Excessive emotional reactivity in a case of behavioral variant frontotemporal dementia with amyotrophic lateral sclerosis

Abstract

Although amyotrophic lateral sclerosis (ALS) is defined as a neuromuscular disease, cognitive and/or behavioral symptoms are relatively common, and a portion of ALS patients will meet criteria for behavioral variant frontotemporal dementia (bvFTD). In this report, we describe the case of a man with ALS with bvFTD (ALS-FTD) presenting with excessive emotional reactivity, including severe anger, aggression, and obsessive thoughts. We contrast this case with the decreased emotional reactivity that is usually observed in patients with bvFTD without ALS. We discuss possible explanations including that: 1) the behavioral symptoms of bvFTD and ALS-FTD are the same, but the motor dysfunction influences the clinical manifestations of the behavioral symptoms in ALS-FTD; 2) the emotional and behavioral symptoms of bvFTD and ALS-FTD are the same, but ALS-FTD patients come to clinical attention earlier in the course of their FTD than bvFTD patients without ALS; and 3) the emotional and behavioral symptoms of bvFTD and ALS-FTD could differ.

Keywords: ALS-FTD; Amyotrophic lateral sclerosis; Behavioral symptoms; Emotional reactivity; Emotional symptoms; Frontotemporal dementia.

Fig. 1.

Radar plot depicting z-scores on behavioral and emotional assessments. Z-scores for IRI and RSMS have been reversed, so that higher scores always mean worse or more abnormal functioning. The red line denotes the 1.5 SD cut-off that suggests clinical significance; any scores outside this red line can be considered clinically significant. FrSBe domains are post-symptom onset only. The RSMS socio-emotional sensitivity value is the total score with the two subscales added together.

Fig. 2.

Visual representation of the “before” and “after” symptom scores on the three FrSBe domains. A T-score above 65 indicates clinical significance. All three domains exceeded this cut-off after symptom onset.