Y Maki1, J Natsume2,3,4, Y Ito1,4,5, Y Okai1,4,6, E Bagarinao4, H Yamamoto1,4, S Ogaya7, T Takeuchi8, T Fukasawa9, F Sawamura1, T Mitsumatsu1, S Maesawa4,10, R Saito10, Y Takahashi1, H Kidokoro1,4. 1. From the Departments of Pediatrics (Y.M., J.N., Y.I., Y.O., H.Y., F.S., T.M., Y.T., H.K.). 2. From the Departments of Pediatrics (Y.M., J.N., Y.I., Y.O., H.Y., F.S., T.M., Y.T., H.K.) junnatsu@med.nagoya-u.ac.jp. 3. Developmental Disability Medicine (J.N.). 4. Brain and Mind Research Center (J.N., Y.I., Y.O., E.B., H.Y., S.M. H.K.), Nagoya University, Nagoya, Japan. 5. Department of Pediatrics (Y.I.), Aichi Prefectural Mikawa Aoitori Medical and Rehabilitation Center, Okazaki, Japan. 6. Department of Pediatric Neurology (Y.O.), Toyota Municipal Child Development Center, Toyota, Japan. 7. Department of Pediatric Neurology (S.O.), Aichi Developmental Disability Center Central Hospital, Kasugai, Japan. 8. Department of Pediatrics (T.T.), Japanese Red Cross Nagoya First Hospital, Nagoya, Japan. 9. Department of Pediatrics (T.F.), Anjo Kosei Hospital, Anjo, Japan. 10. Neurosurgery (S.M., R.S.), Nagoya University Graduate School of Medicine, Nagoya, Japan.
Abstract
BACKGROUND AND PURPOSE: West syndrome is a developmental and epileptic encephalopathy characterized by epileptic spasms, neurodevelopmental regression, and a specific EEG pattern called hypsarrhythmia. Our aim was to investigate the brain activities related to hypsarrhythmia at onset and focal epileptiform discharges in the remote period in children with West syndrome using simultaneous electroencephalography and fMRI recordings. MATERIALS AND METHODS: Fourteen children with West syndrome underwent simultaneous electroencephalography and fMRI at the onset of West syndrome. Statistically significant blood oxygen level-dependent responses related to hypsarrhythmia were analyzed using an event-related design of 4 hemodynamic response functions with peaks at 3, 5, 7, and 9 seconds after the onset of each event. Six of 14 children had focal epileptiform discharges after treatment and underwent simultaneous electroencephalography and fMRI from 12 to 25 months of age. RESULTS: At onset, positive blood oxygen level-dependent responses were seen in the brainstem (14/14 patients), thalami (13/14), basal ganglia (13/14), and hippocampi (13/14), in addition to multiple cerebral cortices. Group analysis using hemodynamic response functions with peaks at 3, 5, and 7 seconds showed positive blood oxygen level-dependent responses in the brainstem, thalamus, and hippocampus, while positive blood oxygen level-dependent responses in multiple cerebral cortices were seen using hemodynamic response functions with peaks at 5 and 7 seconds. In the remote period, 3 of 6 children had focal epileptiform discharge-related positive blood oxygen level-dependent responses in the thalamus, hippocampus, and brainstem. CONCLUSIONS: Positive blood oxygen level-dependent responses with hypsarrhythmia appeared in the brainstem, thalamus, and hippocampus on earlier hemodynamic response functions than the cerebral cortices, suggesting the propagation of epileptogenic activities from the deep brain structures to the neocortices. Activation of the hippocampus, thalamus, and brainstem was still seen in half of the patients with focal epileptiform discharges after adrenocorticotropic hormone therapy.
BACKGROUND AND PURPOSE: West syndrome is a developmental and epileptic encephalopathy characterized by epileptic spasms, neurodevelopmental regression, and a specific EEG pattern called hypsarrhythmia. Our aim was to investigate the brain activities related to hypsarrhythmia at onset and focal epileptiform discharges in the remote period in children with West syndrome using simultaneous electroencephalography and fMRI recordings. MATERIALS AND METHODS: Fourteen children with West syndrome underwent simultaneous electroencephalography and fMRI at the onset of West syndrome. Statistically significant blood oxygen level-dependent responses related to hypsarrhythmia were analyzed using an event-related design of 4 hemodynamic response functions with peaks at 3, 5, 7, and 9 seconds after the onset of each event. Six of 14 children had focal epileptiform discharges after treatment and underwent simultaneous electroencephalography and fMRI from 12 to 25 months of age. RESULTS: At onset, positive blood oxygen level-dependent responses were seen in the brainstem (14/14 patients), thalami (13/14), basal ganglia (13/14), and hippocampi (13/14), in addition to multiple cerebral cortices. Group analysis using hemodynamic response functions with peaks at 3, 5, and 7 seconds showed positive blood oxygen level-dependent responses in the brainstem, thalamus, and hippocampus, while positive blood oxygen level-dependent responses in multiple cerebral cortices were seen using hemodynamic response functions with peaks at 5 and 7 seconds. In the remote period, 3 of 6 children had focal epileptiform discharge-related positive blood oxygen level-dependent responses in the thalamus, hippocampus, and brainstem. CONCLUSIONS: Positive blood oxygen level-dependent responses with hypsarrhythmia appeared in the brainstem, thalamus, and hippocampus on earlier hemodynamic response functions than the cerebral cortices, suggesting the propagation of epileptogenic activities from the deep brain structures to the neocortices. Activation of the hippocampus, thalamus, and brainstem was still seen in half of the patients with focal epileptiform discharges after adrenocorticotropic hormone therapy.
Authors: Aaron E L Warren; A Simon Harvey; Simon J Vogrin; Catherine Bailey; Andrew Davidson; Graeme D Jackson; David F Abbott; John S Archer Journal: Neurology Date: 2019-06-21 Impact factor: 9.910
Authors: K Haginoya; K Kon; S Tanaka; M Munakata; R Kato; M Nagai; H Yokoyama; S Maruoka; T Yamazaki; K Iinuma Journal: Brain Dev Date: 1999-03 Impact factor: 1.961
Authors: Ingrid E Scheffer; Samuel Berkovic; Giuseppe Capovilla; Mary B Connolly; Jacqueline French; Laura Guilhoto; Edouard Hirsch; Satish Jain; Gary W Mathern; Solomon L Moshé; Douglas R Nordli; Emilio Perucca; Torbjörn Tomson; Samuel Wiebe; Yue-Hua Zhang; Sameer M Zuberi Journal: Epilepsia Date: 2017-03-08 Impact factor: 5.864