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Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment
H T Chugani1, W D Shields, D A Shewmon
1Department of Neurology, University of California, Los Angeles 90024.
Insights
Positron emission tomography (PET) effectively identified unsuspected focal cortical dysplasia in infants with infantile spasms. Surgical removal of these foci led to seizure freedom, improving prognosis for these children.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Medical Imaging
Background:
- Infantile spasms, particularly cryptogenic spasms, present a diagnostic challenge.
- Identifying the underlying cause is crucial for effective treatment and improved prognosis.
Purpose of the Study:
- To evaluate the utility of Positron Emission Tomography (PET) in detecting focal cortical dysplasia in infants with cryptogenic spasms.
- To correlate imaging findings with electroencephalography (EEG) and surgical outcomes.
Main Methods:
- PET scans assessed local cerebral glucose metabolism in 13 infants with cryptogenic spasms.
- Cranial computed tomography (CT) and Magnetic Resonance Imaging (MRI) were performed.
- EEG monitored brain activity, and surgical intervention was guided by electrocorticography.
Main Results:
- PET revealed unilateral hypometabolism in the parieto-occipito-temporal region in 5 infants.
- MRI showed subtle abnormalities in only 1 infant, highlighting PET's sensitivity.
- EEG demonstrated hypsarrhythmia or localized abnormalities corresponding to PET findings.
- Four infants underwent surgery for suspected cortical dysplasia and achieved seizure freedom.
Conclusions:
- PET is a valuable tool for identifying unsuspected focal cortical dysplasia in infants with cryptogenic spasms.
- Early detection through PET facilitates surgical intervention, leading to improved seizure control and prognosis.
Abstract:
Positron emission tomography (PET) of local cerebral glucose metabolism in 13 children with infantile spasms of undetermined cause (cryptogenic spasms) revealed unilateral hypometabolism involving the parieto-occipito-temporal region in 5 female infants. Cranial computed tomography showed normal findings in all infants. Magnetic resonance imaging (MRI) demonstrated a normal appearance in 4 of the 5 infants; in 1 infant, MRI revealed a subtle abnormality consisting of poor demarcation between occipital gray and white matter. Surface electroencephalography (EEG) in 4 showed hypsarrythmia at some time in the patients' courses, but at other times showed localized or lateralized abnormalities corresponding to areas of PET-detected hypometabolism. Because of poor seizure control, 4 infants underwent surgical removal of the cortical focus guided by intraoperative electrocorticography and were seizure free postoperatively. Neuropathological examination of resected tissue in each showed microscopic cortical dysplasia. Our findings indicate that in infants with cryptogenic spasms, PET can effectively identify those due to unsuspected focal cortical dysplasia, for which resective surgery offers improved prognosis.