Preferred Label : Amyotrophic lateral sclerosis with frontotemporal dementia;
ICD-11 definition : Cognitive impairment and behavior changes particularly reflecting dysfunction of frontal
lobe functions, such as planning, executive functions, and performing complex sequential
tasks, occur in a proportion of ALS patients. There is a continuum, ranging from mild
changes to frank frontotemporal dementia (FTD). Neuroimaging may demonstrate atrophy,
hypoperfusion, and hypometabolism of frontal and temporal lobes. Patients with C9ORF72
expansions may present with FTD, ALS or overlap syndromes.;
Origin ID : 2134326880;
Currated CISMeF NLP mapping
Cognitive impairment and behavior changes particularly reflecting dysfunction of frontal
lobe functions, such as planning, executive functions, and performing complex sequential
tasks, occur in a proportion of ALS patients. There is a continuum, ranging from mild
changes to frank frontotemporal dementia (FTD). Neuroimaging may demonstrate atrophy,
hypoperfusion, and hypometabolism of frontal and temporal lobes. Patients with C9ORF72
expansions may present with FTD, ALS or overlap syndromes.
