Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia

Amyotrophic lateral sclerosis (ALS) with dementia (ALS-D) is known to exhibit characteristics of frontotemporal dementia. However, in clinical situations, it is often difficult to evaluate their cognitive functions because of impaired voluntary speech and physical disabilities. In order to identify...

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Main Authors: Hiroo Ichikawa, Shinichi Koyama, Hideki Ohno, Kenji Ishihara, Kiyomi Nagumo, Mitsuru Kawamura
Format: Article
Language:English
Published: Wiley 2008-01-01
Series:Behavioural Neurology
Online Access:http://dx.doi.org/10.1155/2008/814846
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author Hiroo Ichikawa
Shinichi Koyama
Hideki Ohno
Kenji Ishihara
Kiyomi Nagumo
Mitsuru Kawamura
author_facet Hiroo Ichikawa
Shinichi Koyama
Hideki Ohno
Kenji Ishihara
Kiyomi Nagumo
Mitsuru Kawamura
author_sort Hiroo Ichikawa
collection DOAJ
description Amyotrophic lateral sclerosis (ALS) with dementia (ALS-D) is known to exhibit characteristics of frontotemporal dementia. However, in clinical situations, it is often difficult to evaluate their cognitive functions because of impaired voluntary speech and physical disabilities. In order to identify characteristic and diagnostic cognitive symptoms of relatively advanced ALS-D patients, we retrospectively reviewed the clinical features of seven cases of clinically definitive ALS who had dementia, impaired voluntary speech, and physical disability. Their medical records showed that six out of seven patients made writing errors, and all of the patients demonstrated anosognosia. The writing errors consisted of paragraphia such as substitution, omission, or syntactic errors with individual differences in error types. Dissociation between kana and kanji were also observed. Anosognosia was evaluated by a self-rating scale with which the patients and the medical staff evaluated the patient's physical ability; the results indicated a large discrepancy between the evaluation by the patients and the medical staff. We emphasize that aphasic writing errors have been underestimated, particularly in ALS-D patients with impaired voluntary speech. We also reported that anosognosia was the most important and quantifiable symptom in ALS-D. The relationship between writing errors and anosognosia should be investigated further.
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spelling doaj-art-a936b31e0dbb4a9ab8e43c3fc7e68da82025-08-20T02:20:33ZengWileyBehavioural Neurology0953-41801875-85842008-01-0119310711610.1155/2008/814846Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with DementiaHiroo Ichikawa0Shinichi Koyama1Hideki Ohno2Kenji Ishihara3Kiyomi Nagumo4Mitsuru Kawamura5Department of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanDepartment of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanDepartment of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanDepartment of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanDepartment of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanDepartment of Neurology, Showa University School of Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, JapanAmyotrophic lateral sclerosis (ALS) with dementia (ALS-D) is known to exhibit characteristics of frontotemporal dementia. However, in clinical situations, it is often difficult to evaluate their cognitive functions because of impaired voluntary speech and physical disabilities. In order to identify characteristic and diagnostic cognitive symptoms of relatively advanced ALS-D patients, we retrospectively reviewed the clinical features of seven cases of clinically definitive ALS who had dementia, impaired voluntary speech, and physical disability. Their medical records showed that six out of seven patients made writing errors, and all of the patients demonstrated anosognosia. The writing errors consisted of paragraphia such as substitution, omission, or syntactic errors with individual differences in error types. Dissociation between kana and kanji were also observed. Anosognosia was evaluated by a self-rating scale with which the patients and the medical staff evaluated the patient's physical ability; the results indicated a large discrepancy between the evaluation by the patients and the medical staff. We emphasize that aphasic writing errors have been underestimated, particularly in ALS-D patients with impaired voluntary speech. We also reported that anosognosia was the most important and quantifiable symptom in ALS-D. The relationship between writing errors and anosognosia should be investigated further.http://dx.doi.org/10.1155/2008/814846
spellingShingle Hiroo Ichikawa
Shinichi Koyama
Hideki Ohno
Kenji Ishihara
Kiyomi Nagumo
Mitsuru Kawamura
Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
Behavioural Neurology
title Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
title_full Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
title_fullStr Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
title_full_unstemmed Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
title_short Writing Errors and Anosognosia in Amyotrophic Lateral Sclerosis with Dementia
title_sort writing errors and anosognosia in amyotrophic lateral sclerosis with dementia
url http://dx.doi.org/10.1155/2008/814846
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AT kenjiishihara writingerrorsandanosognosiainamyotrophiclateralsclerosiswithdementia
AT kiyominagumo writingerrorsandanosognosiainamyotrophiclateralsclerosiswithdementia
AT mitsurukawamura writingerrorsandanosognosiainamyotrophiclateralsclerosiswithdementia