Adriana M. Strutt

Detecting frontotemporal dysfunction in ALS: Utility of the ALS Cognitive Behavioral Screen (ALS-CBS™)

Abstract Up to half of patients with ALS develop cognitive impairment during the course of the illness. Despite this, there is no simple tool for screening patients in the clinical setting. This study examines the sensitivity, specificity and accuracy of the ALS Cognitive Behavioral Screen (ALS-CBS™). We administered the measure to 112 ALS patients, including 31 who also underwent comprehensive neuropsychological testing. Screen results were validated by determining the accuracy against the full battery. Optimal cut-off scores for predicting the correct diagnosis were determined, and mean scores were compared between patients, controls and different diagnostic groups. The results demonstrated that mean cognitive scores differed between ALS and normal controls (p <0.0001). The cognitive section differentiated ALS-FTD from other ALS patients with 100% accuracy. Cognitively normal ALS patients could be distinguished from those with any cognitive deficit with 71% specificity and 85% sensitivity. A separate behavioral score was significantly lower in the ALS cohort compared to controls (p <0.0001) and predicted ALS-FTD with 80% sensitivity and 88% specificity. In conclusion, the ALS-CBS™ can aid in detecting cognitive and behavioral impairment in a clinical setting, although it does not replace formal diagnostic assessment. Further validation with larger sample sizes will clarify its clinical utility.

Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis

Background:  Cognitive impairment secondary to frontal lobe atrophy exists in 40–60% of Amyotrophic Lateral Sclerosis (ALS) cases. We aimed to determine the prevalence of frontal‐lobe mediated behavioral impairment in (ALS) and to ascertain its relationship to cognitive impairment.

A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS

Abstract Our objective was to test the hypothesis that changes in body mass index (BMI) are associated with changes in the clinical course of ALS. We examined the relationships between BMI at first clinical visit and changes in BMI up to a two-year follow-up, and multiple clinical variables related to ALS: age of onset, rate of progression of motor symptoms, and survival. Baseline BMI was classified according to the World Health Organization (WHO) criteria. Changes in BMI were classified as a loss of >1 unit, no change, or a gain of >1 unit. Our results showed that baseline BMI was not associated with age of onset, rate of progression or survival. In contrast, a loss of BMI >1 over two years was associated with significantly shorter survival and a faster rate of progression. In a multiple regression model, these results were independent of gender, site of onset, history of diabetes mellitus and apolipoprotein (ApoE) genotype. In summary, a change in BMI after ALS diagnosis was significantly associated with rate of progression and survival. This raises the possibility that early changes in BMI may identify patients likely to have a more malignant course of the disease. However, further research is needed to clarify the relationship between BMI and ALS.

ALS disease onset may occur later in patients with pre-morbid diabetes mellitus.

Background  Several metabolic derangements associated with diabetes mellitus type 2 (DM) have been associated with a better outcome in amyotrophic lateral sclerosis (ALS), including hyperlipidemia and obesity. Here, we tested the hypothesis that DM would have a positive effect on the motor and cognitive findings of ALS.

Cognitive Outcome and Reliable Change Indices Two Years Following Bilateral Subthalamic Nucleus Deep Brain Stimulation

Subthalamic nucleus deep brain stimulation (STN-DBS) is currently the treatment of choice for medication-resistant levodopa-related motor complications in patients with Parkinsons disease (PD). While STN-DBS often results in meaningful motor improvements, consensus regarding long-term neuropsychological outcome continues to be debated. We assessed the cognitive outcomes of 19 STN-DBS patients compared to a group of 18 medically-managed PD patients on a comprehensive neuropsychological battery at baseline and two years post-surgery. Patients did not demonstrate changes in global cognitive functioning on screening measures. However, neuropsychological results revealed impairments in nonverbal recall, oral information processing speed, and lexical and semantic fluency in STN-DBS patients compared to PD controls 2 years post-surgery in these preliminary analyses. Additionally, reliable change indices revealed that approximately 50% of STN-DBS patients demonstrated significant declines in nonverbal memory and oral information processing speed compared to 25-30% of PD controls, and 26% of STN-DBS patients declined on lexical fluency compared to 11% of PD patients. Approximately 30% of both groups declined on semantic fluency. The number of STN-DBS patients who converted to dementia 2 years following surgery was not significantly different from the PD participants (32% versus 16%, respectively). Our results suggest that neuropsychological evaluations may identify possible mild cognitive changes following surgery.

Changes in cognitive-emotional and physiological symptoms of depression following STN-DBS for the treatment of Parkinson’s disease

Background and purpose:  Subthalamic nucleus deep brain stimulation (STN‐DBS) has been shown to have beneficial effects on the motor features of Parkinson’s disease (PD), but its impact on non‐motor symptoms, most notably mood, has not been fully explored.

Assessment of cognition in early dementia.

Better tools for assessing cognitive impairment in the early stages of Alzheimers disease (AD) are required to enable diagnosis of the disease before substantial neurodegeneration has taken place and to allow for detection of subtle changes in the early stages of progression of the disease. The National Institute on Aging and the Alzheimers Association convened a meeting to discuss state‐of‐the art methods for cognitive assessment, including computerized batteries, as well as new approaches in the pipeline. Speakers described research using novel tests of object recognition, spatial navigation, attentional control, semantic memory, semantic interference, prospective memory, false memory, and executive function as among the tools that could provide earlier identification of individuals with AD. In addition to early detection, there is a need for assessments that reflect real‐world situations so as to better assess functional disability. It is especially important to develop assessment tools that are useful in ethnically, culturally, and linguistically diverse populations as well as in individuals with neurodegenerative disease other than AD.

A comprehensive neuropsychological profile of women with psychogenic nonepileptic seizures

The purpose of this study was to compare the neuropsychological profile of women with VEEG-confirmed diagnoses of psychogenic nonepileptic seizures (PNES) with that of an age- and education-matched group of women with left temporal lobe epilepsy (LTLE). Results indicate that in a relatively homogenous PNES sample, no severe neurocognitive impairments were present, further supporting a psychologically versus neurologically driven pathology of PNES. In comparison to age-stratified normative data, the PNES group demonstrated only a modest deficiency across neuropsychological domains and a relative area of weakness in attention and working memory, and generally outperformed their counterparts with LTLE. Although the attentional deficits in the PNES group may have been influenced by their elevated levels of emotional distress, symptoms of depression and anxiety were significant and common in both patients with PNES and those with LTLE, and therefore, the utility of psychological factors in discriminating these groups is limited. The present findings warrant the use of longitudinal research with patients with PNES to identify changes in the presentation of this condition as well as its subsequent neurocognitive and emotional impairments.

Screening for neurocognitive impairment in HIV individuals: the utility of the Montreal cognitive assessment test.

Human Immunodeficiency virus (HIV) associated neurocognitive disorders have been reported in up to 50% of patients on highly active antiretroviral therapy (HAART) [1]. Even though screening for neurocognitive impairment (NCI) is not routinely done or recommended by current treatment guidelines [2], NCI is associated with HAARTnoncompliance and functional impairment [3], and is currently being linked to cerebrospinal fluid escape (positive CSF HIV RNA PCR in the setting of undetectable serum HIV RNA PCR) [4]. The Montreal cognitive assessment (MoCA) has been validated and widely used in several countries and languages in non-HIV infected populations to screen for cognitive impairment [5,6]. There is very limited data in HIV positive patients [7], a population that is associated with high rates of co-existing infections and comorbidities including depression and drug abuse. The purpose of our study was to evaluate the utility of the MoCA as a screening tool for NCI in HIV infected individuals.

Neurocognitive deficits increase risk of poor retention in care among older adults with newly diagnosed HIV infection.

Objective:To evaluate the role of neurocognitive impairment on retention in care across the lifespan in antiretroviral-naïve persons newly diagnosed with HIV. Design:A prospective observational study of 138 antiretroviral-naive newly diagnosed HIV-positive participants who presented to an urban clinic between August 2010 and April 2013. Methods:All participants underwent a baseline evaluation that included a neuromedical examination and brief neuropsychological test battery. Retention in care was operationalized as attending at least two visits separated by more than 90 days during the 12-month follow-up period. Results:Fifty-five per cent of participants were retained in care over the study observation period. In a logistic regression controlling for ethnicity, there was a significant interaction between age and neurocognitive impairment in predicting retention in care (P = 0.009). Planned post-hoc analyses showed that neurocognitive impairment was associated with a significantly lower likelihood of retention in care among participants aged 50 years and older (P = .007), but not among younger participants (P > 0.05). Conclusion:Extending prior research on antiretroviral adherence and medication management, findings from this study indicate that neurocognitive impairment may be an especially salient risk factor for poor retention in care among older adults with newly diagnosed HIV infection.