scholarly journals Higher Cortical Dysfunction Resembling Corticobasal Syndrome in 2 Patients with Multiple Sclerosis

2018 ◽  
Vol 1 (1) ◽  
pp. 01-06
Author(s):  
Margaret M Swanberg

The cognitive dysfunction in Multiple Sclerosis is classically defined as deficits in executive function, processing speed and memory. This pattern has been termed “subcorticaldy sfunction”. However, a growing body of literature suggests that there is cortical disruption in patients with Multiple Sclerosis and higher cortical dysfunction may manifest as symptoms. Here is presented two patients with Multiple Sclerosis who developed symptoms suggestive of Corticobasal syndrome, a disorder of higher cortical dysfunction due to a number of different pathologic substrates.

2019 ◽  
Vol 34 (6) ◽  
pp. 973-973
Author(s):  
C Schieszler-Ockrassa ◽  
F Bylsma

Abstract Objective Multiple sclerosis (MS) is an autoimmune demyelinating disease more common in women than men, usually diagnosed between 20-30 years of age. Approximately 50% of individuals with MS develop cognitive dysfunction, with men and progressive MS subtype cases at higher risk (Beatty & Aupperle, 2002). Mr. Doe’s case is unique because he was diagnosed with primary progressive MS at age 56 and demonstrated only mild cognitive dysfunction. Method Mr. Doe presented to his neurologist with complaints of extreme fatigue, slowed processing, and sensory and motor disturbances. He was seen for neuropsychological evaluation one year after diagnosis and was reassessed one year later. He reported worsening mood including passive suicidal ideation since diagnosis. He reported difficulties with work duties (attorney) and household demands due to gradual motor and sensory disturbances, slowed processing speed, fatigue, and mood disturbance. Results Mr. Doe’s initial neuropsychological assessment revealed variability in auditory working memory, weakness in sustained visual attention, and mild deficits in upper extremity fine motor dexterity. Memory, executive functioning, language, and processing speed were all intact unless a motor component was involved (mild decline after one year). His cognitive performances remained generally stable after one year, but depression, anxiety, and hopelessness levels were all significantly worse. Conclusions Although Mr. Doe’s impairments are extremely mild and somewhat unexpected given the primary progressive MS diagnosis, his gender, and age, the affected domains are consistent with the diagnosis. This case demonstrates the importance of understanding base rates for conditions we assess, but also not ruling out lower base rate conditions.


2010 ◽  
Vol 16 (5) ◽  
pp. 829-835 ◽  
Author(s):  
RALPH H.B. BENEDICT ◽  
SARAH A. MORROW ◽  
BIANCA WEINSTOCK GUTTMAN ◽  
DIANE COOKFAIR ◽  
DAVID J. SCHRETLEN

AbstractCognitive reserve is widely recognized as a moderator of cognitive decline in patients with senile dementias such as Alzheimer’s disease. The same effect may occur in multiple sclerosis (MS), an immunologic disorder affecting the central nervous system. While MS is traditionally considered an inflammatory, white matter disease, degeneration of gray matter is increasingly recognized as the primary contributor to progressive cognitive decline. Our aim was to determine if individual differences in estimated cognitive reserve protect against the progression of cognitive dysfunction in MS. Ninety-one patients assessed twice roughly 5 years apart were identified retrospectively. Cognitive testing emphasized mental processing speed. Cognitive reserve was estimated by years of education and by performance on the North American Adult Reading Test (NAART). After controlling for baseline characteristics, both years of education (p = .013) and NAART scores (p = .049) significantly improved regression models predicting cognitive decline. Symbol Digit Modalities Test (SDMT) performance showed no significant change in patients with > 14 years of education, whereas it declined significantly in patients with ≤ 14 years of education. We conclude that greater cognitive reserve as indexed by either higher premorbid intelligence or more years of education protects against the progression of cognitive dysfunction in MS. (JINS, 2010, 16, 829–835.)


2006 ◽  
Vol 12 (1) ◽  
pp. 77-87 ◽  
Author(s):  
U Nocentini ◽  
P Pasqualetti ◽  
S Bonavita ◽  
M Buccafusca ◽  
M F De Caro ◽  
...  

Cognitive dysfunction is considered one of the clinical markers of multiple sclerosis (MS). However, in the literature there are inconsistent reports on the prevalence of cognitive dysfunction, and separate data for the relapsing-remitting (RR) type of the disease are not always presented. In this study, we submitted 461 RRMS patients to a battery of neuropsychological tests to investigate their impairment in various cognitive domains. As a consequence of the exclusion criteria, the sample is not fully representative of the entire population of RRMS patients. In this selected sample, when only the eight scores of a core battery (Mental Deterioration Battery) were considered (with respective cutoffs), it emerged that 31% of the patients were affected by some degree of cognitive deficit. In particular, 15% had mild, 11.2% moderate and 4.8% had severe impairment. Information processing speed was the most frequently impaired area, followed by memory. When two other tests (SDMT and MCST) were added and cognitive domains were considered, it emerged that 39.3% of the patients were impaired in two or more domains. When four subgroups were obtained by means of cluster analysis and then compared, it emerged that information processing speed and memory deficits differentiated the still cognitively unimpaired from the mildly impaired MS patients. Significant associations were found between cognitive and clinical characteristics. However, due to the large sample size, clinically irrelevant relationships may also have emerged. Even with the limitations imposed by the sample selection and the possible underestimation of the prevalence and severity of cognitive dysfunction, these results seem to provide further evidence that information processing speed deficit may be an early and important marker of cognitive impairment in MS patients.


Author(s):  
Swati Kumar ◽  
Goutam Gangopadhyay ◽  
Atanu Biswas ◽  
Souvik Dubey ◽  
Alak Pandit ◽  
...  

Abstract Background To compare the frequency and pattern of cognitive impairment in neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) patients. Results Twenty NMOSD and forty MS patients were included. Clinical and detailed neuropsychological assessment was done using frontal assessment battery and Kolkata cognitive battery supplemented with additional standard tests for different domains of cognitive functions. Domain wise tests were performed and compared. 15/20 (75%) NMOSD and 32/40 (80%) MS patients had cognitive impairment (p = 0.65). Executive function, verbal fluency, information processing speed, visuo-constructional ability, attention, complex calculation, and memory were more commonly involved in NMOSD in decreasing order. Compared to MS, the pattern was similar except that verbal fluency was more impaired in NMOSD. Expanded Disability Status scale (EDSS) correlated with cognitive involvement in NMOSD (p = 0.02) as against MS. Conclusions Executive function, verbal fluencies, and information processing speed were more affected compared to visual and verbal memory in NMOSD patients. The pattern of cognitive performance was similar in the MS group, even though clinical and radiological characteristics and pathophysiology is different, suggesting similar brain involvement.


2021 ◽  
Author(s):  
Shay Menascu ◽  
Roy Aloni ◽  
Mark Dolev ◽  
David Magalashvili ◽  
Keren Gutman ◽  
...  

Abstract BackgroundPrevention of cognitive decline in Multiple Sclerosis (MS) is of major importance. We explored the effect of short-term computerized game training on cognitive performance in MS patients with mild cognitive impairment.MethodsWe enrolled in this prospective study 100 eligible MS patients treated with Interferon-beta-1a (Rebif). All had mild cognitive impairment in either executive function or information processing speed. Patients were randomized 1:1 to either use the cognitive games platform byHappyNeuron (HN) or receive no intervention. Executive function and information processing speed scores were measured at 3 and 6 months from baseline to evaluate the effect of game training on cognitive scores.ResultsIn both executive function and information processing speed, the game Training group showed significant improvement after 3 and 6 months. The Non-Training group showed mild deterioration in both domains at 3 months, and further deterioration that became significant at 6 months in executive function. Furthermore, at 6 months, the percent of patients in the Training group that improved or remained stable in both cognitive domains was significantly higher compared to the Non-Training group.ConclusionsOur findings suggest that cognitive game training has a beneficial effect on cognitive performance in MS patients suffering from mild cognitive impairment. While further evaluation is required to assess the longevity of that effect, we nonetheless recommend to MS patients to be engaged in cognitive gaming practice as part of a holistic approach to treating their condition.


Brain ◽  
2019 ◽  
Vol 142 (9) ◽  
pp. 2800-2812 ◽  
Author(s):  
Anthony Feinstein ◽  
Cecilia Meza ◽  
Cristiana Stefan ◽  
Richard W. Staines

AbstractCognitive dysfunction affects 40–80% of patients with multiple sclerosis. Smoking cannabis may add to these deficits. It is unclear whether coming off cannabis results in cognitive improvement. To address this question, 40 patients with multiple sclerosis who started using cannabis after the onset of multiple sclerosis and who used it for at least 4 days a week over many years were divided by odd-even number selection into two groups: cannabis continuation and cannabis withdrawal. Assessments took place at baseline and after 28 days and included serial versions of the Brief Repeatable Neuropsychological Battery for multiple sclerosis containing tests of verbal and visual memory, processing speed and executive function; structural and functional MRI, the latter entailing a compatible version of the Symbol Digit Modalities Test; urine for cannabinoid metabolites to detect compliance with abstinence. Only those participants deemed globally impaired at baseline (failure on at least two cognitive domains) were enrolled. The results revealed that the two groups were well matched demographically and neurologically. One subject was removed from the withdrawal group because of failed abstinence. Urine analysis revealed the cannabinoid consumed was predominantly tetrahydrocannabinol (THC). There were no baseline between group cognitive differences, but by Day 28 the withdrawal group performed significantly better on every cognitive index (P < 0.0001 for all). Significant within group differences were present for every test over time, but only in the abstinent group (P < 0.0001 for all tests). There were no between group baseline or Day 28 differences in structural MRI indices (global atrophy, total T1 and T2 lesion volume). At index assessment the two groups had a similar performance on the functional MRI-compatible Symbol Digit Modalities Test and there were no group differences in brain activation. However, by Day 28, the withdrawal group completed more trials correctly (P < 0.012) and had a faster reaction time (P < 0.002), associated with significantly increased activation in brain regions known to be associated with performance of the test (bilateral inferior frontal gyri, caudate and declive/cerebellum, P < 0.001 for all regions). These results reveal that patients with multiple sclerosis who are frequent, long-term cannabis users can show significant improvements in memory, processing speed and executive function after 28 days of drug abstinence. The absence of similar improvements in a matched multiple sclerosis group that remained on cannabis shows that beneficial cognitive change after stopping cannabis is not solely attributable to the effects of practice.


2021 ◽  
pp. 174749302110128
Author(s):  
Stefania Nannoni ◽  
Laura Ohlmeier ◽  
Robin B Brown ◽  
Robin G Morris ◽  
Andrew D Mackinnon ◽  
...  

Background and aim Whether cerebral microbleeds (CMBs) cause cognitive impairment remains uncertain. We analysed whether CMBs are associated with cognitive dysfunction in patients with symptomatic cerebral small vessel disease (cSVD), and whether this association is independent of other cSVD neuroimaging markers. Methods We analysed consecutive patients with MRI-confirmed lacunar stroke included in DNA-Lacunar-2 multicentre study. CMBs were graded using the Brain Observer Microbleed Rating Scale (BOMBS). Neuropsychological assessment was performed using the Brief Memory and Executive Test (BMET). We analysed the association between CMBs, BMET and the following subdomains: executive function/processing speed (EF/PS) and orientation/memory (O/M). We also searched for an independent association between CMBs and vascular cognitive impairment (VCI), defined as BMET≤13. Results Out of 688 included patients, CMBs were detected in 192 (27.9%). After adjusting for WMH severity, lacune count and other confounders, both the presence and number of CMBs were significantly associated with impaired cognitive performance [β=-13.0; 95%CI= (-25.3;-0.6) and β=-13.1; 95%CI= (-19.8;-6.4), respectively]. On analysis of specific cognitive domains, associations were present for EF/PS [β=-5.8; 95%CI= (-9.3;-2.2) and β=-4.3; 95%CI= (-6.2;-2.4), respectively] but not for O/M [β=-0.4; 95%CI= (-4.0;3.2) and β=-2.1; 95%CI= (-4.0;0.1), respectively]. We also found an independent association between the presence and number of CMBs and VCI [adjusted OR=1.48; 95%CI= (1.01;2.18) and OR=1.43; 95%CI= (1.15;1.79), respectively). Conclusion In a large cohort of symptomatic cSVD patients, after controlling for other neuroimaging markers of cSVD severity, CMBs were associated with cognitive dysfunction. Executive function and processing speed were predominantly impaired. This might suggest a causal role of CMBs in determining VCI.


2021 ◽  
pp. 135245852110113
Author(s):  
Curtis Wojcik ◽  
Tom A Fuchs ◽  
Hoan Tran ◽  
Michael G Dwyer ◽  
Dejan Jakimovski ◽  
...  

Background: The sequence in which cognitive domains become impaired in multiple sclerosis (MS) is yet to be formally demonstrated. It is unclear whether processing speed dysfunction temporally precedes other cognitive impairments, such as memory and executive function. Objective: Determine the order in which different cognitive domains become impaired in MS and validate these findings using clinical and vocational outcomes. Methods: In a longitudinal sample of 1073 MS patients and 306 healthy controls, we measured performance on multiple, consensus-standard, neurocognitive tests. We used an event-based staging approach to model the sequence in which cognitive domains become impaired. Linear and logistic mixed-effects models were used to explore associations between stages of impairment, neurological disability, and employment status. Results: Our model suggested that the order of impairments was as follows: processing speed, visual learning, verbal learning, working memory/attention, and executive function. Stage of cognitive impairment predicted greater neurological disability, β = 0.16, SE = 0.02, p < 0.001, and probability of unemployment, β = 1.14, SE = 0.001, p < 0.001. Conclusion: This is the first study to introduce a cognitive staging and stratification system for MS. Findings underscore the importance of using the Symbol Digit Modalities Test in routine screening for cognitive impairment and memory testing to assess patients later in disease evolution.


Author(s):  
Shay Menascu ◽  
Roy Aloni ◽  
Mark Dolev ◽  
David Magalashvili ◽  
Keren Gutman ◽  
...  

Abstract Background Prevention of cognitive decline in Multiple Sclerosis (MS) is of major importance. We explored the effect of a 6 months computerized game training program on cognitive performance in MS patients with mild cognitive impairment. Methods This was a single-center, randomized prospective study. We enrolled in this study 100 eligible MS patients treated with Interferon-beta-1a (Rebif). All had mild cognitive impairment in either executive function or information processing speed. Patients were randomized 1:1 to either use the cognitive games platform by HappyNeuron (HN) or receive no intervention. Executive function and information processing speed scores were measured at 3 and 6 months from baseline to evaluate the effect of game training on cognitive scores. Results In both executive function and information processing speed, the game Training group showed significant improvement after 3 and 6 months. The Non-Training group showed mild deterioration in both domains at 3 months, and further deterioration that became significant at 6 months in executive function. Furthermore, at 6 months, the percent of patients in the Training group that improved or remained stable in both cognitive domains was significantly higher compared to the Non-Training group. Conclusions Our findings suggest that cognitive game training has a beneficial effect on cognitive performance in MS patients suffering from mild cognitive impairment. While further evaluation is required to assess the longevity of that effect, we nonetheless recommend to MS patients to be engaged in cognitive gaming practice as part of a holistic approach to treating their condition.


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