認知機能障害を呈したALSの1例―書字障害を中心とした神経心理学的検討

Neuropsychological elucidation of writing errors in an amyotrophic lateral sclerosis case with cognitive impairment

　筆談中に書字障害が明らかになり，認知症を伴う筋萎縮性側索硬化症(amyotrophic lateral sclerosis with dementia：ALS-D)例を経験した．そこで，本症例の臨床症状について書字障害を中心に報告する．症例は63歳，女性，球型ALS．認知機能評価では，脱抑制的言動や保続などの前頭葉症状，理解力低下，考え不精，注意集中困難，自発性低下，意欲低下，病識欠如，立ち去り行動など前頭側頭型認知症の症状を認めた．書字評価では，錯書，ジャーゴン失書，脱字を認めた．近年，ALS-D例の報告が多数なされており，認知機能障害や言語機能障害の存在も念頭において評価を行う必要があると思われる．そして，ALS患者の認知機能障害の有無を評価するうえで，書字は重要な意義を有すると推察された．

　Although numerous reports of amyotrophic lateral sclerosis cases have focused on motor symptoms, the presence of cognitive and linguistic impairments should also receive attention during assessment. We report clinical observations and neuropsychological findings focused on the clinical symptoms of writing errors in a case of amyotrophic lateral sclerosis with dementia(ALS-D) whose writing impairment manifested during conversing through writing. Evaluation of the cognitive functions of a 63-year-old female bulbar-onset ALS patient revealed symptoms/signs of fronto-temporal dementia such as speech/behavioral disinhibitions with perseveration, impaired comprehension, lazy thinking, poor concentration, reduced motivation, anosognosia, low spontaneity, and running-away behavior. Writing evaluations demonstrated paragraphia, jargonagraphia, and omission of letters. In evaluating the absence/presence of cognitive impairment in ALS patients, an evaluation of writing seems to be of significant importance.