The range and clinical impact of cognitive impairment in French patients with ALS: A cross-sectional study of neuropsychological test performance

Abstract

Our objective was to assess the spectrum and clinical associations of cognitive impairment in French patients with ALS, and determine the effect of cognitive impairment on survival in this population. One hundred and thirty-one patients were enrolled in a cross-sectional cohort study of neuropsychological test performance. ANOVA and χ(2) tests assessed differences in clinical characteristics between impaired and unimpaired patients; multiple regression determined which features contributed most strongly to cognitive status, and Cox models compared survival. Fifty-three patients (40%) were categorized as cognitively impaired based on test performance. Thirteen (10%) patients had frontotemporal dementia (FTD) clinically; all scored in the moderate to severely impaired range on testing. Impaired patients had less education (p = 0.001), and severely impaired patients were more likely to have bulbar onset than unimpaired patients (p < 0.001). Severe cognitive impairment predicted shorter survival (p = 0.007), even when controlled for motor severity (p = 0.001). In summary, 10% of a consecutive series of French ALS patients had overt dementia and 40% were cognitively impaired by neuropsychological testing. We conclude that lower education level and possibly bulbar-onset ALS were associated with impairment. As in other causes of dementia, higher education attainment may protect against clinical cognitive deterioration in ALS. French patients with severe cognitive impairment have shorter survival time.

Predicting Survival of Patients with Amyotrophic Lateral Sclerosis at Presentation: A 15-Year Experience

“…Bulbar onset occurs with increased frequency in older age groups [25], but this association does not appear to fully explain the worse prognosis of those with bulbar-onset symptoms [10]. The worse outcome in patients with bulbar-onset ALS could be related to poorer compliance with noninvasive ventilation, increased airway obstruction and aspiration due to excessive secretions or greater association with extramotor findings including dementia [26]. The delay between symptom onset and first visit, a surrogate for the rapidity of disease progression, has also been shown to predict survival, with shorter intervals portending worse prognosis [2,3,4,5,6,8,11,25,27,28,29,30].…”

No Change in Executive Performance in ALS Patients: A Longitudinal Neuropsychological Study

“…Further evidence of a relation comes from genetics [10,11] and neuroimaging studies [12,13,14]. It should be noted, however, that the majority of cognitively impaired ALS patients (70-80%) do not develop full-blown FTD, but instead remain in a sub-dementia state [3,8,9,15]. …”

Signs of MRI affection of brain in сlassical Amyotrophic Lateral Sclerosis patients

“…У 50 % пациентов со спорадическим БАС выявляются когнитивные и поведенческие расстройства, а у 10 % пациен-тов -фронтотемпоральная деменция [1, 15,27]. Клиническая гетерогенность БАС проявляется так-же случаями вовлечения экстрапирамидной и цере-беллярной систем, глазодвигательными расстрой-ствами, дисфункцией сенсорной и вегетативной нервной системы [13,16,43,44].…”