The Japanese journal of neuropsychology

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ArticleTitle	Language impairment in amyotrophic lateral sclerosis
Language	J
AuthorList	Hiroo Ichikawa
Affiliation	Department of Neurology, Showa University Fujigaoka Hospital
Publication	Japanese Journal of Neuropsychology: 28 (3), 207-214, 2012
Received
Accepted
Abstract	Amyotrophic lateral sclerosis (ALS), a representative motor neuron disease (MND), has long been thought of as a neurodegenerative disorder with selective involvement of the motor neuron system. However, it has recently been established that ALS or MND (ALS/MND) is a multisystem disorder involving both the motor system and cognitive factors that usually present with a feature of frontotemporal lobar degeneration (FTLD). Frontotemporal dementia (FTD), a behavioral variant of FTLD, is a typical cognitive impairment in ALS/MND, but language variants including progressive non-fluent aphasia (PA) and semantic dementia (SD) have recently been reported in connection with ALS/MND. However, there is a prescient Japanese account (1893) describing aphasia with bulbar-onset ALS/MND that was described about 100 years before introduction of the concept of FTLD. Thus, language-related problems in ALS may have been overlooked because aphasic problems are difficult to evaluate in ALS patients due to progressive bulbar or pseudo-bulbar palsy resulting in dysarthria. Herein, to connect historical descriptions and recent reports, the following issues are further discussed: 1) the possibility that language problems have been overlooked in ALS/MND, 2) the historical Japanese literature on language problems in ALS/MND, 3) language problems in ALS/MND and FTLD subtypes, 4) noun versus verb dissociation in ALS/MND in alphabetical languages, 5) the characteristics and significance of writing errors in ALS/MND, 6) kana versus kanji dissociation in ALS/MND in Japanese, and 7) isolated agraphia and the additional significance of writing errors in ALS/MND.
Keywords	amyotrophic lateral sclerosis, aphasia, writing errors, kana, kanji