Should be cited as: Psychiatr. Pol. 2014; 48(1): 187–194 ISSN 0033-2674 www.psychiatriapolska.pl Creutzfeldt-Jakob disease in the psychiatric practice – case reports of the ataxic and Heidenhain variant Dominika Berent, Iwona Bączek, Antoni Florkowski, Piotr Gałecki Clinic of Adult Psychiatry, Medical University of Łódź Summary Objectives. Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare fatal neurodegenerative brain disorder of variable clinical manifestation. Making right diagnosis still remains challen- ging. First symptoms are vague and differ between clinical subtypes of the disease. This is to present the symptoms variability and diagnostic dificulties in sCJD based on case reports of two female patients examined at time of the disease duration by psychiatrists. Methods. Data of our patients were collected from hospital medical records. Results. The case of patient A. P.’ ataxic sCJD is an example of clinical picture suggesting neurological background of the disease almost from the symptoms’ onset and being referred by psychiatrist to the neurological ward, where the right diagnosis of probable sCJD was estab- lished. In the opposite is the case of patient I. W.’ Heidenhain variant of sCJD, misdiagnosed with dissociative disorder and delivering huge diagnostic dificulties, even to neurologists. In both patients the certain diagnosis was conirmed at autopsy. Conclusions. In patients with visual disturbances of unknown etiology, even if the ophthal- mological and neurological background is excluded, sCJD should be taken into consideration in the differential diagnosis. Key words: Creutzfeldt-Jakob disease, psychiatry Background Creutzfeldt-Jakob disease is a fatal neurodegenerative brain disorder. Making right diagnosis remains challenging with regard to inconstant presentation and low prevalence being assessed at 1 – 1,6 : 1 000 000. The most frequent type of CJD, spo- radic Creutzfeldt – Jakob (sCJD), accounts for 85-95% of all cases [1]. Moreover, the sCJD patients are a heterogeneous group divided into a few clinical subtypes which vary according to clinical manifestation, disease course and results of a diagnostic workup. World Health Organization (WHO) diagnostic criteria for sCJD are listed in Table 1[2]. A core diagnostic criterium is quickly progressing dementia but there can be seen variable psychiatric symptoms interfering clinical picture of the disease and causing misdiagnoses [3]. We describe cases of two different clinical subtypes of sCJD to emphasize incon- stant clinical manifestation of the disease and diagnostic dificulties it cause. First case,