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Acute-phase electroencephalography for an infantile atypical teratoid/rhabdoid tumor
Yuko Ichimiya1, Soichi Mizuguchi1, Yoshitomo Motomura1
1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Insights
Electroencephalography (EEG) patterns in children with rapidly growing brain tumors, like atypical teratoid/rhabdoid tumors (AT/RT), show rapid cortical suppression. This highlights the critical electrophysiological changes in pediatric brain tumors.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Clinical Electrophysiology
Background:
- Primary brain tumors are a significant cause of mortality in children.
- Electroencephalography (EEG) patterns in pediatric brain tumors require further investigation, especially in rapidly progressing cases.
Observation:
- A 14-month-old boy presented with seizures and unresponsiveness.
- Initial EEG showed disorganized activity, rapidly progressing to suppression-burst and then generalized cortical suppression.
- MRI revealed a midline pineal gland tumor extending to the brainstem.
Findings:
- The tumor was pathologically diagnosed as atypical teratoid/rhabdoid tumor (AT/RT) with SMARCB1 deficiency.
- Rapid electrophysiological deterioration correlated with tumor progression.
- AT/RT can lead to critical clinical and electrophysiological decline within days.
Implications:
- This case underscores the utility of serial EEG monitoring in identifying rapid neurological decline in pediatric brain tumors.
- Understanding these acute EEG changes is crucial for timely diagnosis and management of aggressive pediatric brain tumors.
- AT/RT represents an aggressive pediatric brain tumor with a devastatingly rapid clinical course.
Background:
Primary brain tumor is a leading cause of death in cancer-bearing children. Acutely progressive patterns of electroencephalography (EEG) remain to be investigated for children with rapidly growing brain tumors.
Case Report:
A 14-month-old boy was transferred to our department for prolonged seizures and unrecovered consciousness on his fifth day of illness. The EEG recording on admission showed highly disorganized background activity with high-voltage rhythmic delta waves. Serial EEG monitoring revealed a rapid transition of the background activity to the suppression-burst pattern, and then to generalized suppression of cortical activity within a few hours after admission. Magnetic resonance imaging detected a midline tumor at the pineal gland extending to the midbrain and pons. The tumor was pathologically confirmed as atypical teratoid/rhabdoid tumor (AT/RT) with absent expression of SMARCB1. He died of tumor progression on the 20th day after admission.
Conclusion:
AT/RT is an additional category of brain tumors that cause the clinically and electro-physiologically critical condition in a few days after the onset.
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