caa a22 4500 46576780X CHVBK 20180323111919.0 cr unu---uuuuu 170327e19901001xx s 000 0 eng 10.1007/BF00307636 doi (NATIONALLICENCE)springer-10.1007/BF00307636 An adult-onset case of sporadic motor neuron disease with basophilic inclusions [Elektronische Daten] [H. Kusaka, S. Matsumoto, T. Imai] Summary: A 36-year-old man developed motor neuron signs consisting of weakness and atrophy of the right upper limb, which progressed to involve the other limbs along with development of upper motor neuron signs including pseudobulbar palsy. He died 8.5 years after onset. Bilateral precentral gyri and putamina were grossly atrophic. In addition to severe degeneration of bilateral pyramidal tracts and marked neuronal cell loss of the precentral gyri and putamina, basophilic inclusions were widely distributed in the motor cortex, putamina, general somatic motor neurons such as the hypoglossal nucleus and spinal anterior horns, and other areas like the red nucleus and inferior olive. The inclusions were clearly shown with Nissl stain to be anilinophilic irregular masses with distinct rims. Ultrastructurally the inclusions appeared to consist of thick filamentous structures of 12-25 nm in diameter studded with electron-dense ribosomelike granules. Thick filamentous profiles were relatively short or occasionally fragmentary, haphazardly mingled with various amounts of granules and other organelles. No prominent accumulation of 10-nm neurofilaments or eosinophilic inclusions like Bunina bodies were found. The inclusions were indistinguishable from those reported in so-called "juvenile” amyotrophic lateral sclerosis. Springer-Verlag, 1990 Juvenile amyotrophic lateral sclerosis nationallicence Neurofibrillary tangles nationallicence Putamen nationallicence Motor cortex nationallicence Motor neuron disease nationallicence Kusaka H. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut Matsumoto S. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut Imai T. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut Acta Neuropathologica Springer-Verlag 80/6(1990-10-01), 660-665 0001-6322 80:6<660 1990 80 401 https://doi.org/10.1007/BF00307636 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00307636 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kusaka H. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut NATIONALLICENCE 700 1- Matsumoto S. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut NATIONALLICENCE 700 1- Imai T. Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 13-3, Kamiyama-cho, Kita-ku, 530, Osaka, Japan aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/6(1990-10-01), 660-665 0001-6322 80:6<660 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer