case_images: PMC4899438_01
case_images: (A) Resting brain metabolism as measured by [18F]-FDG-PET superimposed on the patient's CT images in representative horizontal, coronal, and sagittal planes. Preservation of the anterior forebrain and bilateral thalami (yellow and red colors) and striatum (not visible on this image) with loss of posterior brain areas is evident. (B) Bar graphs show standard uptake values in selected cortical regions of interest scaled to the global mean (mean normalized uptake values; mn-UV) in 10 healthy volunteers (HV; blue columns, +-SD) and in the patient (red columns) in the right and left hemispheres. Mn-UV in the patient are normal in the anterior forebrain, including the prefrontal cortices and the left premotor area, however significantly decreased over the posterior cortical areas, including primary sensorimotor areas, bilateral parietal, and occipital cortices (primary visual area). R, right; L, left; A, anterior; P, posterior; OFC, orbitofrontal; vmPFC, dlPFC, ventromedial and dorsolateral prefrontal cortices; PMC, premotor cortex; SM1, primary sensorimotor cortex; aCGc, mCGc, pCGc, anterior, medial and posterior cingulate cortices; sPPC, iPPC, superior and inferior posterior parietal cortices; precun, precuneus; V1, primary visual cortex.
case_images: fnins-10-00259-g0001.jpg
case_images: PMC4899438_01_fnins-10-00259-g0001.jpg
case_images: F1
case_images: Bilateral primary sensorimotor cortices along with widespread areas in parietal and occipital lobes showed marked reduction of metabolic signal (Figure 1).
case_images: Absolute EEG power frequency spectra in wakefulness (red lines), stage 2 sleep (blue lines), and slow-wave sleep (green lines) over select fronto-central and occipital channels. During wakefulness the EEG demonstrates normal fronto-central beta (16-28 Hz; at Cz, FC1, FC2) activity, abnormally present central theta (4-7 Hz; at Cz) activity, and abnormal lack of posterior alpha (9-12 Hz; at Oz, O1, O2) activity, suggesting normal fronto-central and abnormal posterior brain functions. During stage 2 and slow wave sleep, sleep spindles (13-15 Hz; FC1, FC2) are seen maximally over fronto-central channels, and during slow-wave sleep increased delta (< 4 Hz, all electrodes) is seen.
case_images: fnins-10-00259-g0002.jpg
case_images: PMC4899438_01_fnins-10-00259-g0002.jpg
case_images: F2
case_images: Visual review and spectral analysis of the wakeful EEG showed characteristic frontal-central beta-range activity during wakefulness, and canonical features of stage 2 and slow wave sleep stages during sleep (Figure 2). However, the EEG also demonstrated an abnormal central theta feature and there was no posterior alpha rhythm during wakefulness (Figure 2).
case_images: (A) Determination of command following using EEG: Spectra are based on 9 s-long EEG segments from each "move" (red lines) and "stop" (blue lines) trials after combining two runs (total of 16 commands in each condition). Significant differences in power spectral frequency content between conditions in each channel were determined using a z-test (p < 0.05; marked with stars). Significant differences are evident over left centro-parietal regions. (B) Determination of time course of signal differences: Topoplots show power differences between "move" and "stop" conditions at 5 +- 1 Hz frequency range in all channels in four 3-s long EEG segments. Red colors represent more power during trials of "move" condition, compared to "stop," blue colors the opposite. Circled areas shows significant difference between the conditions at p < 0.05. During the first 3-s time segment ("command period"), some increased frontal power during the "move" condition is seen, however it does not reach significance. Significant differences between the conditions (with more power during "stop" conditions) are prominent throughout the "response period" in the three 3-s long time segments, however with slightly varying intensity and localization.
case_images: fnins-10-00259-g0003.jpg
case_images: PMC4899438_01_fnins-10-00259-g0003.jpg
case_images: F3
case_images: Statistically significant differences in EEG spectral power between "move" and "stop" conditions localized over the left centro-parietal areas (Figure 3A), a similar pattern expected in healthy control subjects (contralateral electrodes over sensorimotor regions). Additionally, further analysis revealed that the signal appeared after the command ended and lasted throughout the response period with slightly varying intensity and localization (Figure 3B).