G.P.310

NEUROMUSCULAR DISORDERS(2014)

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摘要
There is a clinical, genetic and pathological overlap between amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). In 2008, our meta-analysis on the cognitive profile of ALS showed that fluency deficits, executive dysfunction and impairment of language and memory can be present in non-demented ALS patients. To further elucidate the cognitive profile of ALS we aimed to update the meta-analysis. We searched Embase, Medline and PsycInfo. Articles were selected if healthy volunteers and non-demented ALS patients, fulfilling El Escorial criteria, underwent at least one validated cognitive test. All tests were categorized in cognitive domains and effect sizes were calculated per domain. Demographic and clinical data were also extracted. We included 29 new articles, resulting in a total of 45 articles (n = 1292 patients and 1144 controls). The median number of neuropsychological (sub) tests administered to patients and controls was 8 (1–34). Patients (63% men, 33% bulbar onset) had a mean age of 59.1 years, a mean educational level of 11.8 years and a mean disease duration of 24 months. Most patients had mild to moderate disability (mean ALSFRS-R 33.1). The domains with the largest, significant effect sizes (Hedges’ g > 0.50) were visuoconstructive functions (0.63), global cognition (0.59), fluency (0.58), language (0.56), social cognition (0.55) and delayed verbal memory (0.52). Executive function had a significant effect size of 0.44. The update of the meta-analysis revealed a new cognitive domain with a large effect size, i.e. social cognition, which encompasses insight into social situations and recognition of emotional states of others. This highlights the clinical overlap between ALS and FTD. The update further reinforces that the cognitive profile extends beyond executive dysfunction and includes language and memory impairment and deficits in social cognition. In the visuoconstructive domain bias due to motor impairment could not be ruled out.
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