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Longitudinal Findings of MRI and PET in West Syndrome with Subtle Focal Cortical Dysplasia
Y Sakaguchi1, H Kidokoro1,2, C Ogawa1
1From the Departments of Pediatrics (Y.S., H. Kidokoro, C.O., Y.O., Y.I., H.Y., A.O., T. Nakata, J.N.).
Background And Purpose:
Despite the development of neuroimaging, identification of focal cortical dysplasia remains challenging. The purpose of this study was to show the longitudinal changes of MR imaging and FDG-PET in patients with West syndrome and subtle focal cortical dysplasia.
Materials And Methods:
Among 52 consecutive patients with West syndrome, 4 were diagnosed with subtle focal cortical dysplasia on 3T MR imaging. MR imaging and PET findings were evaluated longitudinally at onset and at 12 and 24 months of age.
Results:
At the onset of West syndrome, MR imaging demonstrated focal signal abnormalities of the subcortical white matter in 2 patients. In the other 2 patients, focal subcortical high-intensity signals became visible on follow-up T2WI as myelination progressed. PET at onset showed focal cortical hypometabolism in 3 patients, with 1 of these patients also having focal hypermetabolism and 1 having normal findings. On PET at 24 months, hypometabolism persisted in 2 patients and disappeared in 1, and hypermetabolism disappeared in 1. In 1 patient with normal MR imaging and PET findings at onset, focal hyperintensity and hypometabolism first appeared at 24 months of age. The findings on MR imaging and PET in these patients evolved differently with brain maturation and the clinical course.
Conclusions:
Subtle focal cortical dysplasia can be undetectable on MR imaging at the onset of West syndrome and is not always accompanied by hypometabolism or hypermetabolism on PET. Longitudinal MR imaging and PET studies may be useful for detecting such lesions. Even in West syndrome with a congenital structural abnormality, PET findings evolve differently with brain maturation and the clinical condition.
Insights
Subtle focal cortical dysplasia in West syndrome may not be visible on initial MR imaging or PET scans. Longitudinal neuroimaging studies are crucial for detecting these evolving changes in brain maturation.
Area of Science:
- Neuroimaging
- Pediatric Neurology
- Epilepsy Research
Background:
- Focal cortical dysplasia (FCD) diagnosis is challenging despite advanced neuroimaging.
- West syndrome often presents with subtle FCD that can be difficult to detect early.
Purpose of the Study:
- To investigate the longitudinal changes in MR imaging and FDG-PET findings in pediatric patients with West syndrome and subtle FCD.
- To assess the detectability of subtle FCD over time using serial neuroimaging.
Main Methods:
- Retrospective analysis of 52 West syndrome patients, identifying 4 with subtle FCD on 3T MR imaging.
- Longitudinal evaluation of MR imaging and FDG-PET scans at onset, 12, and 24 months of age.
Main Results:
- MR imaging showed evolving subcortical signal abnormalities and myelination-related changes.
- FDG-PET revealed dynamic changes in cortical metabolism, including hypometabolism and hypermetabolism, which varied over time.
- One patient developed detectable FCD findings only at 24 months of age.
Conclusions:
- Subtle FCD in West syndrome may be initially undetectable by MR imaging and PET.
- Longitudinal MR imaging and FDG-PET are valuable for identifying evolving FCD lesions.
- Metabolic and structural changes in FCD associated with West syndrome evolve with brain maturation and clinical course.
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