Impact of epilepsy on language and discourse: Two self-limited focal epileptic syndromes of childhood.

Self-limited focal epilepsy with centro-temporal spikes, also known as Rolandic epilepsy (RE), is a well-established focal epilepsy of childhood, characterized with language impairment. To investigate the relationship between language deficits and clinical parameters of self-limited focal epilepsies of childhood (SFEC), 21 patients with RE, 10 patients with childhood occipital epilepsy of Gastaut type (COE-G) (another SFEC that is not typically associated with language impairment), and 31 healthy controls were recruited. A broad panel of language tests also including narration sample was administered, and clinical features were documented. The language was significantly impaired in both RE and COE-G. Patients with COE-G showed worse scores than patients with RE in subtests measuring semantic functions. Clinical parameters were not associated with impaired language domains. Language impairment is experienced in different types of SFEC, emphasizing the broad representation of the language network. In SFEC, recent activity of epilepsy does not affect the severity of language dysfunction.

Effect of post-transplant diabetes mellitus on cardiovascular events and mortality: a single-center retrospective cohort study.

PURPOSE: This study aims to investigate the impact of post-transplant diabetes mellitus (PTDM) on cardiovascular events, graft survival, and mortality and to determine the risk factors involved in developing PTDM. METHODS: A total of 703 patients who underwent kidney transplantation were included in the study. The total sample was subdivided into three groups: (i) patients with PTDM; (ii) patients who had diabetes before the transplantation (DM); and (iii) patients without diabetes (NoDM). The data on graft failure, cardiovascular events, all-cause mortality, and the potential risk factors that play a role in developing PTDM were recorded and analyzed. RESULTS: The patients were followed for a median of 80 (6-300) months after transplantation. Out of all patients, 41 (5.8%) had DM before transplantation, and 101 (14.4%) developed PTDM. Recipient BMI, post-transplant fasting plasma glucose, and hepatitis C seropositivity were independent risk factors for PTDM development. The incidence of cardiovascular events was 6.1% in the NoDM group, 14.9% in the PTDM group, and 29.3% in the DM group (p < 0.001). In PTDM patients, hepatitis C seropositivity and the recipient's age at transplant were independent predictors of a cardiovascular event. There were no significant differences between the groups regarding the risk of graft loss. PTDM had no significant effect on all-cause mortality. However, the survival rates of DM patients were significantly reduced compared to those with NoDM or PTDM. CONCLUSIONS: PTDM had no impact on patient survival. Hepatitis C seropositivity and recipient age at transplant predicted cardiovascular events in PTDM patients.

Picnic, Accident or Cookies? A Systematic Approach to Guide the Selection of the Picture Definition Tasks in Linguistic Assessment.

OBJECTIVE: Picture description tasks are used to elicit language samples in individuals with aphasia and other cognitive disorders. However, task selection may affect the type of language variables elicited. In this study, our goal is to compare the strengths and the weaknesses of the two internationally used picture description tasks with a novel picture description task developed specifically for Turkish-speaking individuals. MATERIALS AND METHODS: In sum, 20 cognitively healthy individuals with a mean age of 52 ± 14/3 were included. Three different picture description tasks composed of single pictures were used: The Picnic Scene from the Western Aphasia Battery-Revised, the Cookie Theft picture from the Boston Diagnostic Aphasia Examination and the Accident Scene from the Turkish Language Assessment Test for Aphasia. All language samples were recorded using a digital voice recorder. Automated and semi-automated approaches were used for the systematic analysis of the language features that were classified into four levels: general language measures, the morphosyntactic level, the lexicosemantic level and the sentence level. RESULTS: Participants showed greater efficiency and produced the greatest number of subordinate clauses, derivational suffixes, passive voice suffixes and relative past tense construction during the Accident Scene description. On the lexicosemantic level, the Picnic Scene was particularly superior to the Cookie Theft Picture in eliciting nouns. Imperfective aspect use was less frequent in the Accident Scene description. CONCLUSION: All three tasks had limitations and advantages compared with each other. We recommend the development of novel picture description tasks that would be more effective in eliciting specific language features in Turkish-speaking individuals.

Developmental and Behavioral Consequences of Intrauterine Anti-Seizure Medication Exposure.

Introduction: In this cross-sectional study, whether there is a difference in the prevalence of developmental/behavioral problems in children of those who received mono/polytherapy during pregnancy; How Valproic Acid (VPA) exposure affects developmental/behavioral characteristics compared to other antiseizure medications (ASM) was also investigated. Method: 64 children of 46 women with epilepsy (WWE) with children aged 0-18 years were included. Ankara Development and Screening Inventory (ADSI) for their children up to the age of six and The Child Behavior Checklist for Ages 4-18-CBCL/4-18 scale was applied for the ages of 6-18. Children exposed to prenatal ASM were divided into two groups as polytherapy and monotherapy. Children exposed to monotherapy were investigated by drug exposure, as well as exposure to VPA and other ASMs. Chi-square test was used to compare qualitative variables. Results: When monotherapy and polytherapy groups were compared, a significant difference was found in the language cognitive development area of the ADSI (p=0.015) and in terms of the sports activity variable in CBCL/4-18 (p=0.039). When the VPA monotherapy and other ASM monotherapy groups were compared, a significant difference was found in terms of sports activity in CBCL-4-18 (p=0.013). Conclusion: It was found that language and cognitive development can be delayed, the level of engagement in sports activities can be reduced in children exposed to polytherapy. The rate of doing sports activities in valproic acid monotherapy exposure may decrease.

Does Electrical Status Epilepticus in Sleep Adversely Affect Language in Self-Limiting Focal Epilepsies of Childhood?

Introduction: The electrical status epilepticus in sleep (ESES) accompanies a wide spectrum of focal and generalized epilepsies, which manifest with cognitive-linguistic regression. Both ESES and language impairment can be seen in self-limited focal epileptic syndromes of childhood (SFEC). The association between the presence of ESES pattern on the EEG and the severity of the language impairment has not been adequately clarified. Methods: Twenty-eight SFEC cases without intellectual and motor disabilities and 32 healthy children were recruited. Cases with active ESES (A-ESES, n=6) and without ESES pattern on EEG (non-ESES, n=22) were compared in terms of clinical features and linguistic parameters by both standard and descriptive assessment tools. Results: The only significantly different clinical feature in the A-ESES group was the increased prevalence of polytherapy. While most of the linguistic parameters were impaired in A-ESES and non-ESES groups compared to healthy controls, A-ESES patients differed from non-ESES patients only in terms of decreased complex sentence production, which was assessed by narrative analysis. A-ESES patients also showed trends toward producing lower numbers of words, nouns, verbs, and adverbs during narrative analysis. There were no differences among patients under polytherapy and monotherapy in terms of these language parameters. Conclusion: Our results show that ESES increases the negative effect of chronic epilepsy on complex sentence and word production. Linguistic distortions that are not reflected in objective tests can be detected by narrative tools. Complex syntactic production obtained by narrative analysis is an important parameter that extensively characterizes language skills in school-age children with epilepsy.

The Effect of Interleukin-1 Antagonists on Brain Volume and Cognitive Function in Two Patients With Megalencephalic Leukoencephalopathy With Subcortical Cysts.

BACKGROUND: Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare leukodystrophy characterized by early-onset macrocephaly and progressive white matter vacuolation. The MLC1 protein plays a role in astrocyte activation during neuroinflammation and regulates volume decrease following astrocyte osmotic swelling. Loss of MLC1 function activates interleukin (IL)-1ß-induced inflammatory signals. Theoretically, IL-1 antagonists (such as anakinra and canakinumab) can slow the progression of MLC. Herein, we present two boys from different families who had MLC due to biallelic MLC1 gene mutations and were treated with the anti-IL-1 drug anakinra. METHODS: Two boys from different families presented with megalencephaly and psychomotor retardation. Brain magnetic resonance imaging findings in both patients were compatible with the diagnosis of MLC. The diagnosis of MLC was confirmed via Sanger analysis of the MLC1 gene. Anakinra was administered to both patients. Volumetric brain studies and psychometric evaluations were performed before and after anakinra treatment. RESULTS: After anakinra therapy, brain volume in both patients decreased significantly and cognitive functions and social interactions improved. No adverse effects were observed during anakinra therapy. CONCLUSIONS: Anakinra or other IL-1 antagonists can be used to suppress disease activity in patients with MLC; however, the present findings need to be confirmed via additional research.

Permanent Impairment of Language Functions in an Adolescent Case of Autoimmune Encephalitis.

Introduction: In pediatric and adolescent population, autoimmune encephalitis (AE) may present with a wide variety of symptoms including cognitive regression accompanied with loss of language skills. Despite its high prevalence in AE, linguistic functions have not been investigated in extensive detail. Case: A 12-year-old girl with no significant premorbid history and normal school performance presented with fever, hypersomnia, nocturnal myoclonus and behavioral changes. Although neurological examination was normal, psychiatric evaluation revealed euphoria, mild irritability and visual hallucinations. Cranial MRI was normal, whereas cerebrospinal fluid (CSF) analysis showed elevated protein concentration and lymphocyte count, electroencephalogram (EEG) showed diffuse slow waves. A panel for anti-neuronal antibodies demonstrated glutamic acid decarboxylase (GAD) antibodies in the serum. Following immunotherapy, all neurological and behavioral symptoms vanished. However, the patient suffered from significant worsening of school performance. Psychiatric evaluation revealed severe depression. Assessment of intelligence done on the 10th and 18th month of follow-up yielded significantly low scores at mental retardation level. Linguistic assessment showed significant impairment in all domains but especially in semantics. Conclusion: Our case emphasizes the fact that AE may cause permanent cognitive dysfunction and language impairment even in patients with normal MRI/neurological examination findings and relatively mild treatment-responsive disease course.

Oxysterol species generated by auto-oxidation in subclinical hypothyroidism.

BACKGROUND: Auto-oxidized oxysterols are implicated in the pathogenesis of various chronic diseases. Their concentrations are indicators of oxidative stress in vivo and associated with atherosclerosis. Subclinical hypothyroidism is related with cardiac diseases and oxidative stress, but the exact mechanisms underlying these associations are not clear yet. OBJECTIVE: To investigate the auto-oxidized oxysterols, 7-ketocholesterol (7-KC) and cholestane-3ß,5α,6ß-triol (chol-triol), in patients with subclinical hypothyroidism, as well as to evaluate the impact of restoring euthyroidism on oxysterol concentrations. METHODS: In this prospective observational study, 64 patients with newly diagnosed autoimmune thyroiditis (41 with subclinical hypothyroidism and 23 euthyroidism), and 45 healthy controls were enrolled. Age, gender, and body mass index were matched among patient groups and healthy controls. Anthropometric measurements were obtained and fasting plasma 7-ketocholesterol and cholestane-3ß,5α,6ß-triol concentrations were measured by using liquid chromatography coupled with tandem mass spectrometry. Levothyroxine was then administered to all patients with subclinical-hypothyroidism. After three months, measurements of the oxysterols and serum cholesterols from the patients who have become euthyroid were repeated. RESULTS: Concentrations of 7-ketocholesterol and cholestane-3ß,5α,6ß-triol were significantly higher in patients with subclinical-hypothyroidism when compared to both euthyroid patients and healthy controls (p < 0.001 for both oxysterols). After restoration of euthyroidism, concentrations of 7-ketocholesterol and cholestane-3ß,5α,6ß-triol decreased significantly and reached similar concentrations observed in healthy controls (p < 0.001 for both oxysterols). CONCLUSIONS: Auto-oxidized oxysterol species are higher in patients with mild thyroid dysfunction, and supported the rationale for treating subclinical-hypothyroidism.

Glial fibrillary acidic protein (GFAP)-antibody in children with focal seizures of undetermined cause.

Anti-neuronal antibodies that are related with autoimmune encephalitis syndromes may also be found in children with new onset seizures or chronic epilepsy. To unravel the significance of autoimmune astrocytopathy in epilepsy, we investigated serum antibody to glial fibrillary acidic protein (GFAP), another autoantigen described in autoimmune encephalitis with seizures, in 38 children with focal seizures of undetermined cause. GFAP antibody was screened with cell based assay and indirect immunohistochemistry and was found in two boys with normal brain MRI and unrevealing medical history prior to seizures. The 2-year-old boy had chronic treatment-resistant frontal lobe epilepsy. The 2.5-year-old boy had a single episode of focal seizures and remained seizure free thereafter in a follow-up period of 4 years. Nevertheless, he showed severe cognitive and language impairment. These results suggest that autoimmune astrocytopathy may be present in some epilepsy patients. Whether this immune response is a bystander effect generated by seizure-induced astrocytosis or directly involved in epileptogenesis needs to be further studied.