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Miller-Dieker syndrome (MDS) is characterized by facial abnormalities and lissencephaly and is caused by a microdeletion in the region containing the LIS1 gene at chromosome 17p13.3. We report a case in which postnatal neuroimaging revealed severe lissencephaly. A 9-month-old boy presented with infantile spasms syndrome. Because of the refractory course of seizures and continued poor vitality, total corpus callosotomy was performed at 28 months of age. Intraoperative electroencephalogram (EEG) showed that the bilateral synchronous epileptiform discharges disappeared immediately after the disconnection. Postoperatively, the epileptic spasms (ES) in clusters disappeared, and single ES followed by focal seizures became the main symptom. The patient smiled more and became more responsive to stimuli. Postoperative scalp interictal EEG showed desynchronized multifocal spike and wave discharges with a marked decrease in the bilateral synchronous spike and wave discharges. Our findings suggest that the corpus callosum is involved in the mechanism ES in clusters in MDS-associated lissencephaly, and total callosotomy could be a therapeutic option.

Dysembryoplastic neuroepithelial tumors (DNTs) are benign brain tumors classified as grade 1 in the 2021 World Health Organization (WHO) classification of central nervous system tumors. DNTs rarely undergo malignant transformation and cause symptomatic intracranial hemorrhage. We report a case of malignant transformation of DNT presenting with intraventricular hemorrhage and review the literature on malignant transformation of DNTs. An 18-year-old woman with a history of epilepsy presented with a sudden headache and vomiting. Radiological examination revealed a mass lesion in the left parietal lobe and intraventricular hemorrhage. The patient underwent an emergency craniotomy for brain tumor resection. The lesion was pathologically diagnosed as a malignant transformation of DNT. She had been followed up without tumor recurrence for 2 years after surgery.

Background: Glioblastoma (GBM) is a rapidly growing malignant brain tumor that varies in shape and size and causes various clinical symptoms. A case of a right temporal lobe GBM with a very rare presentation as an acute ischemic stroke due to anterior choroidal artery (AchoA) occlusion is presented along with a review of the literature. Case description: A 74-year-old woman presented with acute onset of severe left hemiparesis. Imaging showed an acute ischemic lesion in the territory of the right AchoA and a right temporal lobe tumor. Tumor resection with anterior temporal lobectomy was performed. The operative findings showed that the AchoA was encased in the tumor. The pathological examination and the genetic analysis led to the diagnosis of GBM. Conclusions: Though it is very rare, GBM can cause acute ischemic stroke by mechanical compression, arterial dissection, or tumor infiltration to the vascular wall.

We present a case of drug-resistant focal motor seizures in which separate cortico-cortical epileptic networks within the supplementary motor area (SMA) proper and primary motor area (PMA) were proven by ictal high-frequency oscillation (HFO) and cortico-cortical evoked potential (CCEP). A 12-year-old girl presented with two types seizures: type A, tonic extension and subsequent clonic movements of the right arm; and type B, tonic and clonic movements of the right leg. MRI was normal and karyotype genetic analysis revealed 46,X,t(X;14)(q13;p12). She underwent placement of chronic subdural electrodes over the left hemisphere. We recorded a total of nine seizures during 10 days of epilepsy monitoring. Type A seizures started from the lower part of the left SMA proper and early spread to the hand motor area of the PMA. Type B seizures started from the upper part of the SMA proper and early spread to the leg motor area of the PMA. CCEPs of both SMA proper and PMA activated two identical routes for evoked potentials correlating with separate pathways. Corticectomy of the left SMA proper and PMA achieved seizure-free without hemiparesis. Within a small homunculus of the SMA proper, separate epileptic networks were proven and validated by seizure semiology, ictal HFO, and CCEP.

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare pathological lesions that can present anywhere in the central nervous system. Symptoms vary depending on the location, though they often include seizures, especially in intracranial and supratentorial lesions. A case of intracranial supratentorial CAPNON presenting with drug-resistant left temporal lobe epilepsy is reported. The patient had a history of drug-resistant focal seizures for over 36 years. The lesion was located in the left mesial temporal lobe, but hippocampal sclerosis and hippocampal invasion were not apparent. The lesion was removed without hippocampectomy, and the patient has been seizure-free for one year. Keywords: Calcifying pseudoneoplasms of the neuraxis, CAPNON, drug-resistant epilepsy, supratentorial, surgical treatment, temporal lobe epilepsy