Long-term quality of life after ETV or ETV with consecutive VP shunt placement in hydrocephalic pediatric patients.

PURPOSE: Endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunting are well-established treatments of obstructive hydrocephalus (HCP) in adult and pediatric patients. However, there is a lack of data with regard to the quality of life (QoL) of these patients during long-term follow-up METHODS: Inclusion criteria were pediatric patients with endoscopic treatment of hydrocephalus at the University Medicine Greifswald between 1993 and 2016. Patients older than 14 years at present were assessed with the Short Form-12 (SF-12) questionnaire. Patients younger than 14 years of age were assessed with the KINDL-R questionnaire that was completed by their parents. Patients' values were compared with the scores of a corresponding age-matched group of the healthy population and with patients who received later shunt treatment. Information about comorbidities, current symptoms, and educational level were gained by an additional part. Comparative analysis between patients with ETV success and failure (defined as shunt implantation after ETV) was performed. RESULTS: A total of 107 patients (53 m, 54f) were included. Fifty-seven/107 patients (53.3%) were considered as ETV success. Mean age at ETV was 6.9 ± 5.9 years. Fifty-four statements of 89 patients that are still alive were gained (response rate 63%). Of these, 49 questionnaires were complete and evaluable (23 m, 26f; mean age 19.8 ± 10.0 years with an average follow-up period of 13.7 ± 7.2 years). Twenty-six/49 patients (53.1%) are considered ETV success. No statistically significant differences could be obtained between patients with ETV success and ETV failure. Patients older 14 years show QoL within normal range, patients younger than 14 years show significantly lower result regarding their environment of peers and social contacts. Patients younger than 6 months at the time of ETV and patients with posthemorrhagic HCP show significantly lower physical QoL. Gait disturbance, fatigue, and seizures are associated with a lower QoL, and educational level is lower than in the normal population. CONCLUSIONS: Patients who underwent ETV in childhood do not have a lower health-related QoL in general. Subsequent insertions of ventriculoperitoneal (vp) shunts do not lower QoL. Certain subgroups of the patients show lower results compared to the healthy population.

Testing social cognition in multiple sclerosis: Difference between emotion recognition and theory of mind and its influence on quality of life.

BACKGROUND: Deficits in social cognition can occur in multiple sclerosis (MS) patients, and different methods are utilized for its assessment. The aim of this study was to compare two tests of social cognition in a cohort of multiple sclerosis patients with respect to other clinical variables. Additionally, the impact of social cognition on quality of life was investigated. METHODS: In total, 50 patients were included in the study. Two tests of social cognition, emotion recognition and theory of mind, were performed and controlled for disease disability, depression, fatigue, and cognition in a multiple linear regression. Assessment of quality of life was also conducted. RESULTS: Accuracy on emotion recognition was better compared to theory of mind (86.5 ± 9.5% and 63.6 ± 10.1%, respectively). Cognition was associated with both social cognition tasks, accounting for more variance in the emotion recognition task. Quality of life was not related to social cognition. CONCLUSION: Studies on social cognition in MS have to keep in mind the higher degree of cognitive influence of emotion recognition compared to theory of mind.

Clinical predictors of the long-term social outcome and quality of life in juvenile myoclonic epilepsy: 20-65 years of follow-up.

OBJECTIVE: The long-term social outcome in patients with juvenile myoclonic epilepsy (JME) is still controversial. The aim of this study was both to investigate the long-term social outcome in relation to clinical variables and to identify epilepsy-related factors that affect the quality of life (QoL) in JME patients with a follow-up of at least 20 years. METHODS: A retrospective selection of 33 of 90 patients (21 female) from a tertiary epilepsy center diagnosed with JME and followed for ≥20 years (mean 37.8 years) was studied. All patients were evaluated with a thorough review of their medical records, and a subsequent face-to-face or telephone interview. QOLIE-31-P questionnaire (QoL In Epilepsy) and Beck Depression Inventory-II were used to assess the QoL and the presence and severity of depressive symptoms, respectively. RESULTS: Of 33 patients, 18 (54.5%) became seizure-free; in 4 of the patients (22.2%), antiepileptic drug (AED) treatment was discontinued. Early and long-term seizure freedom improves both social adjustment (p = 0.02) and occupational integration (p = 0.02) and associates with a better QoL (odds ratio [OR] 2.25). A high seizure burden highly affects both aspects of personal life-family and work; notably the occurrence of frequent and/or late onset generalized tonic-clonic seizures increases the risk of concomitant diseases (p = 0.05) and lifelong AED treatment (p = 0.03), decreases the patient's employability (p = 0.02), increases the rate of employment disability pension (p = 0.05), and considerably increases public/social spending. Seizure freedom significantly increases the QoL (p = 0.001), whereas more severe courses of epilepsy (OR 3.2), AED side effects (p = 0.04), depression (p = 0.02), and sleep disturbances (OR 2.7) considerably decrease the patient's QoL. SIGNIFICANCE: Although patients with JME are a heterogeneous group, several predictors for the long-term social, family, educational, and occupational outcome have been identified in our study and should be considered in the effort to both improve the patient's QoL as well as preserve economic resources.

Impaired recognition of emotional facial expressions in patients with multiple sclerosis.

BACKGROUND: Disturbances that occur in patients with multiple sclerosis (MS) are not restricted to motor, sensory, or urinary functions; they also include cognitive dysfunction, fatigue, and depression. Moreover, people with MS are known to have fewer social activities and a reduced quality of life. One aspect of social interaction is accurate recognition of facial expressions. Several studies have suggested impairment in the processing of facial expressions in patients with multiple sclerosis, but it is not clear if these deficits are based on cognitive, depressive, or other attendant symptoms. OBJECTIVE: To investigate emotion recognition and facial identity recognition abilities and their relation with cognitive functions, depression, and fatigue in a cohort of MS patients. METHODS: Emotion recognition and facial identity recognition abilities were investigated in a cohort of 61 MS patients with unimpaired visual acuity and 53 healthy controls using the Florida Affect Battery. Additionally, we investigated possible relationships between impaired facial expression recognition and other clinical features. RESULTS: MS patients were not impaired in facial identity discrimination, but showed a poor performance in all subtests that required emotion recognition. CONCLUSION: Impaired recognition of facial emotions by patients with MS seems to be associated with both cognitive and affective (depression) aspects of the disease.

Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25-63 years of follow-up.

PURPOSE: The long-term seizure outcome of juvenile myoclonic epilepsy (JME) is still controversial; the value of factors that are potentially predictive for seizure outcome remains unclear. The aim of this study was both to investigate the long-term seizure outcome in patients with JME after a follow-up of at least 25 years and to identify factors that are predictive for the seizure outcome. METHODS: Data from 31 patients (19 women) with JME were studied. All of them had a follow-up of at least 25 years (mean 39.1 years) and were reevaluated with a review of their medical records and direct telephone or face-to-face interview. KEY FINDINGS: Of 31 patients 21 (67.7%) became seizure-free; in six of them (28.6%) antiepileptic drug (AED) treatment was discontinued due to seizure freedom. The occurrence of generalized tonic-clonic seizures (GTCS) preceded by bilateral myoclonic seizures (BMS) (p = 0.03), a long duration of epilepsy with unsuccessful treatment (p = 0.022), and AED polytherapy (p = 0.023) were identified as significant predictors for a poor long-term seizure outcome, whereas complete remission of GTCS under AED significantly increased the chance for complete seizure freedom (p = 0.012). The occurrence of photoparoxysmal responses significantly increases the risk of seizure recurrence after AED discontinuation (p = 0.05). SIGNIFICANCE: This study shows conclusively that JME is a heterogeneous epilepsy syndrome. Life-long AED treatment is not necessarily required to maintain seizure freedom. Several long-term outcome predictors that can potentially increase the ability of clinicians and their confidence to recommend different treatment options to patients with JME were identified.

Prefrontal function associated with impaired emotion recognition in patients with multiple sclerosis.

Multiple sclerosis (MS) is associated with the occurrence of white matter plaques in the central nervous system. These are frequently located in areas interconnecting areas associated with the processing of emotions. Although recent behavioral studies indicated social and affective disturbances in many of these patients, functional studies investigating specific emotional recognition in MS are lacking. We used functional magnetic resonance imaging (fMRI) and lesion mapping in MS-patients to investigate correlates between these measures and emotional facial recognition. Eleven patients whose affective ability was impaired were compared with eleven unimpaired MS-patients and eleven healthy controls (HCs) using a facial expression matching task. Decreased recognition performance was limited to the detection of unpleasant facial expressions (sad, fearful, angry). In evaluating the functional activation maps for the unpleasant facial expressions, we found decreased insular and ventrolateral prefrontal cortex (VLPFC) activation in the impaired group versus the unimpaired groups. We found a close relationship between the inability of solving the task and decreased activation of the left VLPFC and the left anterior insula. In addition, we found a correlation between decreased performance accuracy and the presence of lesions in the left temporal white matter. These data suggest that emotion recognition deficits in MS-patients might be due to the interruption of processing emotionally relevant information, which leads to decreased activation of the VLPFC and the insula.