Spontaneous intracranial hypotension masquerading as frontotemporal dementia

F.D. exhibited the cognitive and behavioral profile of frontotemporal dementia in the context of spontaneous intracranial hypotension (SIH). Symptoms included orthostatic headache, as well as cognitive and personality changes. He underwent CT, EEG, and MRI as well as neuropsychological evaluations before and after corticosteroid treatment. The initial evaluation documented significant cognitive impairment with a predominance of executive dysfunction. Following treatment, a second evaluation revealed marked improvement in cognition and behavior. Rapid diagnosis and treatment can yield a favorable outcome. Both quantitative and qualitative information from measures of executive functioning were discussed, as well as their anatomical substrates

Deconstructing the symbol digit modalities test in multiple sclerosis: The role of memory

Background The Symbol Digit Modalities Test (SDMT) is a sensitive measure of impaired cognition in people with MS. While the SDMT is primarily considered a test of information processing speed, other components such as visual scanning and oral-motor ability have also been linked to performance. The objective of this study was to determine the role of memory in the performance of the SDMT. Methods Two version of a modified computerized SDMT (c-SDMT) were employed, a fixed and a variable. For each group 50 MS and 33 healthy control (HC) participants were recruited. In the fixed c-SDMT, the symbol-digit code is kept constant for the entire test whereas in the variable version, it changes eight times. Unlike the traditional SDMT which records the correct number of responses, the c-SDMT presented here measures the mean response time (in seconds) for the eight trials. Results MS participants were slower than HC on the fixed (p < 0.001) and variable (p = 0.005) c-SDMT. Trend analysis showed performance improvement on the fixed, but not on the variable c-SDMT in both MS and HC groups. Furthermore, immediate visual memory recall was associated with the fixed (β = −0.299, p = 0.017), but not variable (B = −0.057, p = 0.260) c-SDMT. Immediate verbal memory was not associated with either versions of the c-SDMT. Conclusions Given that the fixed and variable c-SDMTs are identical in every way apart from the fixity of the code, the ability of participants to speed up responses over the course of the fixed version only points to the contribution of incidental visual memory in test performance

Revisiting cognitive reserve and cognition in multiple sclerosis: A closer look at depression

Background: The protective effect of cognitive reserve (CR) on cognition in people with multiple sclerosis (PwMS) has been well described. Objective: To explore the relationship between aspects of CR, namely, leisure pursuits and depression. Methods: In a cross-sectional study, a sample of 155 PwMS and 115 healthy controls (HC) underwent cognitive testing with the Minimal Assessment of Cognitive Function in Multiple Sclerosis (MACFIMS) battery. Leisure activity was retrospectively recorded using the Leisure Activity Scale (LAS). Depression was assessed using the Hospital Anxiety and Depression Scale. Results: PwMS demonstrated greater decreases in leisure activity over time compared to the HC group, particularly in the past year (p < 0.001). Here, depression accounted for 17% of the variance in determining the level of leisure activity (p < 0.001). Premorbid IQ and leisure activity within the past year emerged as significant predictors of information processing speed, learning, memory and executive function. After controlling for depression, the influence of leisure activity on cognition was insignificant. Conclusion: Depression can cause significant changes in behaviour which can influence indices of CR, such as leisure pursuits. Successfully treating depression may lead to a more active lifestyle thereby offsetting in part the cognitive burden of disease

A longitudinal evaluation of cognitive fatigue on a task of sustained attention in early relapsing-remitting multiple sclerosis

Background: Cognitive fatigue can be objectively measured on tasks of sustained attention and can be defined as decreased performance as a result of sustained cognitive effort. Individuals with multiple sclerosis (MS) early in their disease are vulnerable to cognitive fatigue, although this has yet to be evaluated longitudinally. We aimed to evaluate cognitive fatigue over a 3-year interval in individuals with early-phase relapsing-remitting MS (RRMS). The sensitivity of the Paced Auditory Serial Addition Test (PASAT) at detecting cognitive fatigue was evaluated, as was the impact of scoring method. Methods: 32 people with MS and 32 controls completed the 3- and 2-second PASAT (PASAT-3" and -2") as a measure of sustained attention at baseline and 3-year follow-up. Results: Performance on the PASAT remained stable across time, with improvement noted on the PASAT- 2" likely due to practice and the small sample size. Cognitive fatigue was noted at both times, although sensitivity varied based on scoring method. No evidence of worsening cognitive fatigue was noted over time. The MS group performed worse only when cognitive fatigue was the outcome variable. Conclusions: Although individuals with MS continue to be vulnerable to cognitive fatigue at follow-up, severity does not seem to increase with time. Cognitive fatigue may be a more sensitive marker of cognitive impairment than overall task performance in those with early-phase RRMS, which has important implications given t

Predictive Models of Cognitive Fatigue in Multiple Sclerosis

Objective: Cognitive fatigue (CF) can be defined as decreased performance with sustained cognitive effort. The present study examined the interrelatedness of disease severity, fatigue, depression, and sleep quality in order to evaluate their predictive roles of CF in MS. Four theoretical models examining these variables were assessed. Methods: Fifty-eight individuals with a diagnosis of MS were recruited. CF was measured by examining last third versus first third performance on the Paced Auditory Serial Addition Test (PASAT). The PASAT and self-report measures of fatigue, depression, and sleep quality were administered. Path analysis was used to evaluate each of the models. Results: CF was correlated only with depression (r = .362, p = .006) and sleep quality (r = .433, p = .001). Sleep quality was the greatest significant independent predictor of CF (β = .433, t(1,55) = 3.53, p < .001), accounting for 17.3% of the total variance. The best fitting model showed sleep quality as the largest contributor to CF; however, depression played a smaller predictive role. Furthermore, depression emerged as the strongest predictor of sleep quality and fatigue. Disease severity weakly predicted depression. Conclusions: Sleep quality is the most significant predictor of CF in MS. As such, sleep quality may be a treatable cause of CF. Sleep quality itself, however, accounted for only 17.3% of the variance in C

Cognitive Fatigability Interventions in Neurological Conditions: A Systematic Review

Introduction: Although fatigue is a well-studied concept in neurological disease, cognitive fatigability (CF) is less understood. While most studies measure fatigue using subjective self-report, fewer have measured CF objectively. Given the negative impact of CF on quality-of-life, there is a need for targeted interventions. The objective of this review was to determine which procedural, behavioural and pharmacological treatments for objectively measured CF are available to people living with neurological conditions. Methods: In accordance with the PRISMA guidelines, systematic searches for randomized control trials (RCTs), case-controlled studies and case reports/series were conducted across the Ovid Medline, PsycInfo, EMBASE and Cochrane Library databases. English-language articles published between 1980 and February 2019 were considered for eligibility. Included were those that objectively measured CF in individuals with neurological disease/disorder/dysfunction between the ages of 18 and 65 years. Studies were reviewed using a modified Cochrane Data Extraction Template. Risk of bias was assessed using the Cochrane Risk of Bias tool. The review process was facilitated using Covidence software (www.covidence.org). Two authors reviewed articles independently, with a third resolving conflicts regarding article inclusion. Results: The search identified 450 records. After duplicates were removed and remaining titles/abstracts were screened for eligibility, 28 full-text articles were assessed, and two studies were included in the qualitative synthesis. Studies were a priori divided into those with pharmacological, procedural or behavioural interventions. Two studies met eligibility criteria; both of these included participants with multiple sclerosis. One s

Autosomal dominant cerebellar ataxia, deafness, and narcolepsy (ADCA-DN) associated with progressive cognitive and behavioral deterioration

Objective: Autosomal dominant cerebellar ataxia with deafness and narcolepsy (ADCA-DN) is an emerging syndrome caused by mutations in the C-terminus end of the TS domain of the DNMT1 gene. ADCA-DN is also associated with sensorimotor polyneuropathy, extrapyramidal, and dysautonomic signs, as well as dementia. Little has been reported about the progressive cognitive impairment associated with ADCA-DN. Our objective is to provide a detailed characterization of the cognitive profile of ADCA-DN. Method: Three members of a kindred with ADCA-DN underwent comprehensive neuropsychological testing and neuroimaging. Results: At baseline, 2 individuals demonstrated cognitive profiles with executive difficulties in some areas consistent with frontalsystem dysfunction behaviorally and on standardized testing. The third individual was further in the disease course and exhibited more globally impaired cognition consistent with a diagnosis of dementia. Conclusions: This family demonstrated progressive neurodegeneration beginning with isolated areas of executive dysfunction and leading to globally impaired cognition and dementia. Cognitive decline occurred in parallel with neurological deterioration. The cognitive profile is similar to case reports of other individuals with an allelic neurological phenotype, H

Longitudinal stability of cognition in early-phase relapsing-remitting multiple sclerosis; Does cognitive reserve play a role?

Background: Up to 70% of people with multiple sclerosis (MS) experience cognitive impairment. Some remain cognitively intact despite advanced disease. Cognitive reserve (CR) theory postulates that individuals with higher levels of intellectual enrichment can tolerate more pathology than others before exhibiting cognitive impairment. Methods: Thirty-two individuals with early-phase relapsing-remitting MS with mild physical disability and disease duration less than 10 years and 32 controls were recruited. At baseline and after 3 years, participants completed neuropsychological tests evaluating several cognitive domains. The CR was assessed via a cognitive reserve index (CRI) using educational levels and North American Adult Reading Test scores. Change in cognition was assessed using a reliable change index. Results: At baseline, people with MS performed worse than controls on visual memory. There were no significant group difference

The Computerized test of information processing (CTIP) Offers an alternative to the PASAT for assessing cognitive processing speed in individuals with multiple sclerosis

Objective: To compare the ability of the Computerized Test of Information Processing (CTIP) to detect impaired cognitive processing speed in patients with multiple sclerosis (MS) with a traditional 3.0 second Paced Auditory Serial Addition Test (PASAT) and the Adjusting-PASAT which allows for calculation of a speed score. Background: A primary cognitive deficit in MS is an impaired ability to process information quickly. Unfortunately, relatively few clinical tests effectively measure information processing speed. Of these, the PASAT is generally acknowledged to be the most sensitive, but use of this test is constrained by several factors. Methods: All tests were administered to 30 adults with relapsing-remitting MS and 30 control participants. Results: A series of analysis of variances revealed MS participants performed significantly worse than controls on the CTIP and the 3.0 second PASAT, whereas no significant difference was observed for the Adjusting-PASAT. Conclusions: The results suggest the CTIP can detect deficits in the speed at which people with MS process information. Thus, the CTIP offers an alternative means to the 3.0 second PASAT included in the Multiple Sclerosis Functional Composite for assessing such impairment. Copyrigh

Reaction time: An alternative method for assessing the effects of multiple sclerosis on information processing speed

The ability of a newly developed measure of information processing to detect deficits in cognitive functioning associated with multiple sclerosis (MS) was investigated. The Computerized Tests of Information Processing (CTIP; Tombaugh, T., & Rees, L. (1999). Computerized Tests of Information Processing (CTIP). Unpublished test. Ottawa, Ontario, Canada: Carleton University) was administered to 60 clinically definite MS patients and 60 healthy controls. MS patients responded significantly slower than controls on the reaction time tests composing the CTIP. Moreover, as the CTIP tests became more difficult (i.e. as processing demands increased), the difference between the performances of the two groups progressively increased. These results suggest the CTIP is sensitive to the cognitive deficits observed in MS and that this measure has the potential to serve as a viable alternative to traditional measures of information processing speed currently in use with MS patients