TL;DR: It is suggested that some patients may develop a psychotic illness after resection of a ganglioglioma that led to intractable seizures and the mechanism of this association remained unclear.
Abstract: Summary: Purpose: David Taylor and Murray Falconer suggested that some patients may develop a psychotic illness after resection of a ganglioglioma that led to intractable seizures. They implied that the mechanism of this association remained unclear. This concept is currently not universally accepted (M. Trimble, personal communication).
Methods: We studied six children or young adults from four centers who developed psychosis after resection of a ganglioglioma or dysembryoplastic neuroepithelioma (DNET).
Results: All patients were operated on because of intractable epilepsy. The lesions involved mainly the temporal lobe. Patients had good outcomes for seizure control. In none of the six was potentially psychogenic medication used nor were the psychotic symptoms postictal in nature. The psychosis was schizophreniform with paranoid features and prominent depressive symptoms. Although some behavioral abnormalities were described preoperatively, none had been psychotic before operation. This type of psychotic reaction was not encountered in the four centers in a comparable period after resection of other types of lesions. This complication is rare; it occurred in only one of 39 patients who had such a lesion resected.
Conclusions: Psychotic illness may rarely occur after resection of a ganglioglioma or DNET for treatment of intractable epilepsy. This does not seem to occur after removal of other types of lesions. Because the patients had good outcomes for seizures, the mechanism may be related to “forced normalization.” The original observations of Taylor and Falconer are confirmed by this study; the reasons for the selective occurrence, however, remain speculative.
TL;DR: An updated and comprehensive review on dysembryoplastic neuroepithelial tumor (DNET) focusing on differential diagnosis, atypical presentation, seizure outcome, and risk of malignant transformation is conducted.
TL;DR: An additional imaging sign is described, the hyperintense ring sign on FLAIR images, which is distinct and is fairly sensitive and specific for DNET, and it is believed this sign is a helpful adjuvant to preoperatively diagnose these tumors.
Abstract: Purpose The aim of our study is to describe the hyperintense ring sign on fluid-attenuated inversion recovery (FLAIR) images in patients with dysembryoplastic neuroepithelial tumors (DNET), to discuss the radiopathologic correlation for this appearance, and to determine its role in preoperative diagnosis of DNETs. Materials and methods We retrospectively analyzed imaging features in 11 patients with pathological diagnosis of DNET. All patients had undergone surgery for refractory seizures. All had FLAIR imaging sequences performed on a 1.5-T magnetic resonance scanner. Clinical and pathological details in all cases were examined. Twenty-one age matched patients with pathologically confirmed low-grade glioma (n = 11), oligodendroglioma (n = 2), and ganglioglioma (n = 8) in similar locations acted as control cases. Ten patients had follow-up imaging. Results There were 11 patients with DNET (5 girls and 6 boys). The age of presentation varied from 4 to 18 years (average, 9 years 1 month). Tumors were located in the temporal (n = 5), frontal (n = 4), parietal (n = 1), and occipital (n = 1) lobes. In 9 patients (82% sensitivity), the FLAIR images showed a well-defined hyperintense ring around these tumors, either as a complete or incomplete ring. Among the 21 control cases, the hyperintense ring sign was seen in 2 cases (90% specificity): one with low-grade glioma and one with ganglioglioma. Pathological evaluation of the DNETs suggested the hyperintense ring might correspond to the presence of peripheral loose neuroglial elements. Postoperative imaging showed partial residual ring in 3 patients, all of whom had persistent seizures. One patient had recurrent DNET at second surgery. Conclusion Magnetic resonance imaging findings of DNET are well described. We describe an additional imaging sign, the hyperintense ring sign on FLAIR images, which is distinct and is fairly sensitive and specific for DNET. We believe this sign is a helpful adjuvant to preoperatively diagnose these tumors. The presence of this ring on postoperative imaging may indicate residual or recurrent tumor.
TL;DR: Evidence from the study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.
Abstract: We report our experience regarding evaluation, surgical treatment and outcomes in a population of 21 children with histopathologically confirmed developmental tumours [nine dysembryoplastic neuroepithelial tumours (DNET), ten gangliogliomas (GG) and two gangliocytomas (GC)] and related epilepsy, analyzing video-EEG, MRI and neuropsychological data, before and after surgery. Most children had focal epilepsy correlating well with lesion location. One patient had epileptic spasms and generalized discharges. Tumours were located in the temporal lobe in 13 patients. Mean age at surgery was 11.16 years. Postsurgical MRI showed residual tumour growth in one DNET. One child had a recurrent ganglioglioma with anaplastic transformation. At latest follow-up (mean 4.68 years) 95.2% of patients were seizure-free and no significant neuropsychological declines were observed. Evidence from our study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.
TL;DR: In children, DNET may have atypical location, characteristics and behavior, including growth of primary or residual lesions and multifocal tumor, and the need for follow-up of patients with DNET post-resection is emphasized.
Abstract: Introduction
Dysembryoplastic neuroepithelial tumors (DNET) are classically considered as benign, cortically based tumors that are stable However, there were case reports that suggested DNET may not be as benign as previously thought The purpose of our study was to identify atypical location, characteristics, and behavior of DNET in children