The Association Between Maladaptive Metacognitive Beliefs and Emotional Distress in People Living With Amyotrophic Lateral Sclerosis.

OBJECTIVE: Approximately half of all people living with amyotrophic lateral sclerosis (ALS) experience persistent or recurrent emotional distress, yet little is known about the psychological processes that maintain emotional distress in this population. The self-regulatory executive functioning (S-REF) model specifies that maladaptive metacognitive beliefs and processes are central to the development and maintenance of emotional distress. This study explored whether maladaptive metacognitive beliefs are associated with emotional distress after controlling for demographic factors, time since diagnosis, and current level of physical functioning. DESIGN: In a cross-sectional design, 75 adults with a diagnosis of ALS completed self-report questionnaires. Participants had a mean age of 60.40 years, mean duration of symptoms 63.92 months, and male:female gender ratio of 14:11. MAIN OUTCOME MEASURES: Questionnaires assessed emotional distress (HADS, adapted for ALS), physical functioning (ALSFRS-R), repetitive negative thinking (RTQ-10), metacognitive beliefs (MCQ-30), and demographic factors. RESULTS: Maladaptive metacognitive beliefs explained additional variance in emotional distress after controlling for age, gender, time since diagnosis, physical functioning, and repetitive negative thinking. Repetitive negative thinking partially mediated the relationships between positive and negative metacognitive beliefs and emotional distress. CONCLUSIONS: These data support the utility of the metacognitive model in understanding emotional distress in people with ALS. Examination of the temporal relationship between maladaptive metacognitive beliefs and emotional distress in people living with ALS may help to guide the development of therapeutic approaches.

Visual Hallucinations in Eye Disease and Lewy Body Disease.

OBJECTIVES: Visual hallucinations (VH) most commonly occur in eye disease (ED), Parkinson disease (PD), and Lewy body dementia (LBD). The phenomenology of VH is likely to carry important information about the brain areas within the visual system generating them. METHODS: Data from five controlled cross-sectional VH studies (164 controls, 135 ED, 156 PD, 79 [PDD 48 + DLB 31] LBD) were combined and analyzed. The prevalence, phenomenology, frequency, duration, and contents of VH were compared across diseases and sex. RESULTS: Simple VH were most common in ED patients (ED 65% versus LBD 22% versus PD 9%, χ(2) = 31.43, df = 2, p < 0.001), whereas complex VH were more common in LBD (LBD 76% versus ED 38%, versus PD 28%, χ(2) = 96.80, df = 2, p < 0.001). The phenomenology of complex VH was different across diseases and sex. ED patients reported more "flowers" (ED 21% versus LBD 6% versus PD 0%, χ(2) = 10.04, df = 2, p = 0.005) and "body parts" (ED 40% versus LBD 17% versus PD 13%, χ(2) = 11.14, df = 2, p = 0.004); in contrast, LBD patients reported "people" (LBD 85% versus ED 67% versus PD 63%, χ(2) = 6.20, df = 2, p = 0.045) and "animals/insects" (LBD 50% versus PD 42% versus ED 21%, χ(2) = 9.76, df = 2, p = 0.008). Men reported more "machines" (13% versus 2%, χ(2) = 6.94, df = 1, p = 0.008), whereas women reported more "family members/children" (48% versus 29%, χ(2) = 5.10, df = 1, p = 0.024). CONCLUSIONS: The phenomenology of VH is likely related to disease-specific dysfunctions within the visual system and to past, personal experiences.

Pharmacological interventions for epilepsy in people with intellectual disabilities.

BACKGROUND: The prevalence of epilepsy among people with intellectual disabilities is much higher than in the general population. Seizures in this population are often complex and refractory to treatment and antiepileptic medication may have a profound effect upon behaviour (Kerr 1997).This is an updated version of a Cochrane Review first published in Issue 3, 2007. OBJECTIVES: To assess the data available from randomised controlled trials (RCTs) of the efficacy of antiepileptic drug (AED) interventions in people with epilepsy and intellectual disabilities. SEARCH METHODS: For the latest update of this review, we searched the Cochrane Epilepsy Group Specialised Register (2 September 2014), the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online (CRSO) (2 September 2014), MEDLINE (Ovid, 1946 to 3 September 2014) and PsycINFO (EBSCOhost, 1887 to 3 September 2014). SELECTION CRITERIA: Randomised and quasi-randomised controlled trials (RCTs) of pharmacological interventions for people with epilepsy and a learning disability. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed trial quality and extracted data. We contacted study authors for additional information. We assessed epilepsy/seizure, behavioural and cognitive outcomes, as well as quality of life and adverse effects. MAIN RESULTS: We included 14 RCTs (1116 participants) in the present review. Data were heterogenous and a descriptive analysis is presented. In the majority of cases where antiepileptic drugs (AEDs) were trialled in this population, we found moderate reductions in seizure frequency in that there was a significantly higher rate of responders (reduction of 50% or more) in the treatment group compared with the placebo group, with some studies reporting a higher incidence of seizure freedom in the treatment group. In general, AEDs that are proven to be effective in the general epilepsy population are also effective for refractory epilepsy in people with intellectual disability. It is not possible to comment on the relative efficacy of medications, making clinical decisions difficult.In trial settings patients continued on treatment in the majority of cases. Placebo groups often experienced fewer adverse events. Where adverse events were experienced they appeared similar to those in the general population. The methods by which adverse events were recorded and reported appeared to be inconsistent, resulting in very large variation between studies. This is problematic as clinically relevant interpretation of these findings is limited.The quality of evidence provided in the present review is low to moderate. Additionally the majority of studies lacked or used non-reliable measures of behavioural exacerbation. However, where measured, little obvious impact on behaviour was seen in terms of behaviour disorder. AUTHORS' CONCLUSIONS: This review broadly supports the use of AEDs to reduce seizure frequency in people with refractory epilepsy and intellectual disability. The evidence suggests that adverse events are similar to those in the general population and that behavioural adverse events leading to discontinuation are rare; however, other adverse effects are under-researched.

Non-pharmacological interventions for people with epilepsy and intellectual disabilities.

BACKGROUND: Approximately 30% of patients with epilepsy remain refractory to drug treatment and continue to experience seizures whilst taking one or more antiepileptic drugs (AEDs). Several non-pharmacological interventions that may be used in conjunction with or as an alternative to AEDs are available for refractory patients. In view of the fact that seizures in people with intellectual disabilities are often complex and refractory to pharmacological interventions, it is evident that good quality randomised controlled trials (RCTs) are needed to assess the efficacy of alternatives or adjuncts to pharmacological interventions.This is an updated version of the original Cochrane review (Beavis 2007) published in The Cochrane Library (2007, Issue 4). OBJECTIVES: To assess data derived from randomised controlled trials of non-pharmacological interventions for people with epilepsy and intellectual disabilities.Non-pharmacological interventions include, but are not limited to, the following.• Surgical procedures.• Specialised diets, for example, the ketogenic diet, or vitamin and folic acid supplementation.• Psychological interventions for patients or for patients and carers/parents, for example, cognitive-behavioural therapy (CBT), electroencephalographic (EEG) biofeedback and educational intervention.• Yoga.• Acupuncture.• Relaxation therapy (e.g. music therapy). SEARCH METHODS: For the latest update of this review, we searched the Cochrane Epilepsy Group Specialised Register (19 August 2014), the Cochrane Central Register of Controlled Trials (CENTRAL) via CRSO (19 August 2014), MEDLINE (Ovid, 1946 to 19 August 2014) and PsycINFO (EBSCOhost, 1887 to 19 August 2014). SELECTION CRITERIA: Randomised controlled trials of non-pharmacological interventions for people with epilepsy and intellectual disabilities. DATA COLLECTION AND ANALYSIS: Two review authors independently applied the inclusion criteria and extracted study data. MAIN RESULTS: One study is included in this review. When two surgical procedures were compared, results indicated that corpus callosotomy with anterior temporal lobectomy was more effective than anterior temporal lobectomy alone in improving quality of life and performance on IQ tests among people with epilepsy and intellectual disabilities. No evidence was found to support superior benefit in seizure control for either intervention. This is the only study of its kind and was rated as having an overall unclear risk of bias. The previous update (December 2010) identified one RCT in progress. The study authors have confirmed that they are aiming to publish by the end of 2015; therefore this study (Bjurulf 2008) has not been included in the current review. AUTHORS' CONCLUSIONS: This review highlights the need for well-designed randomised controlled trials conducted to assess the effects of non-pharmacological interventions on seizure and behavioural outcomes in people with intellectual disabilities and epilepsy.

Neuropsychological and psychological interventions for people with newly diagnosed epilepsy.

BACKGROUND: Many people with epilepsy report experiencing psychological difficulties such as anxiety, depression and neuropsychological deficits including memory problems. Research has shown that these difficulties are often present not only for people with chronic epilepsy but also for people with newly diagnosed epilepsy. Despite this, there are very few published interventions that detail means to help people with newly diagnosed epilepsy manage these problems. OBJECTIVES: To identify and assess possible psychological and neuropsychological interventions for adults with newly diagnosed epilepsy. SEARCH METHODS: We searched the following databases on 30 June 2015: the Cochrane Epilepsy Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (Ovid), SCOPUS, PsycINFO, CINAHL, ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). SELECTION CRITERIA: This review includes all randomised controlled trials, quasi-randomised controlled trials, prospective cohort controlled studies, and prospective before and after studies which include psychological or neuropsychological interventions for people with newly diagnosed epilepsy. We excluded studies that included people with epilepsy and any other psychological disorder or neurological condition. We excluded studies carried out which recruited only children. DATA COLLECTION AND ANALYSIS: We used the standard methodological procedure expected by The Cochrane Collaboration. Two authors independently completed data extraction and risk of bias analysis. The results of this were cross-checked and third author resolved any discrepancies. In the event of missing data, we contacted the study authors. Meta-analysis was not completed due to differences in the intervention and outcomes reported in the two studies. MAIN RESULTS: We included two randomised controlled trials assessing psychological interventions for people with newly diagnosed epilepsy. One study assessed a cognitive behavioural intervention (CBI) in an adolescent population. This study was rated as low quality. One study assessed a specialist nurse intervention in an adult population. This study was rating as very low quality.We rated one study as having unclear risk of bias and one study as having high risk of bias.The CBI study indicated that this intervention could significantly reduce depressive symptoms in people with subthreshold depressive disorder. However, the study assessing the effectiveness of a nurse intervention found no significant benefit for depressive symptoms,but did find that in individuals with the least knowledge of epilepsy, a nurse intervention could increase their knowledge of epilepsy scores. AUTHORS' CONCLUSIONS: Meta-analysis was not possible as we identified only two studies and they utilised different interventions and outcome measures.Previous research has highlighted the impact of psychological and neuropsychological difficulties experienced by people with epilepsy and the negative effect this has on their quality of life. The main finding of this review is that there is a paucity of research assessing possible neuropsychological and psychological interventions for adults with newly diagnosed epilepsy.