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Open Access Research article

Neuropsychological study of amyotrophic lateral sclerosis and parkinsonism-dementia complex in Kii peninsula, Japan

Akihiro Shindo1, Yukito Ueda2, Shigeki Kuzuhara3 and Yasumasa Kokubo4*

Author Affiliations

1 Department of Neurology, Mie University Graduate School of Medicine, Tsu, Mie, Japan

2 Department of Rehabilitation, Mie University Hospital, Tsu, Mie, Japan

3 Department of Medical Welfare, Faculty of Health Science, Suzuka University of Medical Science, Suzuka, Mie, Japan

4 Kii ALS/PDC Research Center, Mie University, Graduate School of Regional Innovation Studies, Mie 514-8507, Japan

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BMC Neurology 2014, 14:151  doi:10.1186/1471-2377-14-151

Published: 21 July 2014

Abstract

Background

The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC).

Methods

The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer’s disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies. These patients and their carer/spouse were asked to report any history of abulia-apathy, hallucination, personality change and other variety of symptoms. Patients also underwent brain magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and neuropsychological tests comprising the Mini Mental State Examination, Raven’s Colored Progressive Matrices, verbal fluency, and Paired-Associate Word Learning Test and some of them were assessed with the Frontal Assessment Battery (FAB).

Results

All patients with Kii ALS/PDC had cognitive dysfunction including abulia-apathy, bradyphrenia, hallucination, decrease of extraversion, disorientation, and delayed reaction time. Brain MRI showed atrophy of the frontal and/or temporal lobes, and SPECT revealed a decrease in cerebral blood flow of the frontal and/or temporal lobes in all patients with Kii ALS/PDC. Disorientation, difficulty in word recall, delayed reaction time, and low FAB score were recognized in Kii ALS/PDC patients with cognitive dysfunction.

Conclusions

The core neuropsychological features of the patients with Kii ALS/PDC were characterized by marked abulia-apathy, bradyphrenia, and hallucination.

Keywords:
Abulia; Apathy; Dementia; Amyotrophic lateral sclerosis; Parkinsonism–dementia complex