Neurocognitive profile in patients with idiopathic generalized epilepsies: Differences between patients, their biological siblings, and healthy controls.

BACKGROUND: Idiopathic generalized epilepsy (IGE) is one of the most common epilepsies and is believed to have a strong genetic origin. Patients with IGE present largely heterogeneous neurocognitive profiles and might show some neurocognitive impairments. Furthermore, IGE siblings may demonstrate worse results in neuropsychological tests as well. In our study, we aimed to map the neurocognitive profile both in patients with IGE and the siblings. We also sought to establish a neurocognitive profile for each IGE syndrome. METHODS: The research sample included 110 subjects (IGE n = 46, biological siblings BS n = 16, and healthy controls n = 48) examined. Subjects were neuropsychologically examined in domains of intelligence, attention, memory, executive, and motor functions. The data obtained from the examination were statistically processed to determine whether and how IGE patients (including distinct syndromes) and the siblings differed neurocognitively from healthy controls (adjusted z-scores by age, education, and gender, and composite z-scores of cognitive domains). Data on anti-seizure medication, including defined daily doses, were obtained and included in the analysis. RESULTS: IGE patients and their biological siblings performed significantly worse in most of the neuropsychological tests than healthy controls. The neurocognitive profile of composite z-scores showed that IGE and biological siblings had equally significantly impaired performance in executive functions. IGE group also demonstrated impaired composite attention and motor function scores. The profile of individual IGE syndromes showed that JAE, JME, and EGTCS had significantly worse performance in composite execution score and motor function score. JAE presented significantly worse performance in intelligence and attention. JME exhibited significantly worse composite score in the attention domain. Anti-seizure medication, depression, and quality of life were unrelated to cognitive performance in IGE group. The level of depression significantly predicted the overall value of quality of life in patients with IGE, while cognitive domains, sociodemographic, and clinical factors were unrelated. CONCLUSION: Our study highlights the importance to consider the neurocognitive profile of IGE patients that can lead to difficulties in their education, acceptance, and management of coping strategies. Cognitive difficulties of IGE siblings could support a hypothesis that these impairments emerge from heritable traits.

How long shall we record electroencephalography?

BACKGROUND: The duration of electroencephalography (EEG) recordings varies widely among laboratories. Although several recommendations had been published, there are no previous studies directly addressing this. AIMS OF THE STUDY: To assess the effect of the recording duration on detection of EEG abnormalities in a tertiary referral centre for epilepsy. METHODS: We have reviewed 1005 EEG recordings and determined the shortest recording duration necessary to identify interictal EEG abnormalities. RESULTS: Standard, awake recordings shorter than 20 min yielded a significantly lower incidence of abnormal findings as compared to longer recordings. Although there was an increase in the diagnostic yield from 30 to 180 min recording duration, this failed to reach the level of significance. For sleep recordings, there was no significant increase in the diagnostic yield beyond 30 min. CONCLUSIONS: Our results provide evidence for recommending at least 20 min recording duration for standard awake EEGs and 30 min for sleep EEG recordings. As data were derived from patients referred to our epilepsy centre, the results are only valid for epilepsy-related indications.

[Treatment for volume upgrading of the low-grade supratentorial glioma after the subtotal neurosurgical resection]. / Lécba nízkostupnového gliomu supratentoriální oblasti mozku po neurochirurgické subtotální resekci s projevy objemového up-gradingu.

BACKGROUND: Low-grade gliomas WHO II (LGG) are mostly detected in patients with neurological symptomatology between 20 and 45 years of age very often as secondary epilepsy. We present two cases in which low-grade gliomas attacked neurological zones. Neurosurgical resection was subtotal because of the risk of the damage in neurocognitive functions in both these patients. After the operation, both patients were followed at neurosurgery department in regular intervals using different imaging methods (MRI, MRS and PET). After resections, the MRI detected the enlargement of the volumes of the tumor residua in both patients. PATIENTS AND METHODS: Owing to the risk of up-grading to high-grades glial tumors (overexpression of EGFR and VEGF), both patients were indicated for curative treatment by external beam radiotherapy combined with chemotherapy (Temodal®) and adjuvant chemotherapy. RESULTS: After the end of this treatment, the MRI proved considerable partial regressions in both patients. Moreover, three months later, the MRI did not prove any residual disease. CONCLUSION: Radiotherapy combined with the administration of Temodal should prolong the OS and TTP in patients with a high risk of up-grading of low-grade gliomas of the brain. Both the patients are in a follow-up program, also because of the risk of duplicite brain tumor.

Sulphates of 3beta-hydroxy-5-ene steroids in women with epilepsy.

Epilepsy is associated with various reproductive disorders and some antiepileptic drugs also influence the steroid metabolism. There is only limited data concerning the role of steroid sulphates in human epilepsy. Moreover, the substitution treatment with therapeutic substances also improves cognitive functions in humans. Therefore, we evaluated the balance between free and Delta5 sulphated steroids in women with epilepsy on various antiepileptic drugs. The study included 28 patients (17.0-51.0 years), with generalized (n=16) or catamenial epilepsy (n=12) followed in the follicular (FP) and luteal (LP) phases of menstrual cycle. Fifteen patients were on monotherapy and 13 were on polytherapy with 2 or 3 drugs. RIA was used for the steroid analyses. Statistical evaluation was done by Mann-Whitney tests and multivariate regression with reduction of dimensionality (Orthogonal Projections to Latent Structures, O2PLS). The final O2PLS model found a single significant predictive component extracting the variability shared between carbamazepine therapy, age of the subjects, and steroid levels and correlating with the variables as follows pregnenolone sulphate (PregS)-FP: R= -0.844, p<0.01; DHEAS-FP: R= -0.923, p<0.01; PregS-LP: R= -0.876, p<0.01; DHEAS-LP: R= -0.902, p<0.01; carbamazepine therapy: R=0.441, p<0.01; age of the participants (R=0.584, p<0.01). Carbamazepine significantly decreased DHEAS in both FP (p=0.02) and LP (p=0.003) and PregS in LP (p=0.03) and tended to decrease the PregS levels in FP (p=0.10), while primidone decreased DHEAS in both FP and LP (both p=0.05) and did not significantly change the levels of PregS. In conclusion, carbamazepine and primidone therapies significantly suppressed the sulphated steroids in serum.