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Purpose: For a diagnosis of Rasmussen syndrome (RS), clinical course together with electroencephalography (EEG) and magnetic resonance imaging (MRI) findings are considered important, but there are few reports on functional neuroimaging. This study investigated cerebral blood flow (CBF)-single photon emission computed tomography (SPECT), central benzodiazepine receptor (BZR)-SPECT, and fluorine-18 fluorodeoxy glucose-positron emission tomography (FDG-PET) in RS patients, and correlated neuroimaging results with MRI and pathological findings., Methods: Twenty-three patients diagnosed with RS according to Bien's (2005) diagnostic criteria (including 12 patients with a histological diagnosis) were studied. CBF-SPECT, BZR-SPECT and FDG-PET images were visually evaluated, and the findings correlated with MRI and histological findings., Results: Hypoperfusion areas were observed in 16 of 22 patients by interictal CBF-SPECT. Hyperperfusion areas were observed in 10 of 12 patients by ictal CBF-SPECT, which correlated with ictal onset area by ictal EEG (IOAE). In the limited data of BZR-SPECT in nine patients, lowered uptake was detected in all nine patients, including two with no MRI abnormalities. Lowered glucose metabolism was observed in affected areas in all five patients by FDG-PET. Histological examination revealed findings of chronic encephalitis in all 12 patients examined, concomitant with focal cortical dysplasia in five patients., Conclusion: In RS patients, functional neuroimaging reveals clear abnormal findings, even before the appearance of MRI abnormalities. BZR-SPECT and FDG-PET could detect the IOAE efficiently even in the absence of MRI abnormalities, while interictal CBF-SPECT occasionally failed to detect IOAE if MRI was normal. Based on BZR-SPECT, refractory epileptic seizures in RS may suggest possible impairment of inhibitory neurons., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Electrocorticogram (ECoG) spike activity is enhanced under general anesthesia with 1.5 minimum alveolar concentration (MAC) sevoflurane compared with lower concentrations in adult patients with epilepsy. However, the effect of concentration of sevoflurane on ECoG in children with epilepsy is less known., Aims: The primary endpoint was to investigate the effects of sevoflurane on ECoG spike activity in pediatric patients undergoing epilepsy surgery. The secondary endpoint was to examine its effects on baseline ECoG including burst suppression., Methods: Children of age 3-18 years with medically intractable epilepsy undergoing corpus callosotomy or resection of the epileptic foci (n = 11) were enrolled. Electrodes were placed on the brain surface and ECoG was recorded under anesthesia with endtidal carbon dioxide tension at 30 mmHg and sevoflurane at 2.5%, followed by age-adjusted 1.5 MAC (3.1-3.4%) for 10 min. The number of leads with spikes, the average number of spikes per lead per minute, median frequency of ECoG, and duration of suppression of ECoG ≥ 1 s were compared between 2.5% and 1.5 MAC sevoflurane., Results: The number of leads with spikes increased [11 vs 14, P = 0.003, difference in mean (95% CI) is 3 (2-5)], and the average number of spikes increased [9 vs 14·lead -1 ·min -1 , P = 0.003, difference in mean (95% CI) is 5 (2-8) lead -1 ·min -1 ] under anesthesia with 1.5 MAC compared with 2.5% sevoflurane. Median frequency was decreased [2.8 Hz vs 2.0 Hz, P = 0.003, difference in mean (95% CI) is 0.8 (0.4-1.2) Hz], and the duration of suppression was increased [105 s vs 262 s, P < 0.001, difference in mean (95% CI) is 156 (90-223) s] with 1.5 MAC compared with 2.5% sevoflurane., Conclusions: Sevoflurane at 1.5 MAC significantly increased the extent and the number of spikes, prolonged the duration of suppression, and decreased median frequency of ECoG compared with those at 2.5% sevoflurane., (© 2017 John Wiley & Sons Ltd.)

Hemorrhagic shock and encephalopathy syndrome is a devastating disease, but the pathogenesis remains unclear. The aim of this study was to examine the usefulness of neuroimaging in establishing a diagnosis and elucidating the pathogenesis. We analyzed the neuroradiologic features of 22 patients who fulfilled the Levin criteria. All patients underwent brain computed tomography (CT), and 14 patients underwent brain magnetic resonance imaging (MRI) including diffusion-weighted imaging in 10 patients. Initial CT showed normal findings in 14 of 18 (78%) patients, but subsequently hypodensities appeared in bilateral watershed zones and progressed to whole brain edema. MRI revealed cytotoxic edema, showing hyperintensities in bilateral watershed zones on diffusion-weighted imaging with a low apparent diffusion coefficient. Serial neuroimaging showed characteristic features of a widespread brain ischemic event mainly in watershed zones in hemorrhagic shock and encephalopathy syndrome., (© The Author(s) 2014.)

This study examined the usefulness of 123I-iomazenil SPECT (IMZ-SPECT), a type of brain scintigram that focuses on the central benzodiazepine receptor in order to determine its distribution and the function of inhibitory neurons. IMZ-SPECT has been used for the detection of epileptogenic foci, especially when surgical intervention is considered. Interictal study by IMZ-SPECT is widely available at numerous institutions and its usefulness has been confirmed in patients with not only focal cortical dysplasia and hippocampal sclerosis, but also tuberous sclerosis and neuronal migration disorders, even when magnetic resonance image fails to demonstrate any abnormal findings. When interpreting scintigrams, the developmental dynamic change of the central benzodiazepine receptor in childhood and the duration of the benzodiazepine exposure period should be taken into consideration. It is expected that IMZ-SPECT will be used in various neurological disorders other than epilepsy in the future allow medical services to be provided based on findings in the inhibitory synaptic system obtained with IMZ-SPECT.