UNASSIGNED: Focal cortical dysplasia (FCD) is a significant cause of drug-resistant epilepsy, often necessitating surgical intervention. Type IIb FCD poses challenges due to its strong association with drug-resistant seizures. Effective management involves advanced imaging, intraoperative neurophysiological monitoring, and precise surgical techniques. This case study illustrates these strategies in an 11-year-old female with drug-resistant epilepsy attributed to Type IIb FCD.
UNASSIGNED: The patient, an 11-year-old female, had drug-resistant seizures despite various anticonvulsant treatments. Preoperative 3 Tesla (3T) MRI revealed an ill-defined lesion in the right frontal operculum. The surgical team used neuro-navigation for intraoperative guidance and electrocorticography for lesionectomy. Pathology confirmed Type IIb FCD with rare concentric calcifications.
UNASSIGNED: Drug-resistant seizures in FCD often require surgery when medications fail. This case highlights the importance of comprehensive preoperative evaluations and advanced imaging, such as 3T MRI, to accurately identify lesions. Intraoperative neurophysiological monitoring, including electrocorticography, ensures precise resection of the epileptogenic zone. The unusual finding of concentric calcifications in Type IIb FCD is noteworthy, suggesting the need for further research to understand their impact on the disease.
UNASSIGNED: Microsurgical lesionectomy is crucial for managing drug-resistant seizures in Type IIb FCD. Combining advanced imaging with intraoperative monitoring improves surgical precision and outcomes. The rare pathological finding of calcifications highlights the diversity of FCD manifestations, warranting further study. These techniques can significantly enhance seizure control and quality of life in patients with drug-resistant epilepsy.

The transmantle sign is considered to be a magnetic resonance imaging feature specific to patients with type II focal cortical dysplasia; however, this sign can be difficult to distinguish from other pathologies, such as a radial-oriented white matter band in tuberous sclerosis. Here, we report a case showing a high-intensity area on T2-weighted and fluid-attenuated inversion recovery images extending from the ventricle to the cortex associated with atypical histopathological findings containing corpora amylacea. This case demonstrates that some instances of transmantle signs may be due to corpora amylacea accumulation.

Musicogenic epilepsy is a rare form of reflex epilepsy in which seizures are provoked by music. Different musicogenic stimuli have been identified: pleasant/unpleasant music or specific musical patterns. Several etiologies have been uncovered, such as focal cortical dysplasia, autoimmune encephalitis, tumors, or unspecific gliosis. In this article, we report two patients with musicogenic seizures. The first patient was diagnosed with structural temporal lobe epilepsy. Her seizures were elicited by music that she liked. Interictal and ictal video-electroencephalography (video-EEG) and signal analysis using independent component analysis revealed the right temporal lobe seizure onset extending over the neocortical regions. The patient underwent right temporal lobectomy (including the amygdala, the head, and the body of the hippocampus) and faced an Engel IA outcome 3 years post-surgery. The second patient was diagnosed with autoimmune temporal lobe epilepsy (GAD-65 antibodies). Her seizures were triggered by contemporary hit radio songs without any personal emotional significance. Interictal and ictal video-electroencephalography (video-EEG) and independent component analysis highlighted the left temporal lobe seizure onset extending over the neocortical regions. Intravenous immunoglobulin therapy was initiated, and the patient became seizure-free at 1 year. In conclusion, musicogenic seizures may be elicited by various auditory stimuli, the presence or absence of an emotional component offering an additional clue for the underlying network pathophysiology. Furthermore, in such cases, the use of independent component analysis of the scalp EEG signals proves useful in revealing the location of the seizure generator, and our findings point toward the temporal lobe, both mesial and neocortical regions.

BACKGROUND: The reevaluation and management of seizure relapse following resective surgery in magnetic resonance imaging (MRI)-negative pharmacoresistant epilepsy remains a significant challenge.
METHODS: A 25-year-old right-handed male with medically refractory epilepsy presented with nonlocalizing electroencephalography (EEG) and MRI. Stereo-EEG (SEEG) implantation based on semiology and positron emission tomography imaging revealed a left frontal opercular focus with rapid bilateral insular ictal synchrony. The initial epileptogenic zone was resected and pathologically proven to be type 2A focal cortical dysplasia (FCD). Seizure relapse after 9 months was eventually reinvestigated, and repeat SEEG revealed a secondary epileptogenic focus in the contralateral insula. A novel technique of volumetric stereotactic radiofrequency ablation (vRFA) was utilized for the right insular focus, following which, the patient remains seizure-free for 20 months. He suffered a transient bilateral opercular syndrome following the second intervention that eventually resolved.
CONCLUSIONS: The authors present clinical evidence to suggest epileptogenic nodes distant from the primary focus as a mechanism for seizure relapse following FCD surgery and the importance of bilateral insular SEEG coverage. The authors also describe a novel technique of minimally invasive vRFA that allows ablation of a larger volume of cerebral cortex when compared to conventional bedside SEEG electrode thermocoagulation.

BACKGROUND: Focal cortical dysplasias (FCD) represent highly intrinsically epileptogenic lesions that require complete resection for seizure control. Resection of pure motor strip FCD can be challenging. Effective control of postoperative seizures is crucial and extending the boundaries of resection in an eloquent zone remains controversial.
METHODS: The authors report a 52-year-old right-handed male with refractory epilepsy. The seizure phenotype was a focal crisis with preserved awareness and a clonic motor onset of right-hemibody. Epilepsy surgery protocol demonstrated a left pure motor strip FCD and a full-awake resective procedure with motor brain mapping was performed. Further resection of surgical boundaries monitoring function along intraoperative motor tasks with no direct electrical stimulation corroborated by intraoperative-neuromonitorization was completed as the final part of the surgery. In the follow-up period of 3-years, the patient has an Engel-IB seizure-control with mild distal lower limb palsy and no gate compromise.
CONCLUSIONS: This report represents one of the few cases with pure motor strip FCD resection. In a scenario similar to this case, the authors consider that this variation can be useful to improve seizure control and the quality of life of these patients by extending the resection of a more extensive epileptogenic zone minimizing functional damage.

BACKGROUND: Focal cortical dysplasias (FCDs) are a heterogenous cluster of histopathologic entities classically associated with medically refractory epilepsy. Because there is substantial histopathologic variation among different types of FCD, there are likely multiple pathogenic mechanisms leading to these disorders. The meninges are known to play a role in cortical development, and disruption of meningeal-derived signaling pathways has been shown to impact neurodevelopment. To our knowledge, there has not yet been an investigation into whether genetic pathways regulating meningeal development may be involved in the development of FCD.
METHODS: The authors reported a patient with refractory epilepsy and evidence of FCD on imaging who received surgical intervention and was found to have an unusual dural anomaly overlying a region of type Ic FCD. To the authors\' knowledge, this was the first report describing a lesion of this nature in the context of FCD.
CONCLUSIONS: The dural anomaly exhibited by the patient presented what could be a potentially novel pathogenic mechanism of FCD. Resection of the cortical tissue underlying the dural anomaly resulted in improvement in seizure control. Although the pathogenesis is unclear, this case highlighted the importance of further investigation into the developmental origins of FCD, which may help elucidate whether a connection between meningeal development and FCD exists.

Cortical tubers are one of the typical intracranial manifestations of tuberous sclerosis complex (TSC). Multiple cortical tubers are easy to diagnose as TSC; however, a solitary cortical tuber without any other cutaneous or visceral organ manifestations can be confused with other conditions, particularly focal cortical dysplasia. We report a surgical case of refractory epilepsy caused by a solitary cortical tuber mimicking focal cortical dysplasia type II, and describe the radiological, electrophysiological, and histopathological findings of our case.

OBJECTIVE: EEG anomalies and epilepsy are a not so rare clinical manifestation in patients with Phosphatase and tensin homolog (PTEN) variants. The main aim of this study is to analyze the characteristics of EEG traces, neuroimaging findings and epilepsy to better define the neurological aspects in a set of patients with PTEN variants collected in four Italian Centres. As a secondary aim, we describe the neurodevelopmental profile and the psychiatric comorbidities of this cohort.
METHODS: Patients with PTEN variants, identified by Sanger sequencing or target resequencing, were enrolled. For each subjects, clinical data were retrospectively extracted from medical charts, with a focus on epilepsy and neuroimaging data.
RESULTS: 54 patients with PTEN variants were enrolled, with a mean age of 18.8 years. 72.2% have at least one psychiatric diagnosis, being Autism Spectrum Disorder and Intellectual Disability the most frequent diagnosis (29 and 25 cases, respectively). 22 subjects show an abnormal EEG and 8 received a diagnosis of epilepsy, mainly focal epilepsy (7/8), with a mean age at seizure onset of 3.8 years. 3/8 subjects have a drug resistant epilepsy, independently from the underlying neuroimaging pattern. The finding of a Focal cortical dysplasia is significantly associated with both an abnormal EEG (p = 0.02) and the occurrence of seizures (p = 0.002).
CONCLUSIONS: EEG should be taken into consideration in the first-line diagnostic flowchart of subjects with PTEN variants. The onset of a focal epilepsy, independently from its response to antiepileptic drugs, highly recommends to carry out a neuroimaging exam.

BACKGROUND: Rasmussen syndrome (RS) is a rare neurological disorder characterized by unilateral chronic inflammation, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. There has been no detailed pathological evaluation or finding, including focal cortical dysplasia, for bilateral RS.
METHODS: A 13-year-old boy presented with status epilepticus with focal to bilateral tonic clonic seizure starting from the left upper limb. At the age of 15, epilepsia partialis continua of the right face and upper extremities appeared, and MRI showed hemispheric abnormal signal intensities with left frontal lobe predominance. Three months later, MRI showed extensive abnormal signal intensities in the right occipitoparietal and left temporal lobes. Tacrolimus was useful in preventing recurrence. Because the seizures were intractable, a corpus callosotomy was performed at 16 years along with a concurrent brain biopsy from the bilateral lateral frontal cortices. We detected dysmorphic neurons in addition to inflammatory changes suspicious for RS, leading to a diagnosis of focal cortical dysplasia (FCD) type Ⅱa and suspected bilateral RS. Total callosotomy and vagus nerve stimulation were not sufficiently effective.
CONCLUSIONS: In bilateral RS, FCD may be present in both cerebral hemispheres. In the current case, an autoimmune response to dysmorphic neurons may have contributed to the pathogenesis of intense inflammation.

BACKGROUND: Focal epilepsy can have a varied etiology, including malformations of cortical development (MCD), that can often be detected by Magnetic Resonance Imaging (MRI).Here we show a distinct characteristic of two forms of MCDs on MRI, with two tight dipole clusters in her MEG magnetoencephalography study, in a patient with electroencephalography (EEG) features of generalized epilepsy.
METHODS: This is a case presentation of a 20 years old female with epilepsy, found to have upon EMU admission two pathologies (FCD, heterotropia) over the right side near the collateral sulcus, and two tight clusters of dipoles over the right parietal and left temporo-parietal region, with generalized inter ictal discharges in her EEG. FCD is a common etiology of medically intractable seizures and usually in EEG it will show either: pseudo-periodic spikes or rhythmic spikes, poly-spike or repetitive electrographic seizures or a brief discharge of fast rhythmic activity, atypical presentation with generalized epileptiform discharges were rarely reported.
CONCLUSIONS: The presence of MCD does not preclude a patient from having other types of epilepsy. Generalized epilepsy and focal related epilepsy have a distinct pathophysiology.