A prospective study of cognitive impairment in ALS

Abstract

Bulbar-onset ALS patients with cognitive impairments and neuronal loss in the anterior cingulate gyrus subsequently developed more profound neuropsychological dysfunction whereas both language and speech capabilities remained relatively preserved. Of note, the absence of bulbar signs did not predict an absence of cognitive decline.

Screening for cognitive and behavioural impairment in amyotrophic lateral sclerosis: Frequency of abnormality and effect on survival

“…Previously, some studies have found that people with bulbar onset of ALS are more likely to have cognitive impairment than those with spinal onset [39,53,54], but others do not [33,35,55]. In our study, the site of onset had no effect of the presence of cognitive impairment as determined by the ACE-III, but site of onset influenced cognitive impairment based on FAB logistic regression results.…”

“…Some studies have found cognitive impairment to be more common in people with bulbar onset than limb onset of ALS [32]. However, other studies suggest that site of onset has no influence on the presence of cognitive impairment [33][34][35]. Therefore further information is required.…”

“…If this were the case, cognitive impairment would correlate with disease severity, but there is little information about this. There is conflicting information about whether there is later development of cognitive impairment in patients who are cognitively normal at onset of ALS [39][40][41][42] . Further information is required about this issue as well.…”

“…One study found that in bulbar onset patients, cognitive impairment was progressive over time [39], another found that 37% of patients developed cognitive deficit over a six month follow-up period [40], and another found that cognitive impairment was greater at the later stages of disease, when only 14% of patients had normal cognition [41], thus providing evidence that cognition is progressively affected in ALS. The previous serial studies of cognition are summarised in Table 8.…”

Degeneration of the Mid-Cingulate Cortex in Amyotrophic Lateral Sclerosis Detected In Vivo with MR Spectroscopy

“…31 We are aware of a single MRSI study of the anterior cingulate in ALS; it showed reduced NAA/Cr specifically in those with bulbar onset disease. 32 Convincing evidence for thalamic involvement in ALS has not been forthcoming from routine postmortem or standard anatomic imaging studies. There have been very few postmortem studies that have specifically examined the thalamus.…”