Abstract

To investigate the behavioral characteristics of semantic dementia (SD) using an instrument originally developed for patients with autism spectrum disorder. The behavioral symptoms of 20 patients with SD and 20 patients with Alzheimer's disease (AD) in both the preclinical state and the dementia state were evaluated using the Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS). The SD group showed high prevalence in four behaviors related to stereotypy and social impairment: eating very few food items, selfishness, difficulty in recognizing others' feeling and thoughts, and interpreting language literally. Scores on the PARS short version, which is sensitive for diagnosis of autism spectrum disorder, were significantly higher in the dementia state than in the preclinical state in both the SD (11.5 ± 6.0 and 1.7 ± 2.5, respectively; t (19) = 6.7, p < 0.001) and AD (6.9 ± 4.6 and 1.7 ± 2.0, respectively; t (19) = 5.1, p < 0.001) groups. PARS short version scores after dementia onset increased in both the SD and AD groups, although the increase was significantly larger in the SD group (F = 5.6, p = 0.023). Additionally, a significantly higher rate of patients exceeded the cutoff score for autism diagnosis in the dementia state in the SD group (75%) than in the AD group (40%; χ2 = 5.0, p = 0.025). PARS scores in the dementia state were significantly correlated with illness duration (r = 0.46, p = 0.04) and Mini-Mental State Examination scores (r = -0.75, p < 0.001) in the SD group only. Although SD and autism spectrum disorder are etiologically distinct diseases, patients with semantic dementia behave like those with autism spectrum disorder. Our findings suggest the symptomatic similarity of the two disorders.

Highlights

  • Semantic dementia (SD) is a subtype of frontotemporal dementia (FTD) characterized by progressive aphasia caused by semantic memory loss and severe focal atrophy in the temporal lobe [1]

  • Scores on the PARS short version, which is sensitive for diagnosis of autism spectrum disorder, were significantly higher in the dementia state than in the preclinical state in both the SD (11.5 ± 6.0 and 1.7 ± 2.5, respectively; t (19) = 6.7, p < 0.001) and Alzheimer’s disease (AD) (6.9 ± 4.6 and 1.7 ± 2.0, respectively; t (19) = 5.1, p < 0.001) groups

  • PARS short version scores after dementia onset increased in both the SD and AD groups, the increase was significantly larger in the SD group (F = 5.6, p = 0.023)

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Summary

Introduction

Semantic dementia (SD) is a subtype of frontotemporal dementia (FTD) characterized by progressive aphasia caused by semantic memory loss and severe focal atrophy in the temporal lobe [1]. Snowden et al compared clinical features of behavioral symptoms in SD and bvFTD using caregiver interviews originally designed to examine behavioral changes in FTD [4] They found that some behavioral symptoms, including selfishness, more selective/ food fads, repetitive themes, and adherence to daily routines, were more frequent in SD than in bvFTD [4]. Bozeat et al compared behavioral symptoms among SD, bvFTD, and AD using an originally designed questionnaire to confirm the neuropsychiatric symptoms commonly reported in FTD and/or AD [5] They showed that some behavioral symptoms, such as mental rigidity, loss of sympathy/empathy, and clockwatching, were more frequent in SD than in bvFTD and AD [5]. It is important to clarify the clinical features of abnormal behaviors in SD

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