caa a22 4500 445808020 CHVBK 20180317145202.0 cr unu---uuuuu 170323e20110901xx s 000 0 eng 10.1007/s00415-011-5992-2 doi (NATIONALLICENCE)springer-10.1007/s00415-011-5992-2 Longitudinal study on MRI intensity changes of Machado-Joseph disease: correlation between MRI findings and neuropathological changes [Elektronische Daten] [Yoshihiko Horimoto, Mitsuhiro Matsumoto, Hiroyasu Akatsu, Akihiro Kojima, Mari Yoshida, Kazuya Nokura, Hiroyuki Yuasa, Eiichi Katada, Takayuki Yamamoto, Kenji Kosaka, Yoshio Hashizume, Hiroko Yamamoto, Shigehisa Mitake] To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI studies were repeated in all cases at different stages of the disease. Ages at the first MRI study ranged from 47 to 65years (55.2±7.1), with observation periods from 4.5 to 10.6years (6.95±2.48). We paid particular attention to two types of previously reported MRI findings detectable using T2-weighted images. One is located just outside the internal capsules, and another in the pons. A linear high-intensity change along the internal capsules was detected in all cases, and showed progression during the observation period. A comparison between MRI and autopsy findings suggested that the lesion might reflect degeneration with neuronal loss, astrocytosis, and gliosis in the internal segment of the globus pallidus. A cross-shaped high-intensity change in the pons was well advanced but still incomplete in all cases. In this region, pontine transverse fibers were atrophic, while longitudinal fibers remained intact. Pontine nuclei showed apparent nerve cell loss, and the remaining cells had many 1C2-positive intranuclear inclusions. Since these findings were detected both by lifetime images and by postmortem examination, MRI intensity changes could track the progression of neurodegeneration. Based on a comparison between MRI findings and neuropathology, the degeneration of an internal segment of the globus pallidus (one of the pathological features of MJD) had progressed following the initial symptoms. Springer-Verlag, 2011 Machado-Joseph disease (MJD) nationallicence Magnetic resonance imaging (MRI) nationallicence Neuropathology nationallicence Globus pallidus nationallicence Pons nationallicence Horimoto Yoshihiko Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Matsumoto Mitsuhiro Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Akatsu Hiroyasu Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Kojima Akihiro Department of Neurology, Toyokawa City Hospital, Toyokawa, Japan aut Yoshida Mari Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan aut Nokura Kazuya Department of Neurology, Fujita Health University, Toyoake, Japan aut Yuasa Hiroyuki Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Katada Eiichi Department of Neurology, Tsushima City Hospital, Tsushima, Japan aut Yamamoto Takayuki Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Kosaka Kenji Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Hashizume Yoshio Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut Yamamoto Hiroko Department of Neurology, Fujita Health University, Toyoake, Japan aut Mitake Shigehisa Department of Neurology, Tosei General Hospital, Seto, Japan aut Journal of Neurology Springer-Verlag 258/9(2011-09-01), 1657-1664 0340-5354 258:9<1657 2011 258 415 https://doi.org/10.1007/s00415-011-5992-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-5992-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Horimoto Yoshihiko Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Matsumoto Mitsuhiro Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Akatsu Hiroyasu Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Kojima Akihiro Department of Neurology, Toyokawa City Hospital, Toyokawa, Japan aut NATIONALLICENCE 700 1- Yoshida Mari Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan aut NATIONALLICENCE 700 1- Nokura Kazuya Department of Neurology, Fujita Health University, Toyoake, Japan aut NATIONALLICENCE 700 1- Yuasa Hiroyuki Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Katada Eiichi Department of Neurology, Tsushima City Hospital, Tsushima, Japan aut NATIONALLICENCE 700 1- Yamamoto Takayuki Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Kosaka Kenji Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Hashizume Yoshio Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan aut NATIONALLICENCE 700 1- Yamamoto Hiroko Department of Neurology, Fujita Health University, Toyoake, Japan aut NATIONALLICENCE 700 1- Mitake Shigehisa Department of Neurology, Tosei General Hospital, Seto, Japan aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/9(2011-09-01), 1657-1664 0340-5354 258:9<1657 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer