Abstract
Background: To assess the role of 2-deoxy-2(18F)-fluoro-D-glucose positron emission tomography (FDG-PET) scans in the comprehensive evaluation and surgical decision-making in patients with schizencephaly. Methods: We evaluated 11 patients (8M) with schizencephaly (mean follow-up: 4.5 years), including detailed clinical, MRI, FDG-PET, EEG, surgical and neuropathology data. Results: Eight patients had unilateral and three had bilateral clefts on MRI. Mean age at seizure onset was 20 months, with seizure being frequent in 10 and rare in one. Multiple seizure types were noted, with complex partial seizures being the most common (n=8) followed by infantile spasms (n=6). FDG-PET showed larger area of involvement than MRI in all the patients which corresponded better with the electrophysiological changes. Five patients (with unilateral disease on MRI) underwent epilepsy surgery (4 hemispherectomy and 1 multilobar resection). Two patients with focal defect on MRI underwent hemispherectomy due to larger area of abnormality revealed by FDG-PET. One patient was excluded from the surgery due to bilateral abnormalities on FDG PET. Six patients (4 with surgery) were seizure-free at last follow-up (average seizure-free duration: 70 months). One patient who underwent hemispherectomy due to apparently unilateral disease on both video-EEG and MRI but having bilateral abnormality on PET continued to have seizures. ACTH treatment had only a brief (1 month to 1 year) or no response in the six infantile spasms patients. Conclusions: FDG-PET typically shows a much larger area of involvement than MRI thus supplementing MRI in defining the full extent of malformation and assessing the functional integrity of the contralateral hemisphere. FDG-PET may prove to be a useful tool to aid in surgical decision-making and predicting surgical outcome, as patients with contralateral abnormality on FDG-PET may have poor surgical outcomes. When the malformation is unilateral with an intact contralateral hemisphere, surgery (usually hemispherectomy) may be curative of the epilepsy.
Highlights
To assess the role of 2-deoxy-2(18F)-fluoro-D-glucose positron emission tomography (FDG-PET) scans in the comprehensive evaluation and surgical decision-making in patients with schizencephaly
Neuroblast migration occurs between 16-20 weeks, and when the insult on the developing brain happens before this time, the cleft is lined by grey matter and results in schizencephaly [4]
Multiple seizure types were seen in conjunction in these patients, including complex partial seizures in 8 patients, infantile spasms in 6, some episodes of generalized tonic-clonic seizures in 3, and absence and myoclonic seizures in at least 2 patients
Summary
To assess the role of 2-deoxy-2(18F)-fluoro-D-glucose positron emission tomography (FDG-PET) scans in the comprehensive evaluation and surgical decision-making in patients with schizencephaly. Can be defined as congenital brain clefts, lined by grey matter, extending from the pial surface to the lateral ventricle. Neuroblast migration occurs between 16-20 weeks, and when the insult on the developing brain happens before this time, the cleft is lined by grey matter and results in schizencephaly [4]. It is classified into open-lip and closed lip, which is more descriptive rather than indicative of a different etiology [5]. It is a rare brain malformation, with a prevalence of around 1.5/100,000 [6, 7], and probably of multifactorial etiology [8, 9]
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