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Although amyotrophic lateral sclerosis with dementia (ALS-D) has been characterized by symptoms of fronto-temporal dysfunction, we report two patients with ALS-D who showed constructive disturbance and low-level perfusion in the parietal areas. The first was a 69-year-old woman (Case 1) who had been diagnosed with the bulbar type of ALS. She showed fronto-temporal dementia as well as low scores and disturbance on block construction and copying; however, she showed a better score on the imitation of finger postures. The second was a 73-year-old woman (Case 2) who had been diagnosed with the leg onset type of ALS. She showed mild impairment of the frontal function as well as mild disturbance on block construction and copying, but no problem on the imitation of finger postures. Case 1 showed more severe symptoms of dementia and constructive disturbance than Case 2, whereas Case 2 showed lower levels of cerebral perfusion over more extensive areas than Case 1. Cases 1 and 2 were compatible with definite ALS according to the El Escorial Criteria, and they showed constructive disturbance with characteristics reported previously, such as both left and right hemisphere damage and constructive disturbance similar to those seen in Alzheimer's disease. In addition, they showed poorer scores on performing tasks requiring the use of objects (block construction and copying) rather than using their body (imitation of finger postures).

Constructive disturbance and low-level perfusion in parietal areas in amyotrophic lateral sclerosis with dementia