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Hypsarrhythmia is associated with widespread, asymmetric cerebral hypermetabolism
Julius K Weng1, Regina Ahn2, Shaun A Hussain1
1Division of Pediatric Neurology, UCLA Mattel Children's Hospital, David Geffen School of Medicine, Los Angeles, CA, USA.
Insights
Hypsarrhythmia, a pattern seen in infantile spasms, is linked to increased brain metabolism, particularly in the left hemisphere. This widespread hypermetabolism suggests hypsarrhythmia may represent a quasi-ictal state.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Epileptology
Background:
- Hypsarrhythmia is the characteristic interictal electroencephalogram (EEG) pattern associated with infantile spasms.
- Understanding the metabolic consequences of hypsarrhythmia is crucial for comprehending the pathophysiology of infantile spasms.
Purpose of the Study:
- To investigate the regional cerebral metabolic activity in children with infantile spasms, comparing those with and without hypsarrhythmia.
- To evaluate the metabolic impact of hypsarrhythmia using fluorodeoxyglucose-positron emission tomography (FDG-PET).
Main Methods:
- Retrospective analysis of patients with video-EEG confirmed infantile spasms who underwent simultaneous interictal EEG and FDG-PET.
- Measurement of pons-normalized relative cerebral metabolic activity (RCA) in 18 cortical and 6 subcortical regions of interest (ROIs).
Main Results:
- Sixty-three patients were analyzed, categorized by EEG patterns: hypsarrhythmia (n=9), high-voltage EEG background (n=20), and multifocal independent spike discharges (MISD) (n=34).
- Hypsarrhythmia was associated with hypermetabolism in 11 of 18 cortical ROIs after adjusting for age.
- The association between hypsarrhythmia and hypermetabolism was more pronounced in the left hemisphere after accounting for lateralized epileptogenic zones.
Conclusions:
- Hypsarrhythmia is linked to widespread elevations in relative cerebral metabolic activity, notably more in the left hemisphere.
- These metabolic changes are not always apparent on routine review of PET scans.
- The findings suggest that hypsarrhythmia may function as a quasi-ictal phenomenon due to extensive cortical hypermetabolism.
Purpose:
Hypsarrhythmia is the interictal EEG pattern most often associated with infantile spasms. We set out to evaluate the metabolic impact of hypsarrhythmia among patients with infantile spasms by contrasting regional cerebral metabolic activity among children with and without hypsarrhythmia.
Methods:
Patients with video-EEG confirmed infantile spasms who underwent simultaneous interictal EEG and FDG-PET as part of a surgical evaluation were retrospectively identified. Pons-normalized relative cerebral metabolic activity (RCA) was ascertained in 18 cortical and 6 subcortical pre-specified regions of interest (ROIs).
Results:
We identified 63 patients with infantile spasms who underwent simultaneous EEG/PET, including children with hypsarrhythmia (n = 9), high-voltage EEG background (n = 20), and multifocal independent spike discharges (MISD) (n = 34). Among them, a putative epileptogenic zone was identified within the left-hemisphere only (n = 27), right-hemisphere only (n = 20), or assumed to be bilateral (n = 16). After adjustment for age at PET, the presence of hypsarrhythmia was associated with hypermetabolism in 11 of 18 cortical ROI's. After adjustment for lateralized epileptogenic zones, the association between hypsarrhythmia and hypermetabolism was generally stronger within the left hemisphere.
Conclusion:
Hypsarrhythmia is associated with widespread-and curiously left more than right-elevations in pons-normalized RCA, which is not evident on routine clinical review of individual PET studies. This study suggests that hypsarrhythmia may be a quasi-ictal phenomenon based on widespread and usually bilateral cortical hypermetabolism.
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