| Abstract | Koreiformne hiperkineze obuhvaćaju poremećaje pokreta koji su obilježeni hiperkinezom, tj. pretjeranim nevoljnim kretnjama različitih dijelova tijela i korejom, tj. brzim, naglim, nevoljnim i nesuvislim pokretima, sličnim plesu (od grčke riječi za pleschoreia), koji postupno zahvaćaju cijelo tijelo. Takvi pokreti nastaju zbog poremećaja bazalnih ganglija, a uzrok mogu biti razni poremećaji koji se mogu podijeliti na nasljedne i stečene. Među nasljednim poremećajima koji uzrokuju koreju su Huntingtonova bolest, neuroakantocitoza, benigna hereditarna koreja i Wilsonova bolest. Od stečenih bolesti koje se mogu klinički manifestirati koreiformnom hiperkinezom su cerebrovaskularne bolesti, metabolički poremećaji i autoimune bolesti. Nasljedni poremećaji se obično klinički manifestiraju u ranijoj dobi nego stečeni poremećaji. Nadalje, nasljedni poremećaji su kroničnog tijeka, a koreja uzrokovana stečenim poremećajima ima najčešće akutan tijek. Također, treba reći da je koreja uzrokovana stečenim poremećajima često reverzibilna nakon liječenja osnovne bolesti. S druge strane, koreja uzrokovana nasljednim poremećajima, kao heredodegenerativna bolest uglavnom se liječi simptomatski, dopaminskim antagonistima i drugim lijekovima. Uz nasljedne poremećaje osim simptoma koreje, često se pojavljuju psihijatrijski poremećaji kao i poremećaj kognitivnih funkcija. Ako koreja zahvati cijelo tijelo, onda su pridruženi poremećaj hoda i govora. U dijagnostici koreje bitno je uzeti temeljitu anamnezu, obiteljsku anamnezu i napraviti detaljan fizikalni i neurološki status. Nadalje, treba napraviti dodatnu dijagnostičku obradu – genetičko testiranje pri sumnji na nasljedne bolesti, MR i ciljane laboratorijske pretrage. |
| Abstract (english) | Choreiform hyperkinesias include movement disorders characterized by hyperkinesia, i.e. excessive involuntary movements of different parts of the body, and chorea, i.e. fast, sudden and involuntary movements that are similar to dancing (from the Greek word for dance – choreia) which gradually affect the whole body. Such movements are caused by disorders of the basal ganglia and the cause can be various disorders that can be divided into hereditary and acquired disorders. Hereditary disorders that cause chorea include Huntington's disease, neuroacanthocytosis, benign hereditary chorea and Wilson's disease. Among the acquired diseases that clinically present with choreiform hyperkinesias are cerebrovascular diseases, metabolic disorders and autoimmune diseases. Hereditary disorders usually present with symptoms at an earlier age than acquired disorders. Furthermore, hereditary disorders have a chronic course, while chorea caused by acquired disorders has an acute course. Also, it should be said that chorea caused by acquired disorders is often reversible after the treatment of the underlying disease. On the other hand, chorea caused by hereditary disorders is mainly treated symptomatically with dopamine antagonists and other drugs. In addition to the symptoms of chorea, psychiatric disorders as well as cognitive function disorders often appear with the latter disorders. If chorea affects the whole body, then it is associated with gait disturbances and speech disorders. In the diagnosis of chorea, it is important to take a thorough history and make a detailed physical and neurological exam. Furthermore, it is necessary to do additional diagnostic investigations - genetic testing in case of suspected hereditary diseases, MRI and targeted laboratory tests. |
