Syntactic comprehension in patients with amyotrophic lateral sclerosis.

Kentarou Yoshizawa,Wakana Hata,Nao Yasuda,Yumi Yukimoto,Michinari Fukuda,Mieko Ogino,Mari Higashikawa,Ikuyo Ishizaka

doi:10.1155/2014/230578

Abstract

Recent neuropsychological studies of patients with amyotrophic lateral sclerosis (ALS) have demonstrated that some patients have aphasic symptoms, including impaired syntactic comprehension. However, it is not known if syntactic comprehension disorder is related to executive and visuospatial dysfunction. In this study, we evaluated syntactic comprehension using the Syntax Test for Aphasia (STA) auditory comprehension task, frontal executive function using the Frontal Assessment Battery (FAB), visuospatial function using Raven's Coloured Progressive Matrices (RCPM), and dementia using the Hasegawa Dementia Scale-Revised (HDS-R) in 25 patients with ALS. Of the 25 patients, 18 (72%) had syntactic comprehension disorder (STA score < IV), nine (36%) had frontal executive dysfunction (FAB score < 14), six (24%) had visuospatial dysfunction (RCPM score < 24), and none had dementia (HDS-R score < 20). Nine of the 18 patients with syntactic comprehension disorder (50%) passed the FAB and RCPM. Although sample size was small, these patients had a low STA score but normal FAB and RCPM score. All patients with bulbar onset ALS had syntactic comprehension disorder. These results indicate that it might be necessary to assess syntactic comprehension in patients with bulbar onset ALS. The implications of these findings are discussed in relation to the pathological continuum of ALS.

Highlights

Amyotrophic lateral sclerosis (ALS) has historically been considered a neurodegenerative disease characterized by the progressive involvement of upper and lower motor neurons at the bulbar and spinal level
Eleven patients were classified with bulbar onset ALS, 10 with upper limb onset ALS, and four with
We should take into account that we might have underestimated the frequency of syntactic comprehension disorder, because the language disorders are often associated with the bulbar presentation

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) has historically been considered a neurodegenerative disease characterized by the progressive involvement of upper and lower motor neurons at the bulbar and spinal level. The consensus criteria have changed, and ALS is considered a multisystem disorder in which motor system deficits are prominent but nonmotor deficits can be observed [1]. Phukan et al [2] reported that, of 160 patients with ALS, 14% fulfilled the Neary criteria for frontotemporal dementia, 21% had executive dysfunction without dementia, and 14% had cognitive impairment without dementia or executive dysfunction. The frontal lobe contributes to executive function, language function, and elementary motor function. Executive function refers to higher-level cognitive functions that contribute to the control and direction of lower-level functions such as language, cognition, behavior, and memory [3]. There have been many reports of executive function in patients with ALS, and they have consistently shown that fluency, set-shifting, attention, inhibition, and working memory are impaired [4,5,6]

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