A 76-year-old woman developed difficulties in dressing. She had progressive problems dressing herself. In particular, she had difficulty handling buttons and arranging her clothing articles on her body. Dressing became more and more prolonged, and she began to manipulate her clothes incoherently until finally needing support from her husband. Her medical history included stable plasmocytoma, atrial fibrillation and residual effects from a minor stroke, and she was orally anticoagulated with phenprocoumon. Her neurological examination on admission revealed a slight right-sided motor impairment with finger tapping and slight dysarthria, which resulted from the previous minor stroke. Neuropsychologically pronounced signs and symptoms of dressing apraxia were found whereby performing simple gestures and pantomiming object use were preserved. Otherwise, there were no signs of constructional and limb ataxia, optic ataxia and visual disturbances. The MoCA test showed 28 of 30 points with slight impairment in the visuospatial items. Her family history was unremarkable. She had not had any previous neurosurgical procedures or corneal transplants []. Her first cranial magnetic resonance images (MRI) showed marked cortical restriction of diffusion bilaterally (positive “ribbon sign”), most prominent in the parietal regions. A cerebrospinal fluid (CSF) analysis revealed a normal cell count (2/µl; normal <4) and slightly elevated protein (523 mg/l; normal <450). Tests for Borrelia burgdorferi, varicella zoster virus and herpes simplex virus were negative. Her beta-amyloid 1–42 was slightly diminished (544.8 pg/ml; normal >630), and the tau-protein (>1397 pg/ml; normal <290) and phospho-tau-protein (98.5 pg/ml; normal <61) were elevated. Her protein 14-3-3 tested positive, and the first test on PrSc was negative. An EEG at this time showed paroxysmal dysrhythmia with short generalized groups of higher voltage sharp and slow waves. The beginning of CJD was suspected and supportive home care arranged. Seven weeks later, the dressing apraxia had progressed to the point that she needed complete support in dressing. In addition, her dysarthria had also progressed; otherwise, the patient was still ambulatory. A second MRI showed progressive cortical restriction of diffusion. In a second CSF analysis, the pattern of the proteins beta-amyloid 1–42, tau- and phospho-tau-protein were nearly unchanged abnormal. In addition to the positive protein 14-3-3, now PrSc test was positive. After a consultation with the Prion Research Group of University Hospital Göttingen, Germany, probable CJD was diagnosed. As her clinical condition rapidly deteriorated with now more generalized apraxia, inability to walk and appearance of spontaneous myocloni, palliative hospital care was initiated. The patient deceased one week later. A post-mortem examination was not performed.