第51回日本神経学会総会
＜シンポジウム19―2＞認知症研究の新しい視点
ALS/FTLD と言語障害
市川 博雄
昭和大学内科学講座神経内科学部門〔〒142―8666 東京都品川区旗の台1―5―8〕
In the 1990s, the concept of frontotemporal lobar degeneration (FTLD) was proposed as a clinicopathological entity preferentially involving the frontotemporal lobes, and recent pathological findings have suggested a linkage of FTLD with ALS. FTLD includes frontotemporal dementia (FTD), reflecting behavioral changes, and progressive non-fluent aphasia (PNFA) and semantic dementia (SD) as language disorders. Although language problems appear to have become of interest relatively recently, an historical Japanese account (1893) written by Watanabe described aphasia in bulbar-onset ALS. This report is entitled "A patient who manifested cortical motor aphasia concurrently with bulbar palsy and progressive muscular atrophy", and is the first case report of aphasia published in Japan. Thus, language problems in ALS may have been overlooked for many years. We have reported that ALS patients frequently show omission and paragraphia of kana letters, and syntactic errors in writing, and that these observations differ across patients. We also showed double dissociation between errors in kana and kanji characters in some patients, with preferential involvement of the frontotemporal lobes and frontal- and temporal-lobe predominance for kana and kanji, respectively. We also showed that the writing errors can appear as agraphia without aphasia.
Full Text of this Article in Japanese PDF (365K)
(臨床神経, 50：1014−1016, 2010)
key words：筋萎縮性側索硬化症，前頭側頭葉変性症，言語障害，書字障害
（受付日：2010年5月22日）
