ABSTRACT
A growing body of research now challenges this assumption, with evidence that a strong link exists between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia (FTLD). A neuropsychologic and neurobehavioral evaluation provides clear confirmation of the presence of frontotemporal dementia or other dementia processes. The evaluation also can identify the more subde cognitive and behavioral abnormalities that may be present. Functional imaging studies provide support for the neural mechanisms for these cognitive and behavioral abnormalities, documenting cerebral abnormalities that extend well beyond the primary, secondary and sensorimotor cortices. Pathologic study of cognitively normal and abnormal ALS patients also supports the continuum hypothesis of the syndrome. A substantial literature exists regarding how clinical depression affects attention span, memory, executive function, social behavior and inhibition. Repeated assessment of depression level in the clinic is essential also to ensure proper identification of this potentially treatable problem.
