Cognitive Effects of Amyotrophic Lateral Sclerosis Increase as Disease Progresses

 

Although it is often referred to as a neurologic disease that attacks the body and not the mind, cognitive effects in amyotrophic lateral sclerosis (ALS) exist and have been reported in a study published in the journal Neurology(August 12, 2018). An accompanying editorial in the journal by Paul Wicks, PhD and Steven M Albert, PhD puts out a call to “stop saying the mind is unaffected in ALS,” noting that frontotemporal dementia is present in 10% to 15% of patients with ALS  and there are subtle cognitive deficits in 33% to 50% of patients with the disease.

Researchers enrolled 161 patients with and 80 persons without ALS and measured behavioral and cognitive symptoms with the Edinburgh Cognitive and Behavioral ALS Screen (ECAS); for patients who were not able to respond independently, caregivers reported on behavioral components including apathy and loss of empathy or sympathy. Complete data were collected for 146 patients with ALS and all 80 people without ALS. 

Of those with ALS, 29% had cognitive symptoms, most frequently in the areas of verbal fluency and executive function. Of 149 patients with ALS with information on behavioral symptoms, 55% had symptoms, the most frequent of which were apathy, loss of sympathy or empathy, and eating behaviors. The incidence of cognitive and behavioral symptoms both increased with disease progression. 

 

Table. Incidence of Cognitive and Behavioral Problems by Disease Stage

Disease Stage (King’s Clinical Staging System)

Cognitive Symptoms

Behavioral Symptoms

Stage 1 (upper extremities)

21%

18%

Stage 2 (all extremities)

21%

27%

Stage 3 (bulbar stage)

33%

36%

Stage 4 (respiratory/feeding tubes needed)

40%

65%

Individuals with ALS had significantly lower total scores (P = .05 after correction for multiple comparisons) than persons without the disease, attributable to differences in the areas of language, executive function, and fluency. Patients with bulbar symptoms were more likely to have cognitive and behavioral symptoms regardless of when in the disease course the bulbar symptoms appeared. 

Study author Sharon Abrams, PhD of the University of Edinburgh in the United Kingdom said, 

“People with ALS and their caregivers should also be informed that changes in thinking skills and behavior can be a part of ALS so they can plan for and watch for these issues and know that they are related to the disease itself,” and suggested that all patients with ALS should be screened for these symptoms.

 

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