ALS related to cognitive and behavioral impairment

Amyotrophic lateral sclerosis (ALS) was associated with cognitive and behavioral changes, particularly later in the disease, Scottish researchers reported.

According to Sharon Abrahams, PhD, of the University of Edinburgh, and colleagues at the University of Edinburgh, in a cross-sectional observational study, patients with ALS experienced cognitive and behavioral disorders at different stages of life. disease. Neurology.

"Unfortunately, we found that people with ALS had thinking problems and behavioral problems such as apathy, changes in eating behaviors and lack of inhibition, even in the early stages of the disease." . "In the last stage of the disease, only a small percentage of people are free from these cognitive and behavioral problems."

Paul Wicks, PhD, of Patients Like Me in Cambridge, Mass., And Steven Albert, PhD, of the University of Pittsburgh, found that cognitive and behavioral symptoms are more common in more advanced stages of ALS. editorial accompaniment.

"When Professor Stephen Hawking died in March 2018 after living with amyotrophic lateral sclerosis for 55 years, the obituaries rightly celebrated a brilliant mind trapped in a failing body," they wrote. "Unfortunately, this abridged description of ALS (found in the medical literature and secular literature) is strongly contradicted by a research base that finds frontotemporal dementia in 10% to 15% of cases and subtle cognitive deficits in 33% to 50%. "

The Abrahams group compared 161 ALS patients and 80 matched healthy controls in three research sites. Participants received the cognitive and behavioral screening of ALS from Edinburgh (ECAS), which measures cognitive impairment and behavioral disorders specific to ALS (executive, linguistic and letter) and non-specific ALS. (memory and visuospatial). Patients were classified according to their clinical stage as measured by the King's clinical classification system at the time of testing.

There were no significant differences between baseline levels between ALS and control groups, including depression and anxiety levels. Most patients (64%) had classic ALS with symptoms in the upper or lower limbs; 26% had a bulbar onset; 9% had a mixed start; and 1% had a respiratory onset.

The researchers compared the cognitive performance of ALS patients to the control group for each ECAS domain and observed significant differences in language, executive functions, fluidity of letters, and memory, but no significant differences in the visuospatial operation.

The ALS-specific composite scores, not specific to ALS and ECAS, all showed significant differences between the groups. Among patients with ALS, 28.5% had a cognitive deficit on the total ECAS. Letter-control disorders were the most frequent (30.4%), followed by management (22.5%) and language (21.3%) dysfunction. Memory (16.8%) and visuospatial (9.4%) disorders were less common.

A behavioral deficiency (according to revised consensus guidelines) occurred in 39.6% of ALS patients: apathy was most common at 30.9%, followed by loss of sympathy / empathy (27%). , 5%), changes in eating behavior (24.8%) and disinhibition (15.4%).

Total ECAS scores and behavioral impairment were related to the stage of ALS disease. About 20% of ALS patients had impaired total ECAS scores in stages 1 and 2; 33.3% at stage 3; and 39.6% at stage 4. In stage 1, 17.7% of patients had behavioral problems; 26.8% at stage 2; 36.4% at stage 3; and 65.4% at stage 4. At stage 4, 80% overall had some kind of cognitive or behavioral deficit.

ALS patients whose disease affected the bulbar region were more likely to have cognitive and behavioral problems than patients whose disease did not affect this area, whether it was the first affected area of ​​the body, noted Abrahams.

Wicks and Albert noted that in a survey of 247 patients and 87 caregivers, 90% of patients and caregivers reported that their health care provider had not told them that cognitive or psychological symptoms could occur.

"In our experience, colleagues report keeping patient information to avoid further distress," they observed. But "if caregivers do not even know what to look for or how to call these events, it can be difficult to report them in brief semi-annual appointments that focus primarily on the patient's motor symptoms."

In the same survey, most respondents (62% of patients, 71% of caregivers) stated that they would have liked to be told that these problems may occur, they added. "The education of patients and caregivers on the fact that cognitive change is part of ALS should not be different from similar discussions to be conducted in the field of multiple sclerosis, Parkinson's disease and diabetes." 39, other diseases. "

This study was limited by its cross-sectional design, noted Abraham's group; a longitudinal study could better explore how cognitive and behavioral symptoms change as patients progress to later stages of the disease. It is possible that patients with lower cognitive functioning or more severe behavioral abnormalities were also less likely to participate, they added.

• Reviewed by
  Robert Jasmer, MD Associate Clinical Professor of Medicine, University of California, San Francisco and Dorothy Caputo, MA, BSN, RN, Nurse Planner

2018-09-12T17: 30: 00-0400