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【抄録】記憶障害と健忘失語または語義失語様の言語障害とともに,活発な幻聴と嫉妬妄想および被害妄想を認めた初老期痴呆の1臨床例について報告する。人格的には浅薄で深刻味に欠けるものの礼容は保たれ,感情的接触も良好で,視空間認知も含めて構成障害以外の失行・失認といった巣症状を示さず,ADLはほぼ保たれていた。画像上は両側側頭葉・前頭葉の限局性脳萎縮とFahr病類似の大脳基底核・小脳歯状核の石灰化を認めた。これらの臨床的特徴は小阪が提唱した「石灰沈着を伴うびまん性神経原線維変化病diffuse neurofibrillary tangles with calcification(DNTC)」と一致するものと考える。
A 67 year-old woman with slowly progressing dementia and psychotic symptoms is reported. At the age of 62, she developed auditory hallucination and delusion of persecution followed by memory disturbance, amnestic aphasia and difficulty in understanding. She had no parietooccital focal signs except for mild constructional apraxia. Bilateral frontotemporal lobar atrophy and intracranial Fahr-type calcifications were detected in brain CT-scan and MRI. These clinical features correspond to those of “diffuse neurofibrillary tangles with calcification”.
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