PETSurfer-Based Brain Segmentation in Patients with Temporal Lobe Epilepsy and Associated Hippocampal Sclerosis.

PURPOSE: In a homogeneous cohort of mesial temporal lobe epilepsy (mTLE) patients with hippocampal sclerosis (HS), this study utilizes the PETSurfer method to quantify and localize areas of cerebral hypometabolism. METHODS: We selected patients from the University Clinical Center of Serbia who all underwent anterior temporal lobectomy with amygdalohippocampectomy and achieved seizure freedom (Engel class I). Our analysis involved integrating FDG-PET and MRI imaging to compare glucose metabolism between the hemispheres ipsilateral and contralateral to HS. RESULTS: The quantitative PETSurfer approach identified significant hypometabolism restricted to the ipsilateral temporal lobe structures-the amygdala, hippocampus, temporal pole, superior and middle temporal gyrus-and the ipsilateral thalamus. The lack of significant hypometabolism in extratemporal regions indicates that these 'pure' mTLE cases may not involve the broader network disruptions typically associated with more extensive epileptic pathologies. The effect sizes ranged from small to medium, indicating variable degrees of metabolic reduction across different structures. CONCLUSION: These findings highlight the localized nature of the epileptogenic focus in HS-related mTLE with good surgical outcome. However, the small sample size and potential cohort bias, necessitate caution in generalizing these results. Future research would benefit from a comparative approach incorporating a control group, providing a broader context for interpreting these hypometabolic patterns.

Multinodular and vacuolating neuronal tumour of the cerebrum: a rare neuroimaging incidentaloma or a potentially treatable cause of focal epilepsy?

Multinodular and vacuolating neuronal tumour (MVNT) of the cerebrum is a relatively new, well defined histopathological and neuroradiological entity, in many cases associated with an early adult-onset epilepsy. These lesions have an indolent course and resemble both malformative and neoplastic processes, combining a focal developmental anomaly and a low-grade tumour. Herein, we report a case of a 48-year-old female patient with left temporal lobe epilepsy associated with MVNT. In addition, a comprehensive review of all the previously published cases is provided with a focus on seizure-related cases, surgical treatment, and postoperative outcome.

Surgical management of meningoencephalocele in temporal bone associated with pharmacoresistant epilepsy: report of two cases.

We report good outcome after surgical treatment of two patients with meningoencephalocele associated with pharmacoresistant temporal lobe epilepsy. Surgical management of meningoencephaloceles may result in seizure freedom, although optimal surgical strategy is still controversial.

Widespread cortical PET abnormalities in an adolescent related to a PNES dissociative state, PTSD, ADHD, and domestic violence exposure.

BACKGROUND: Accumulated evidence indicates that exposure to trauma is associated with the development of cognitive impairments and psychiatric symptoms in children and adolescents. OBJECTIVE: In this case study of a female adolescent of 17 years, we aimed to evaluate how cortical positron emission tomography (PET) abnormalities relate to psychogenic non-epileptic seizure (PNES) dissociative state, post-traumatic stress disorder (PTSD), attention-deficit/hyperactivity disorder (ADHD), and domestic violence exposure. METHODS: Detailed psychiatric and neuropsychological assessment was performed initially, followed by a PET study. The PET imaging was carried out in the resting-state and in the dissociative-state. RESULTS: The adolescent was suffering from multiple episodes of unconsciousness, all found to be psychogenic; thus, PNES was diagnosed. However, at the psychopathology symptom level, the adolescent had heightened impulsivity, hyperactivity, hyperarousal, anxiety, somatic, and dissociative/ functional neurological symptoms present separately or concurrently at some point during her life; thus, the criteria for PTSD and ADHD were also fulfilled. In the resting state, significant hypometabolism was observed in the occipital, occipitotemporal, polar, and mesial parts of the temporal regions bilaterally, fronto-parietal medial and lateral pericental regions, and fronto-temporal and insular region on the left. The most intense metabolism was observed in the posterior cingulate gyrus and the medial parts of the posterior parietal lobe. In the dissociative state, there was a slight increase in the metabolism of the brain globally compared with the resting state, but with identical distribution of the regional changes observed. CONCLUSIONS: Widespread cortical PET abnormalities were found, possibly indicating alterations in large-scale brain networks, in a patient with PNES and a dissociative state, PTSD, and ADHD, who was exposed to chronic domestic violence.

Brain positron emission tomography in patients with myotonic dystrophy type 1 and type 2.

AIM: To determine regions of reduced brain metabolism in patients with myotonic dystrophy type 1 (DM1) and type 2 (DM2) using 18F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET), and to analyse their potential association with cognitive deficit. METHOD: Study included 29 patients (16 DM1 and 13 DM2). FDG-PET and detailed neuropsychological testing were performed in both groups. RESULTS: The most common cognitive findings were executive, visuospatial, and naming dysfunction in DM1, and executive and naming dysfunction in DM2. FDG-PET showed the most prominent glucose hypometabolism in prefrontal, temporal, and pericentral regions in both DM1 and DM2 patients, with additional affection of insula and subcortical grey matter in DM2. In DM1 patients, we found association between right frontotemporal hypometabolism and executive dysfunction (p<0.05). In DM2 patients attention deficit was in association with prefrontal, insular, and striatal hypometabolism, as well as right frontotemporal hypometabolism (p<0.05). Executive dysfunction in DM2 was more common in patients with prefrontal and insular hypometabolism, right parietotemporal and frontotemporal hypometabolism, as well as left striatal hypometabolism (p<0.05). Patients with parietotemporal defect on FDG-PET were more likely to have naming dysfunction (p<0.01). CONCLUSION: FDG-PET findings corresponded well with the results of neuropsychological testing. FDG-PET may be a good biomarker of central nervous system involvement in DM1 and DM2, but this hypothesis will have to be more strongly supported by larger studies.

The utility of FDG-PET in the differential diagnosis of Parkinsonism.

INTRODUCTION: Differential diagnosis of parkinsonian disorders can be difficult on clinical grounds, especially in the early stage. Recent advancements in 18-F-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging reveals different patterns of regional glucose metabolism in idiopathic Parkinson's disease (IPD) and atypical parkinsonian syndromes, such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), which may help differentiating between these conditions. PURPOSE: To assess the utility of FDG-PET imaging in differential diagnosis of Parkinsonism in clinical practice. METHODS: FDG-PET was performed in 72 patients with parkinsonism (age 34-80 years) referred to our center by movement disorder specialists. FDG-PET diagnosis was obtained by visual assessment of individual scans combined with voxel-based statistical parametric mapping analysis. FDG-PET diagnosis assigned at the time of imaging was compared with the final clinical diagnosis made by the movement disorder specialists after ≥2 years follow-up. RESULTS: FDG-PET findings were consistent with IPD in 27, MSA in 18, PSP in 19 and CBS in 2 patients. The final clinical diagnosis was IPD in 29, MSA in 20, PSP in 21 and CBS in 2 patients. Concordance between the FDG-PET and clinical diagnoses was 92% in the overall sample (IPD 93%, MSA 90%, PSP 91% and CBS 100%). The diagnostic accuracy of FDG-PET was 93% for IPD and MSA and 97% for PSP. CONCLUSION: FDG-PET may help differentiate between IPD, MSA, PSP and CBS among patients presenting with parkinsonian symptoms, which is important for patient counselling and making early decisions about treatment.

Dopamine transporter imaging (123)I-FP-CIT (DaTSCAN) SPET in differential diagnosis of dopa-responsive dystonia and young-onset Parkinson's disease.

Dopa-responsive dystonia (DRD) is a genetic disorder characterized by childhood onset dystonia, dominant inheritance, diurnal symptoms fluctuation and positive levodopa response. Adult-onset DRD is frequently combined with parkinsonism and can be mistaken with young onset Parkinson's disease (YOPD). Both conditions are caused by dopamine deficiency, due to nigral cells' loss in YOPD, and due to enzymatic defects in dopamine synthesis in DRD. Single photon emission tomography (SPET) with (123)I-N--fluoropropyl-2b-carbomethoxy-3b-(4-iodophenyl) nortropane ((123)I-FP-CIT)-DaTSCAN is a sensitive neuroimaging method for the assessment of nigrostriatal dopaminergic system integrity and degeneration. Our aim was to evaluate the usefulness of (123)I-FP-CIT( DaTSCAN) SPET in the differential diagnosis of DRD and YOPD in clinical practice. Brain SPET with (123)I-FP-CIT was performed in 13 patients (7 males, 6 females), age 20-58 years, with mean age of onset of their disease, 29 years, eleven patients with early onset parkinsonian symptoms and 2 with genetically proved DRD. The images were evaluated by visual and semiquantitative analyses (ROI). The ratio of specific-striatal to non specific-occipital binding was calculated. Ten out of 11 patients with YOPD had decreased accumulation of DaTSCAN in striatum, especially in putamen, that is typical findings for Parkinson's disease. In three patients DaTSCAN was normal with symmetric tracer uptake in both striata, caudate nucleus and putamen and the diagnosis of DRD was suspected. Two patients with initial dystonic symptoms and genetically proved DRD had normal DaTSCAN. In one patient after normal DaTSCAN findings the initial diagnosis of YOPD was changed to the diagnosis of DRD. Region of interest (ROI) analyses have shown significantly lower(123)I-FP-CIT binding ratios in YOPD than in DRD in all 3 regions of interest: striatum (1.95±0.32) vs (2.76±0.10) P<0.001, putamen (1.76±0.25) vs (2.84±0.14) P<0.0001 and caudate nucleus (2.37±0.51) vs (3.27±0.14) P<0.01. In conclusion, our results indicate that DaTSCAN is an objective neuroimaging method able to distinguisch neurodegenerative disease YOPD from DRD and clarify a clinical dilemma, which is important for the treatment, prognosis and genetic counseling of patients and their families.

Bálint-like syndrome as an unusual representation of non-convulsive status epilepticus.

The clinical signs of posterior cortex dysfunction are, due to their paucity and subtlety, very often ignored as non-specific during clinical evaluation of non-convulsive status epilepticus. Therefore, focal non-convulsive status epilepticus emerging from the posterior cortex, and especially the parietal lobes, can be fairly under-recognised. We report a 66-year-old patient with focal non-convulsive status epilepticus presenting as isolated Bálint-like syndrome, successfully treated to full clinical and electrophysiological recovery. The diagnostic and pathophysiological features are discussed. Focal non-convulsive status epilepticus can be associated with negative phenomena such as neuropsychological deficits mimicking those detected more often in degenerative and vascular brain diseases. [Published with video sequences].