Kleine–Levin syndrome: A neuropsychiatric disorder

Kleine–Levin syndrome (KLS) is a rare, relapsing-remitting disease that affects mostly adolescents. It is characterized by episodes lasting from 1 to several weeks, and comprises neurological (hypersomnia, confusion, slowness, amnesia) and neuropsychiatric symptoms (derealization and apathy). Some psychiatric symptoms (megaphagia, hypersexuality, anxiety, depressed mood, hallucinations, delusions) arise during episodes, albeit less frequently, while patients are normal between episodes. However, sudden severe (>18 h/day of sleep) and recurrent hypersomnia helps to differentiate KLS from other psychiatric mimics. Derealization, the striking feeling of unreality or of being in a dream-like environment, is strongly associated with hypoperfusion of the associative temporoparietal junction cortex, whereas apathy is almost complete loss of autoactivation: teenagers stop using their cell phones and their only spontaneous initiative is to sleep. The cause of KLS is not known, but evidence suggests it could be a recurrent inflammatory encephalitis. Up to 5% of cases are familial, although no abnormal gene has yet been found. Hypersomnia episodes tend to become less frequent and to disappear with advancing age. However, 28% of patients have long-lasting episodes (>30 days), and around 15% have no signs of recovery after >20 years of living with the disorder. Patients’ cognitive and psychiatric status should be regularly checked during asymptomatic periods, as 20–40% develop long-term mild cognitive impairment or mood disorders. Lithium therapy is beneficial for reducing episode frequency, and intravenous steroids can reduce the duration of long episodes.     

Subjective perceived outcome of subthalamic deep brain stimulation in Parkinson's disease one year after surgery

ObjectivesDissatisfaction with subthalamic deep brain stimulation (STN-DBS) despite motor improvements has been observed in Parkinson's disease (PD). Hence, we compared patient's subjective perceived outcome 12 months after surgery (12mFU) with clinical measures to identify risk factors of dissatisfaction.MethodsPatients were examined at baseline and 12mFU. Quality of life (QoL), neuropsychiatric, cognitive and neurological functioning was measured. Patients were classified concerning their subjective outcome (negative = dissatisfaction; mixed; positive = satisfaction) at 12mFU using semi-structured interviews. First, the three groups were compared concerning interview statements. Second, repeated measures ANOVAs with group as between-subjects factor were applied to find significant effects of time, group, or interaction. Third, binary logistic regression determined predictors of dissatisfaction.ResultsOf the 28 enrolled patients, 25% perceived their outcome as negative, 32.1% as mixed, and 42.9% as positive. Concerning interview statements, dissatisfied patients mentioned significantly less often improved QoL and reduced medication, and reported worsening of mental state, and social interaction. For the whole sample, significant improvement over time was found for motor functioning, daily dopamine dosages, and QoL. Apathy significantly worsened over time, but dissatisfied patients were overall more apathetic and depressed than the other groups. Significant interaction of group and time was identified for QoL, which only improved in the mixed and satisfied group. Finally, preoperative apathy and axial symptoms predicted dissatisfaction with STN-DBS.ConclusionsAlthough motor symptoms and QoL improved in the whole sample, 25% of patients showed disappointment with STN-DBS. Especially apathy predicts dissatisfaction and should be considered preoperatively.     

Apathy and Health-Related Quality of Life in Stroke

Objective

To examine differences in health-related quality of life (HRQOL) in stroke survivors with and without apathy.

Design

Cross-sectional study.

Setting

Acute stroke unit in a regional hospital.

Participants

Stroke survivors (N=391) recruited from the acute stroke unit.

Interventions

Not applicable.

Main Outcome Measures

Participants were divided into apathy and nonapathy groups. Participants who scored ≥36 on the Apathy Evaluation Scale, clinician's version formed the apathy group. HRQOL was measured with the 2 component scores, mental component summary (MCS) and physical component summary (PCS), of the Medical Outcomes Study 12-Item Short-Form Health Survey (SF-12). Demographic and clinical information were obtained with the National Institutes of Health Stroke Scale (NIHSS), Barthel Index (BI), Mini-Mental State Examination (MMSE), and Geriatric Depression Scale (GDS).

Results

Thirty-six (9%) participants had apathy. The apathy group had significantly lower MCS and PCS scores. After adjusting for sex, education, diabetes mellitus, and NIHSS, MMSE, GDS, and BI scores, the MCS score in the apathy group remained significantly lower.

Conclusions

Apathy has a significant negative effect on HRQOL in stroke survivors, particularly on their mental health. Interventions for apathy could improve the HRQOL of stroke survivors.     

Apathy after acquired brain impairment: A systematic review of non-pharmacological interventions

Apathy commonly occurs after acquired brain impairment. It is characterised by impaired initiative, diminished activity, and lack of concern; formally delineated as a decrease in cognitive, behavioural and emotional components of goal-directed activity. The impact is widespread, hampering rehabilitation and outcome. This systematic review identifies and assesses the efficacy of non-pharmacological treatments for apathy following four types of acquired brain impairment (traumatic brain injury, dementia, cerebrovascular accident, encephalitis). Nine databases were searched. Studies were reviewed according to the following criteria: age over 16 years, acquired brain impairment, non-pharmacological intervention for apathy, and data reported on treatment efficacy. The methodological quality of the studies was assessed. Searches yielded 1754 articles, with 28 meeting criteria. Methodological quality ranged greatly. The majority of trials involved the dementia population. Cognitive interventions were the most frequent mode of treatment. For those with severe impairments, the strongest evidence suggested music therapy and for milder impairment, the strongest evidence was for cognitive rehabilitation. This review reveals a need for more high quality, methodologically rigorous treatment studies for apathy, particularly within the milder ranges of impairment. Initially, however, a uniform operational definition needs to be utilised in all research studies to minimise variability. Additionally, employing a standardised outcome measure specific to apathy would greatly enhance comparison among treatments.     

Incentive motivation deficits in schizophrenia reflect effort computation impairments during cost-benefit decision-making

Background

Motivational impairments are a core feature of schizophrenia and although there are numerous reports studying this feature using clinical rating scales, objective behavioural assessments are lacking. Here, we use a translational paradigm to measure incentive motivation in individuals with schizophrenia.

Methods

Sixteen stable outpatients with schizophrenia and sixteen matched healthy controls completed a modified version of the Effort Expenditure for Rewards Task that accounts for differences in motoric ability. Briefly, subjects were presented with a series of trials where they may choose to expend a greater amount of effort for a larger monetary reward versus less effort for a smaller reward. Additionally, the probability of receiving money for a given trial was varied at 12%, 50% and 88%. Clinical and other reward-related variables were also evaluated.

Results

Patients opted to expend greater effort significantly less than controls for trials of high, but uncertain (i.e. 50% and 88% probability) incentive value, which was related to amotivation and neurocognitive deficits. Other abnormalities were also noted but were related to different clinical variables such as impulsivity (low reward and 12% probability). These motivational deficits were not due to group differences in reward learning, reward valuation or hedonic capacity.

Conclusions

Our findings offer novel support for incentive motivation deficits in schizophrenia. Clinical amotivation is associated with impairments in the computation of effort during cost-benefit decision-making. This objective translational paradigm may guide future investigations of the neural circuitry underlying these motivational impairments.     

Apathy,poor verbal memory and male gender predict lower psychosocial functioning one year after the first treatment of psychosis

Background

Apathy is a negative symptom associated with poor psychosocial functioning in schizophrenia but has not been sufficiently studied as predictor of poor functioning in first episode psychosis (FEP).

Objective

The main aim of the current study was to evaluate if apathy predicts poor functioning after 1 year in FEP patients in the context of other clinical variables with influence on outcome.

Method

Sixty-four FEP patients completed an extensive clinical and neuro-psychological test battery at baseline and 1-year follow-up. Symptoms were assessed with the Positive and Negative Syndrome scale (PANSS), apathy with the shortened Apathy Evaluation Scale (AES-C-12) and psychosocial functioning with the functioning score from the split version of the Global Assessment of Functioning scale (GAF-F).

Results

High levels of apathy, poor verbal memory and being male were the baseline variables that best predicted poor functioning at 1-year follow-up, explaining 34% of the variance in GAF-F. When PANSS negative factor was included in the analysis, the significance of AES-C-12 diminished.

Conclusion

These findings points to a robust role for apathy among the negative symptoms in the development of persisting psychosocial dysfunction in FEP and supports the current effort in targeting motivation to improve functioning.     

Effects of a Non-focal Plasticity Protocol on Apathy in Moderate Alzheimer's Disease: A Randomized,Double-blind,Sham-controlled Trial

BackgroundApathy is the most common neuropsychiatric symptom in Alzheimer's disease (AD) and it is associated with changes in prefrontal neural circuits involved with generation of voluntary actions. To date no effective treatment for apathy has been demonstrated.ObjectiveWe aimed to investigate the effects and safety of repetitive transcranial direct current stimulation (tDCS) on apathy in moderate AD patients.MethodsForty patients were randomized to receive either active or sham-tDCS over the left dorsolateral prefrontal cortex (DLPFC). Patients received six sessions of intervention during 2 weeks and were evaluated at baseline, at week 1 and 2, and after 1 week without intervention. Clinical raters, patients, and caregivers were blinded. The primary outcome was apathy. Global cognition and neuropsychiatric symptoms were examined as secondary outcomes.ResultsThe mean MMSE score at baseline was 15.2 ± 2.9 and the mean Apathy Scale score was 27.7 ± 6.7. Changes on apathy scores over time were not different between active and sham tDCS (P = 0.552 for repeated measures). Further analyses confirm that changes from baseline did not differ between groups after the sixth session (active tDCS −1.95 (95%CI −3.49, −0.41); sham-tDCS −2.05 (95%CI −3.68, −0.42); P = 0.989]. Similarly, tDCS had no effect on secondary outcomes (P > 0.40). tDCS was well tolerated and not associated with significant adverse effects.ConclusionIn this adequately powered study for minimal clinically significant difference, our findings show that using the parameters we chose for this study, repeated anodal tDCS over the left DLPFC had no effect on apathy in elderly patients with moderate AD.     

轻度认知障碍患者共情能力和淡漠的变化研究及其与灰质萎缩的相关性分析

目的 研究轻度认知障碍患者共情能力与淡漠症状的变化,探索两者的联系及与脑灰质体积的相关性。方法 纳入2019年7月至2020年7月诊断为MCI的病例22例,选择同时期性别、年龄、教育程度无差异的对照组21例,所有受试者完成神经心理学测评,包括MMSE、MoCA、SDS、IRI(包括PT、FS、EC、PD四个子项目)、BES(包括认知移情和情感移情)和AES-I评分,同时测量磁共振的灰质体积,两组结果进行对比并行相关性分析。结果MCI组的MMSE、MoCA评分明显低于对照组(P0.05);MCI组的共情测量评分中IRI总分及PT、FS评分显著低于对照组;在移情测评中,MCI组BES总分低于对照组(P0.05),主要是情感移情评分;淡漠评定量表-知情者版(AES-I)的测评中,MCI组的评分显著升高(P0.05);MCI组的T1加权像VBM测量灰质体积显著小于对照组(P0.05)。在进一步相关性及回归分析中,Pearson分析结果示共情能力与淡漠无相关性; IRI中的PD评分、淡漠评分与灰质萎缩呈显著负相关(B=-4.098,P=0.032;B=-2.987,P=0.022);而情感移情评分与灰质萎缩呈显著正相关(B=3.623,P=0.013)。结论 轻度认知障碍患者共情能力减退,情感移情能力较低,出现淡漠等早期精神行为症状,已有一定的灰质萎缩,且灰质萎缩与患者共情能力减低及淡漠的程度有相关性。     

Multiple factors,including non-motor impairments,influence decision making with regard to exercise participation in Parkinson’s disease: a qualitative enquiry

Purpose: To explore how the meaning of exercise and other factors interact and influence the exercise behaviour of individuals with Parkinson’s disease (PD) enrolled in a 6-month minimally supervised exercise program to prevent falls, regardless of whether they completed the prescribed exercise or not. Method: This qualitative study utilised in-depth semi-structured interviews analysed using grounded theory methodology. Results: Four main themes were constructed from the data: adapting to change and loss, the influence of others, making sense of the exercise experience and hope for a more active future. Participation in the PD-specific physiotherapy program involving group exercise provided an opportunity for participants to reframe their identity of their “active” self. Three new influences on exercise participation were identified and explored: non-motor impairments of apathy and fatigue, the belief in a finite energy quota, and the importance of feedback. A model was developed incorporating the themes and influences to explain decision-making for exercise participation in this group. Conclusion: Complex and interacting issues, including non-motor impairments, need to be considered in order to enhance the development and ongoing implementation of effective exercise programmes for people with PD.

• Implications for Rehabilitation

• Exercise participation can assist individuals to reframe their identity as they are faced with losses associated with Parkinson’s disease and ageing.

• Non-motor impairments of apathy and fatigue may influence exercise participation in people with Parkinson’s disease.

• Particular attention needs to be paid to the provision of feedback in exercise programs for people with Parkinson’s disease as it important for their decision-making about continuing exercise.