Affiliations: Department of Neurology, Showa University School of
Medicine 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan | Division of Clinical Electrophysiology, Department of
Neurology, University of Iowa, IA 52241, USA
Note: [] Corresponding author: Mitsuru Kawamura, M.D., Department of
Neurology, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku,
Tokyo 142-8666, Japan. Tel.: +81 3 3784 8781; Fax: +81 3 3784 1936; E-mail:
kawa@med.showa-u.ac.jp
Abstract: 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.
