MEG Abnormalities in Creutzfeldt-Jakob Disease, a Case Report

Creutzfeldt-Jakob Disease (CJD) is a rare neurodegenerative disease, which belongs to a group of spongiform encephalopathies termed prion diseases. Main symptoms of CJD are progressive dementia, pyramidal and extrapyramidal symptoms, and myoclonus. Currently there is no effective therapy for CJD. Definite diagnosis is based on autopsy findings. Here we report a male patient with a rapidly progressive memory disorder, ataxia and myoclonus. EEG showed slow wave activity, and head MRI revealed increased basal ganglia and left-dominant increase in cortical signal intensity. In cerebrospinal fluid (CSF) 14-3-3 test was positive. The autopsy confirmed the sporadic CJD. A 306-channel MEG measurement was performed to the patient. Spontaneous brain activity and auditory evoked fields (AEF) were recorded. Both hemispheres had slow wave activity and periodic sharp waves, activity being more pronounced in the left, more damaged, hemisphere. The source of auditory N100m were more lateral in the left than in the right hemisphere. P200m was larger and more posterior in the left hemisphere compared with the right hemisphere. This is the first MEG-recording of a patient with sporadic CJD. The more damaged left hemisphere in MRI had more pronounced slow and sharp waves in MEG. The sources of auditory responses and amplitude of later P200m deflection were different between the more and less damaged hemispheres. MEG appears to be suitable to investigate and locate brain activity in prion diseases.