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要旨 今回,Dementia with Lewy bodies(DLB)とargyrophilic grain dementia(AGD)の両者にあてはまる症例を経験したので報告する。死亡時75歳の女性。臨床的にはパーキンソニズム(小刻み歩行,筋固縮など)と痴呆が目立ち,また精神的不穏状態(幻覚(幻視),被害関係念慮,夜間譫妄など)が著明であった。悪性リンパ腫により,発症後1年5カ月後に死亡した。脳重1,153g(固定前),肉眼的に側脳室の拡大は前角が中等度,下角で軽度,中脳黒質・青斑核は着色不良の所見を認めた。光顕所見では大脳皮質で多数のargyrophilic grain(AG)とLewy body(LB)を認め,扁桃核にballooned neuronsや海綿状変化やAGを認めた。Meynert基底核,動眼神経核,黒質,青斑核,迷走神経背側核にLBを認めた。DLB international workshopの病理診断基準によるとDLBのneocortical typeとなる。大脳皮質から扁桃核にかけて多数のAGを認め,ballooned neuronsを扁桃核などで認めることからAGDの病理所見にもあてはまる。臨床病理診断としては,臨床像を重視し「多数のAGの出現したDLB」と考えた。AGDは多くの痴呆性疾患に合併するとされ,その臨床像について十分なコンセンサスは得られていない。症例の蓄積が必要と考え今回報告した。
We report an autopsy case showing neuropathologically abundant Lewy bodies and argyrophilic grains.
A Japanese woman without hereditary burden developed parkinsonian gait at the age of 74, following by insomnia, memory disturbance, delirium, resting tremor, rigidity, and retropulsion. About 8 months later, a visual hallucination, concerning small worms, children, and so on,became obvious. About 16 months later, malignant lymphoma was detected. About 17 months later, she was died of pneumonia. The total duration of illness was approximately one year and five months.
The weight of the brain was 1,153g before fixation. Depigmentation of the substantia nigra and locus ceruleus was prominent. Many argyrophilic grains were seen in the temporal lobe (T3, T4), amygdala, and hippocampal CA1. Some ballooned neurons were found in the amygdala. Many Lewy bodies were encountered in the transentorhinal region and cingulated gyrus. A few Lewy bodies were seen in the temporal, frontal, and parietal lobes. In this case, neuropathological examination is compatible for dementia of Lewy bodies and argyrophilic grain dementia, and clinical course is consistent with dementia of Lewy bodies. This report may contribute to the elucidation of the clinicopathological hallmarks of argyrophilic grain dementia and dementia with Lewy bodies.
(Received : October 19, 2004)
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