Diagnosis of Creutzfeldt-Jacob Disease on Diffusion Weighted MR Imaging Manifesting With Typical Pulvinar or Hockey Stick Sign

Abstract

Creutzfeldt-Jacob disease (CJD) is a rapidly progressing fatal neurodegenerative disease and can manifest with a rapidly progressive cognitive decline, ataxia, behavioral changes, visual disturbances and myoclonus. In our case report, we described a 68-year-old woman presented with rapid cognitive decline, gait disturbance, and cerebellar symptoms. Her laboratory and CSF examinations were found within normal limits. EEG examination revealed generalized periodic sharp and slow wave complexes. She underwent MRI examination including DWI. On DWI, restricted diffusion was detected at bilateral fronto-parietal and temporo-occipital lobes involving cingulate gyrus and bilateral basal ganglia and thalamic regions. The typical bilateral restricted diffusion areas in the medial pulvinar nuclei of the medial called as pulvinar sign or hockey stick sign were also found. DWI plays a crucial role in the diagnosis of CJD, because restricted diffusion abnormalities could be detected within a couple of weeks after the onset of disease symptoms and even before the manifestation of periodic triphasic waves on EEG. Hence, in this case report, we would like to emphasize the invaluable role of DWI in the early diagnosis of CJD cases.

Keywords

• Creutzfeldt-Jacob disease
• Diffusion-weighted imaging
• Prionic disease
• Rapidly progressing dementia