Amyotrophic lateral sclerosis with dementia: neuropsychological aspects

Mitsuru Kawamura and Hiroo Ichikawa

in Amyotrophic Lateral Sclerosis and the Frontotemporal Dementias

Published on behalf of Oxford University Press

Published in print October 2012 | ISBN: 9780199590674
Published online November 2012 | e-ISBN: 9780191753466 | DOI:
Amyotrophic lateral sclerosis with dementia: neuropsychological aspects

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Previously, the most distinctive feature of amyotrophic lateral sclerosis (ALS) was considered to be the retention of full consciousness until the very end of the patient's life, without development of psychiatric or cognitive (dementia) symptoms. However, in 1964, Yuasa reported clinical cases of ALS manifesting dementia, indicating that dementia and ALS could have a single pathogenic cause. Since then, especially in Japan, case reports citing instances of ALS with dementia (ALS-D) have been accumulating. Mitsuyama and colleagues have advocated that ALS-D be distinguished an independent disease entity although some still consider ALS-D to be a subclass of classic ALS. Furthermore, in terms of dementia, ALS-D is commonly considered to be a subclass of frontotemporal dementia (FTD).

Here, we review previously published case reports and describe the key neuropsychological points related to ALS-D as well as the relationship between ALS-D and FTD. Specifically, we will consider the following topics: (a) patients with ALS-D show symptoms of frontal-type dementia consistent with FTD); (b) ALS-D can be divided into two categories in terms of its development (dementia preceding ALS-D and ALS preceding ALS-D) and symptoms (bulbar palsy-type and upper-extremity type ALS); (c) computed tomography (CT) and magnetic resonance imaging images show frontal and anterior temporal lobe atrophy in ALS-D, and single-photon emission CT (SPECT) images typically show decreased uptake in the frontal and temporal lobes, which is detectable earlier than morphological changes, and thus, SPECT is an important diagnostic tool for diagnosis of ALS-D; and (d) typical dementia symptoms include paragraphia and anosognosia.

In addition, a case report written by Watanabe in 1893 is presented. This is the first case report on aphasia published in Japan, and may be the world's first case report on ALS-D.

Bulbar palsy is commonly found in patients with ALS-D, and disordered speech is usually prominent in the early stages of the disease; thus, it is not easy to evaluate higher brain function in patients with ALS-D. There have been some cases of ALS in which the patient was first noticed to have an impaired ability to write (dysgraphia) through evaluation of written communication after the disappearance of spontaneous speech. Additionally, lack of insight has been described as a core symptom of FTD and we have noted that anosognosia is similarly an important feature of ALS-D. However, the objectivity of such findings was not investigated in the previously published report on anosognosia in patients with ALS-D.

In consideration of the problems related to the evaluation of ALS-D, we analysed the clinical features of ALS-D based on a comprehensive review of the cases we have encountered previously, with a focus on analysis of dysgraphia. In addition, we attempted to quantify the degree of anosognosia by using a Japanese version of the anosognosia questionnaire devised by Deckel and Morrison.

Chapter.  6541 words.  Illustrated.

Subjects: Neurology

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