Comparisons of patient self-report, neurologic examination, and functional impairment in MS

Hoogervorst, Erwin L.J.; Winsen, L.M.L. van; Eikelenboom, Merijn; Kalkers, Nynke F.; Uitdehaag, Bernard M. J.; Polman, Chris H.

doi:10.1212/wnl.56.7.934

Cited by 72 publications

(52 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most limitations mentioned are lessened or overcome by the large number of patients for a rare disease, enhancing the power of the study and compensating for missing data. Other strengths are the longitudinal character of the study, with duration of 12½ years, and the use of standardized instruments that have been extensively validated in diverse populations 18, 27, 30, 44, 45. The latter also enables the comparison of the burden of VWM to other patient groups.…”

Section: Discussionmentioning

confidence: 99%

Natural History of Vanishing White Matter

Hamilton

Lei

Vermeulen

et al. 2018

Annals of Neurology

173

View full text Add to dashboard Cite

ObjectiveTo comprehensively describe the natural history of vanishing white matter (VWM), aiming at improving counseling of patients/families and providing natural history data for future therapeutic trials.MethodsWe performed a longitudinal multicenter study among 296 genetically confirmed VWM patients. Clinical information was obtained via disease‐specific clinical questionnaire, Health Utilities Index and Guy's Neurological Disability Scale assessments, and chart review.ResultsFirst disease signs occurred at a median age of 3 years (mode = 2 years, range = before birth to 54 years); 60% of patients were symptomatic before the age of 4 years. The nature of the first signs varied for different ages of onset. Overall, motor problems were the most common presenting sign, especially in children. Adolescent and adult onset patients were more likely to exhibit cognitive problems early after disease onset. One hundred two patients were deceased. Multivariate Cox regression analysis revealed a positive relation between age at onset and both preservation of ambulation and survival. Absence of stress‐provoked episodes and absence of seizures predicted more favorable outcome. In patients with onset before 4 years, earlier onset was associated with more severe disability and higher mortality. For onset from 4 years on, disease course was generally milder, with a wide variation in severity. There were no significant differences for sex or for the 5 eIF2B gene groups. The results confirm the presence of a genotype–phenotype correlation.InterpretationThe VWM disease spectrum consists of a continuum with extremely wide variability. Age at onset is a strong predictor for disease course. Ann Neurol 2018;84:274–288

show abstract

Section: Discussionmentioning

confidence: 99%

Natural History of Vanishing White Matter

Hamilton

Lei

Vermeulen

et al. 2018

Annals of Neurology

173

View full text Add to dashboard Cite

show abstract

“…In the multiple sclerosis (MS) literature, research into self-awareness of cognitive deficits has been inconclusive, with a number of studies suggesting that people with MS are generally accurate in their report of their cognitive functioning (Basso et al, 2008;Benedict et al, 2004;Chiaravalloti & DeLuca, 2003;Kujala, Portin, & Ruutiainen, 1996;Matotek, Saling, Gates, & Sedal, 2001;, and others disputing this (Beatty & Monson, 1991;Christodoulou et al, 2005;Gold et al, 2001;Hoogervorst et al, 2001;Maor, Olmer, & Mozes, 2001;Marrie, Chelune, Miller, & Cohen, 2005;Middleton, Denney, Lynch, & Parmenter, 2006). Researchers have tended to use measures that assess awareness of overall cognitive functioning.…”

Section: Introductionmentioning

confidence: 99%

Awareness of executive functioning deficits in multiple sclerosis: Self versus informant ratings of impairment

Smith

Arnett

2010

Journal of Clinical and Experimental Neuropsychology

View full text Add to dashboard Cite

This study investigates the accuracy of self and informant ratings of executive dysfunction in a multiple sclerosis (MS) and control sample. Participants completed a neuropsychological battery. Participants and informants completed depression measures and the Dysexecutive Questionnaire (DEX). MS participants rated themselves more impaired than controls, while informants' ratings were not statistically different. MS participants' performance on executive tasks was more highly correlated with self than informant DEX ratings. Discrepancies between informant DEX ratings and executive performance were related to executive functioning for the MS participants only. Overall, results indicate that MS patients were able to accurately rate their executive functioning.

show abstract

“…Patients' self-report of physical status correlates better with objective physical examination than patients' self-report of cognitive status correlates with objective cognitive performance [39]. A similar discrepancy was reported between patients' and carers' reports of the impact of MS on daily life: the physical scale achieved better agreement between patients and carers than the psychological scale [40].…”

Section: Discussionmentioning

confidence: 93%

Reports of Patients and Relatives from the CogniCIS Study about Cognition in Clinically Isolated Syndrome: What Are Our Patients Telling Us?

et al. 2013

View full text Add to dashboard Cite

Objective: To assess the Multiple Sclerosis Neuropsychological Questionnaire (MSNQ) in patients with clinically isolated syndrome (CIS). Methods: 130 European CIS patients and 60 relatives completed the MSNQ. Results: The mean (SD) MSNQ score for CIS patients was 15.5 (10.8) and for their informants 11.3 (9.6). Neither the CIS patient nor relative MSNQ report scores correlated with any of the cognitive test scores in the Brief Repeatable Battery of Neuropsychological Tests, but they were significantly related to psychosocial scales including depression. Conclusions: In CIS, patient and relative MSNQ scores are influenced by psychosocial variables rather than actual objective cognitive status. Formal cognitive test assessment is recommended for CIS patients.

show abstract

Comparisons of patient self-report, neurologic examination, and functional impairment in MS

Cited by 72 publications

References 13 publications

Natural History of Vanishing White Matter

Natural History of Vanishing White Matter

Awareness of executive functioning deficits in multiple sclerosis: Self versus informant ratings of impairment

Reports of Patients and Relatives from the CogniCIS Study about Cognition in Clinically Isolated Syndrome: What Are Our Patients Telling Us?

Contact Info

Product

Resources

About