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Purpose: We hypothesized that epilepsy associated with temporal pole encephaloceles (ETPE) could be the consequence and an unrecognized manifestation of idiopathic intracranial hypertension (IIH). To test this hypothesis in patients with ETPEs we evaluated: 1) the frequency of two radiological signs of IIH and 2) whether these patients develop over time clinical manifestations suggestive of elevated intracranial pressure (ICP). Methods: Case-control study comparing two cardinal radiological signs of IIH pituitary gland height (PGH) and the diameter of the two optic nerve sheaths (ONS) between 29 patients with ETPEs (TPE group) and 29 patients with focal epilepsy of other etiologies (control group), adjusted by age, sex, body mass index (BMI), age at epilepsy onset and epilepsy duration. Analysis was performed using conventional and ordinal logistic regression. The measurements in both groups were compared with validated radiological criteria of IIH. Results: Of the patients 17 (63%) in the TPE group had all three measurements over the cut-off values for IIH, while no patients in the control group had all three findings. The TPE group patients had lower PGH (3.2 ± 1.0 mm vs. 4.9 ± 1.3 mm, p < 0.001) and larger diameter of ONS than controls (p < 0.001), being similar to validated data of IIH. No patient with TPE had clinical manifestations of elevated ICP (mean follow-up 15.1 ± 11.7 years). Conclusion: Patients with ETPEs frequently had radiological signs of IIH while not developing typical manifestations of elevated ICP over time. In this way, ETPEs could be an unrecognized manifestation of IIH, and temporal lobe seizures the only clinical expression of this epilepsy syndrome. [ABSTRACT FROM AUTHOR]

Resting state functional connectivity research has shown that general cognitive ability (GCA) is associated with brain resilience to targeted and random attacks (TAs and RAs). However, it remains to be seen if the finding generalizes to structural connectivity. Furthermore, individuals showing performance levels at the very high area of the GCA distribution have not yet been analyzed in this regard. Here we study the relation between TAs and RAs to structural brain networks and GCA. Structural and diffusion-weighted MRI brain images were collected from 189 participants: 60 high cognitive ability (HCA) and 129 average cognitive ability (ACA) individuals. All participants completed a standardized fluid reasoning ability test and the results revealed an average HCA-ACA difference equivalent to 33 IQ points. Automated parcellation of cortical and subcortical nodes was combined with tractography to achieve an 82 × 82 connectivity matrix for each subject. Graph metrics were derived from the structural connectivity matrices. A simulation approach was used to evaluate the effects of recursively removing nodes according to their network centrality (TAs) versus eliminating nodes at random (RAs). HCA individuals showed greater network integrity at baseline and prior to network collapse than ACA individuals. These effects were more evident for TAs than RAs. The networks of HCA individuals were less degraded by the removal of nodes corresponding to more complex information processing stages of the PFIT network, and from removing nodes with larger empirically observed centrality values. Analyzed network features suggest quantitative instead of qualitative differences at different levels of the cognitive ability distribution. [ABSTRACT FROM AUTHOR]

Grapheme-color synesthesia is a neurological phenomenon in which viewing achromatic letters/numbers leads to automatic and involuntary color experiences. In this study, voxel-based morphometry analyses were performed on T1 images and fractional anisotropy measures to examine the whole brain in associator grapheme-color synesthetes. These analyses provide new evidence of variations in emotional areas (both at the cortical and subcortical levels), findings that help understand the emotional component as a relevant aspect of the synesthetic experience. Additionally, this study replicates previous findings in the left intraparietal sulcus and, for the first time, reports the existence of anatomical differences in subcortical gray nuclei of developmental grapheme-color synesthetes, providing a link between acquired and developmental synesthesia. This empirical evidence, which goes beyond modality-specific areas, could lead to a better understanding of grapheme-color synesthesia as well as of other modalities of the phenomenon. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains. Methods: Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions. Results: At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel's class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal-social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG. Conclusions: Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article about aceruloplasminemia and multiple motor system dysfunction of a 33 year-old woman by Juan Bernar in the 2007 issue.