Development of Changes in VEP and Associate Neuropathology on Creutzfeldt-Jakob Disease

A case, 74-year-old female, of Creutzfeldt-Jakob disease (CJD) was reported with a chronological changes of visual evoked potentials (VEPs) and neuropathological findings. The disease started with a disturbance of visual integration and developed to blindness, myoclonus, mental deterioration and akinetic mutism. A clinical diagnosis was made by a periodic synchronous discharge of EEG and other neurological specificity. Autopsy findings showed a peculiar spongy degeneration of the cerebral cortex. The flash VEP showed a loss of w-shaped wave and a marked delay of the peak latencies in the early stage. Subsequently, the once delayed latency was shortened with the advance of the illness in the middle stage and the N70-P100 amplitude became a huge triphasic wave like inversed ERG. Topographic distribution of the focus of huge component appeared on the left parieto-occipital region. At the terminal stage, the amplitude of huge component reduced. The mechanism of specific VEP changes in this case was interpreted to be due to the loss of generator due to spongy degeneration and the existence of cell fusion in the occipital cortex

全脳型Creutzfeldt-Jakob病の1剖検例 : 特にその眼球病変について

1年7か月の経過をとり,臨床的に全脳型Creutzfeld-Jakob病と考えられていた症例を剖検した.脳は重量が695gで,び漫性に萎縮していた.眼球には肉眼的には変化はなかった.病理組織学的には大脳,小脳皮質,基底核,視床などに海綿状態,神経細胞の変性と脱落,原形質性アストロサイトの増殖,脂肪顆粒細胞の出現を認めた.大脳白質から中脳被蓋,橋にかけては広範に変性し,原形質性アストロサイトの増殖,脂肪顆粒細胞の出現を認めた.網膜では周辺網膜の外顆粒層に細胞脱落と空胞化,外網状層の変性,神経節細胞の変性と脱落,神経線維層の粗鬆化がみられた.本例の白質病変は皮質と同様の基質の変化,アストロサイトの増殖,脂肪顆粒細胞の出現などがみられたことより一次性変化と思われた.網膜の細胞脱落,空胞化,基質の粗鬆化なども大脳の変化と同様と思われ,網膜病変は一次性変化と考えられた.A case of the panencephalopathic type of Creutzfeldt-Jakob disease was reported. The patient was a 59-year-old female, who was admitted in July 1982 because of gait disturbance, hallucination, left hemiparesis and Gegenhalten of the left upper extremity. In September 1982 she developed akinetic mutism which lasted until her terminal stage. She died in February 1984 after a course of one year and seven months. The brain weighed 695 g and showed diffuse atrophy. Microscopically, there was marked atrophy and loss of neurons, and proliferations of protoplasmic astrocytes throughout the cerebral and cerebellar cortex, basal ganglia and thalamus. The cerebral white matter, tegmentum of the midbrain, pons, optic nerves, optic chiasm and optic tracts showed diffuse destruction of myelins and axons with fat granule cells and astrocytic proliferations. Loss of the outer nuclear layer of the retina was more prominent in the peripheral area than in the central area. The outer plexiform layer showed a remarkable vacuolization especially in the posterior region. Retinal ganglion cells showed degenerative changes and occasional loss. In this case, white matter involvements and ocular lesions were thought to be the primary degenerative changes of Creutzfeldt-Jakob disease

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Catalytic Hydrogenation of Benzonitrile by Triruthenium Clusters: Consecutive Transformations of Benzonitrile on the Face of a Ru₃ Plane

Reactions of the triruthenium clusters {Cp*Ru­(μ-H)}₃(μ₃-H)₂ (<b>1</b>) and (Cp*Ru)₃(μ₃-H)­(μ-H)₂(μ-CO) (<b>12</b>) (Cp* = η⁵-C₅Me₅) with benzonitrile were investigated in relation to the selective hydrogenation of benzonitrile to benzylamine. Benzonitrile undergoes consecutive transformations into μ₃-η²:η²(⊥)-nitrile, μ₃-η²:η²(⊥)-imidoyl, μ₃-η²(∥)-imidoyl, μ₃-η²-alkylideneamido, μ₃-imido, and μ-amido ligands on the Ru₃ plane accompanied by the uptake of dihydrogen. The reactions are analogues of nitrile hydrogenation on a metal surface. The complexes are structural models of chemisorbed species and catalyze the hydrogenation of benzonitrile. Complex <b>1</b> catalyzes benzonitrile hydrogenation without additives but exhibits only moderate selectivity toward benzylamine. Although the μ₃-benzylimido complex {Cp*Ru­(μ-H)}₃(μ₃-NCH₂Ph) (<b>4</b>) was obtained by reaction of <b>1</b> with benzonitrile, it was readily transformed into to the μ₃-imidoyl complexes (Cp*Ru)₃(μ-H)₂{μ₃-η²:η²(⊥)-PhCNH} (<b>3</b>) and (Cp*Ru)₃(H)₄{μ₃-η²(∥∥)-PhCNH} (<b>4</b>), which are key intermediates in secondary imine formation. Two benzonitrile molecules were incorporated on the Ru₃ plane under the reaction conditions, which decreases the selectivity of primary amine formation. In contrast, μ-carbonyl complex <b>12</b> suppresses the incorporation of additional benzonitrile ligands and the formation of μ₃-imidoyl species due to the presence of CO. These features of <b>12</b> bring about significant improvement in the selectivity of benzonitrile hydrogenation and produce benzylamine in 92% yield

Catalytic Hydrogenation of Benzonitrile by Triruthenium Clusters: Consecutive Transformations of Benzonitrile on the Face of a Ru₃ Plane

Reactions of the triruthenium clusters {Cp*Ru­(μ-H)}₃(μ₃-H)₂ (<b>1</b>) and (Cp*Ru)₃(μ₃-H)­(μ-H)₂(μ-CO) (<b>12</b>) (Cp* = η⁵-C₅Me₅) with benzonitrile were investigated in relation to the selective hydrogenation of benzonitrile to benzylamine. Benzonitrile undergoes consecutive transformations into μ₃-η²:η²(⊥)-nitrile, μ₃-η²:η²(⊥)-imidoyl, μ₃-η²(∥)-imidoyl, μ₃-η²-alkylideneamido, μ₃-imido, and μ-amido ligands on the Ru₃ plane accompanied by the uptake of dihydrogen. The reactions are analogues of nitrile hydrogenation on a metal surface. The complexes are structural models of chemisorbed species and catalyze the hydrogenation of benzonitrile. Complex <b>1</b> catalyzes benzonitrile hydrogenation without additives but exhibits only moderate selectivity toward benzylamine. Although the μ₃-benzylimido complex {Cp*Ru­(μ-H)}₃(μ₃-NCH₂Ph) (<b>4</b>) was obtained by reaction of <b>1</b> with benzonitrile, it was readily transformed into to the μ₃-imidoyl complexes (Cp*Ru)₃(μ-H)₂{μ₃-η²:η²(⊥)-PhCNH} (<b>3</b>) and (Cp*Ru)₃(H)₄{μ₃-η²(∥∥)-PhCNH} (<b>4</b>), which are key intermediates in secondary imine formation. Two benzonitrile molecules were incorporated on the Ru₃ plane under the reaction conditions, which decreases the selectivity of primary amine formation. In contrast, μ-carbonyl complex <b>12</b> suppresses the incorporation of additional benzonitrile ligands and the formation of μ₃-imidoyl species due to the presence of CO. These features of <b>12</b> bring about significant improvement in the selectivity of benzonitrile hydrogenation and produce benzylamine in 92% yield