“Assessment of Neuropsychology in ALS and FTD”

Cognitive assessment in patients presenting with motor symptoms faces particular challenges. Patients’ performance is determined not only by their cognitive functions but also by their motor impairment: bulbar symptoms influence tasks requiring a verbal response (e.g. naming, verbal fluency) while tasks involving writing or drawing can be compromised by a wide range of motor symptoms, from weakness, through tremor, rigidity, akinesia and dystonia to apraxia. However, most currently available neuropsychological tests assume normal motor performance. Hence, they cannot distinguish whether a low score on a particular task is due to motor or cognitive dysfunction, a crucially important question when assessing the cognitive status.
In my presentation, I will discuss examples of the interference between motor and cognitive functions on neuropsychological testing. I will then present a new cognitive screening tool, ECAS (Edinburgh Cognitive Screen for ALS), specifically developed to assess cognitive functions in patients with motor impairment. It has been designed to minimize the influence of motor dysfunction through tasks requiring only minimal motor input such as pointing or yes/no answers. The whole test can be completed in a spoken or a written version. As such, the ECAS allows an adequate cognitive assessment in patients of the ALS/FTD spectrum.
I will then present an overview of the neuropsychological studies in ALS/FTD, focusing on the question whether the cognitive picture can be sufficiently explained by a combination of ALS and FTD or whether ALS/FTD patients show a specific pattern of cognitive dysfunction, distinct from that reported in ALS and FTD alone.