.63. — P. 279—293.][Kendall B.E., Pollock S.S., Bass N.M., Valentine L.S. // Neuroradiology. — 1981. — Vol.22. — P. 1—5.][Kurzke J.F. Neuroepidemiology. In.: C. Braddley et al dds Neurology in Clinical Practice Vol. 1. Butterwarth-Heinemann. — 1996. — P. 673—686.][Mochizuki H., Kamakura K., Masaki T. et al. // Neuroradiology. — 1997. —Vol. 39(3). — P.171—174.][Oder W., Grimm G., Kollegger H., Ferenci P., Schneider B., Deecke L. // J. Neurol. — 1991. — Vol. 238. — P. 281—287.][Roh J.K., Lee T.G., Wie B.A. et al. // Neurology. — 1994. — Vol 44.— P.1064—1068.][Schilsy M.L. // Hepatology. — 1994. — Vol.20 — P. 529—533.][Schwarz J., Antonini A., Kraft E. et al. // Neurology. — 1994. - Vol 44. — P.1079—1082.][Sternlieb I., vanden Hamer C.J.A., Morell A.G. et al. // Gastroenterology. — 1973. — Vol. 64. — P. 99—105.][Sternlieb I., Scheinberg I.H. Wilson’s disease. In: Millward-Sadler G.H., Wright R., Arthur M.J.P. eds. Wright’s liver and biliary disease. 3rd ed. Philadelphia: WB Saunders. — 1992. — P.965—975.][Svetel M., Kozic D., Stefanova E. et al. // Mov. Disord. — 2001. — Vol. 16(4). —P. 719—723.][Svetel M., Sternic N., Pejovic S., Kostic V.S. // Mov. Disord. — 2001. — Vol. 16(3). — P. 568—569.]