認知症を伴う筋萎縮性側索硬化症1例の神経心理症状に関する検討

Neuropsychological state of amyotrophic lateral sclerosis with dementia: A case study

　認知症を伴う筋萎縮性側索硬化症(amyotrophic lateral sclerosis with dementia：ALS-D)は，近年，独立した疾患単位として認められる傾向にある．本研究では，ALS-D1例の神経心理症状について検討した．本症例は上肢の症状とともに球麻痺症状が発現し進行する混合型ALSで，運動性の要素の強い失語症状を呈していた．認知症状については，前頭側頭型認知症(fronto-temporal dementia：FTD)の臨床像を呈していた．脱抑制型，無欲型，常同型のすべての要素を有したが，常同的異常行動が顕著であった．また，本症例は，ジャーゴン失書を特徴的に呈していた．こうした常同的異常行動およびジャーゴン失書は，ALS-Dに関する先行報告例では稀な症状として着目される．

　There is an ongoing tendency to recognize amyotrophic lateral sclerosis with dementia(ALS-D) as an independent disorder in recent years. In this study, we investigated the neuropsychological state of a case with ALS-D. The present case indicated bulbar palsy with accompanying signs of the upper extremities, manifesting a typical mixed-type ALS. The pathological condition of the patient was further complicated with motor aphasia. The cognitive status portrayed a clinical image characteristic of fronto-temporal dementia(FTD), displaying obvious disinhibition, apathetic and stereotypic elements, but most markedly, stereotypic abnormal behavior. Furthermore, the present case also manifested the characteristic features of jargon agraphia. Stereotypic abnormal behavior and jargon agraphia as found in our case have rarely been reported in previous studies on ALS-D patients and deserve attention.