Link To International Asidan Symposium 2016 page

In English 简体中文


Department of Neurology and Neuroscience at Okayama University School of Medicine



Welcome to our home page of Department of Neurology and Neuroscience at Okayama University School of Medicine. Our department is currently tackling 3 major neurological projects. The first is experimental gene therapy and stem cell therapy for ischemic stroke with animal models. After we worldly first developed neuroprotective drug therapy for stroke patients with edaravone, we are developing the second generation-neuroprotectives for stroke patients. Thus we are going to apply these therapies to human patients in the near future. The second project is molecular analysis of human neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and spinocerebellar ataxia. After we found gene mutations in such diseases, we currently analyzing transgenic animal models and also performing gene and stem cell therapy for such diseases. The third project is more clinical neurophysiological study of cerebrovascular disease, dementia, neurodegenerative disorders, and diabetes mellitus. This project is much more clinical with using MRI, CT scan, photic CT, ultrasonic echography, nerve conduction study, photic topography, and MEG (magnetoenchephalogram) etc.
Because the number of the above neurological diseases are emerging quickly not only in the developed countries but also in the developing countries, our basic and clinical research will definitely provide strong impact in these fields. If you are interested in any of our project, let you have contact with me. Because our department is always hosting several international fellows, I will always welcome young people from all over the world.

Koji Abe, MD, PhD

<Short CV of Professor Koji Abe>
1981 graduated Tohoku University School of Medicine, Japan (MD)
1987 graduated Graduate School of Medicine of Tohoku U. (PhD)
1988 - 1990 Research Fellow at the Harvard Medical School (Boston, USA)
1990 Assistant Professor of Department of Neurology at Tohoku U.
1996 Associate Professor of Department of Neurology at Tohoku U.
1998 Professor and Chairman of Neurology at Okayama U. Medical School
2002-2003 Vice President of Okayama U. Hospital
2007  Chairman of 23rd International Meeting of Cerebral Blood Flow and Metabolism
2009-2011 President of International Society of Cerebral Blood Flow and Metabolism 




Detection of Asidan


Among autosomal dominant inheritance of spinocerebellar atrophy (SCA) in Chugoku area in Japan, SCA6 and DRPLA is major (Figure 1). However, we reported the unique SCA cases accompanied by involvement of motor neuron systems (, 2).
Collaborative research with Kyoto University (professor Akio Koizumi) has revealed the causative gene (spinocerebellar ataxia type 36, nicknamed “Asidan”) in a new disease that has symptoms like those of amyotrophic lateral sclerosis (ALS) and spinocerebellar degeneration (SCD), which are known intractable neurological diseases. The study found that this novel disease is caused by a hexanucleotide repeat in the NOP56 gene on chromosome 20 expanding to approximately 1,500–2,500 repeats. These results were published in the American Journal of Human Genetics in 2011 (Figure 2, Article 3).

The clinical features of this disease in 8 families were reported in Neurology in 2012, describing the disease as exhibiting progressive motor neuron symptoms of the tongue and limbs with relatively pure cerebellar ataxia (Figure 3,4, Article 4). Further reports to date have also indicated that cognitive dysfunction, hearing loss (Figure 5), and dysphagia also developed (Articles 5–7).

Furthermore, the progressive loss of NOP56 protein in the spinal cord of ALS model mice (Article 8) and the giant RNA foci in the neurons of Asidan patients were reported (Article 9).
Asidan can be a crossroad disease of SCA and ALS in the point of both clinical and genetic features (Figure 7, 8).

In future, we hope to clarify the pathological mechanism in more detail by differentiating patient-derived iPS cells into neurons and reproducing the disease on a cellular level.


Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7
Figure 8
Article 1 Manabe Y, Shiro Y, Takahashi K, Kashihara K, Abe K (2000) A case of spinocerebellar ataxia accompanied by severe involvement of the motor neuron system. Neuro Res 22:567-70.
Article 2 Ohta Y, Hayashi T, Nagai M, Okamoto M, Nagotani S, Nagano I, Ohmori N, Takehisa Y, Murakami T, Shoji M, Kamiya T, Abe K (2007) Two cases of spinocerebellar ataxia accompanied by involvement of the skeletal motor neuron system and bulbar palsy. Intern Med 46:751-5.
Article 3 Kobayashi H, Abe K, Matsuura T, Ikeda Y, Hitomi T, Akechi Y, Habu T, Liu W, Okuda H, Koizumi A (2011) Expansion of intronic GGCCTG hexanucleotide repeat in NOP56 causes SCA36, a type of spinocerebellar ataxia accompanied by motor neuron involvement. Am J Hum Genet 89:121-30.
Article 4Ikeda Y, Ohta Y, Kobayashi H, Okamoto M, Takamatsu K, Ota T, Manabe Y, Okamoto K, Koizumi A, Abe K (2012) Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan). Neurology 79:333-41.
Article 5Abe K, Ikeda Y, Kurata T, Ohta Y, Manabe Y, Okamoto M, Takamatsu K, Ohta T, Takao Y, Shiro Y, Shoji M, Kamiya T, Kobayashi H, Koizumi A. (2012) Cognitive and affective impairments of a novel SCA/MND crossroad mutation Asidan. Eur J Neurol 19:1070-1078.
Article 6Ikeda Y, Ohta Y, Kurata T, Shiro Y, Takao Y, Abe K. (2012) Acoustic impairment is a distinguishable clinical feature of Asidan/SCA36. J Neurol Sci 324:109-112.
Article 7Morimoto N, Yamashita T, Sato K, Kurata T, Ikeda Y, Kusuhara T, Murata N, Abe K. (2012) Assessment of swallowing in motor neuron disease and Asidan/SCA36 patients with new methods. J Neurol Sci 324:149-155.
Article 8 Miyazaki K, Yamashita T, Morimoto N, Sato K, Mimoto T, Kurata T, Ikeda Y, Abe K. (2013) Early and selective reduction of NOP56 (Asidan) and RNA processing proteins in the motor neuron of ALS model mice. Neurol Res 35:744-754.
Article 9Liu W, Ikeda Y, Hishikawa N, Yamashita T, Deguchi K, Abe K. (2014) Characteristic RNA foci of the abnormal hexanucleotide GGCCUG repeat expansion in spinocerebellar ataxia type 36 (Asidan). Eur J Neurol 2.



Link To International Asidan Symposium 2016 page

In English 简体中文



International Asidan Symposium on Asida River/Japan


Date: July 7-8, 2016
Venue: Hotel Ofutei
136 Tomonoura, Fukuyama, Hiroshima
(TEL: 084-982-1123)
http://www.ofutei.com/english/index.html

(*) Oral presentation on SCA, ALS and
dementia will be greatly welcome !
Access: 30 min by bus from JR Fukuyama Shinkansen Superexpress train station, and walk 5 min from the Tomonoura bus stop). Shuttle bus service is available for pick-up and drop-off between the hotel and Fukuyama railway station.

Tentative Program






Abe’s BPSD Score (ABS)


We created a new simple score of behavioral and psychological symptoms of dementia (BPSD) (Abe’s BPSD Score = ABS).(Click to download)
Please use this score freely for your clinical works. The patent is free.
If you present your works by articles, slides or posters using this score, we would like to ask you to cite the below article.

You can refer the following abstract and papers:


Abe K, Kurata T, Deguchi K, Kono S, Sato K, Omote Y, Matsuzono K, Yamashita T, Ikeda Y et al.A new simple score (ABS) for screening behavioral and psychological symptoms of dementia. 21th European Congress of Psychiatry (EPA2013) in Nice, France

Abe K, Yamashita T, Hishikawa N, Ohta Y, Deguchi K, Sato K, Matsuzono K, Nakano Y, Ikeda Y, Wakutani Y, Takao Y. A new simple score (ABS) for assessing behavioral and psychological symptoms of dementia. J Neurol Sci. (2015) 350:14-17.

Koji Abe, MD, PhD
Department of Neurology
Okayama University Medical School
2-5-1 Shikatacho, Okayama, 700-8558
Japan




What is ABS


Neuropsychiatric Inventory (NPI) scores are often used to evaluate the psychological symptoms of dementia.
Abe’s BPSD Score (ABS) (Figure 1) shows good correlation with the NPI scores (Figure 2) and the decreased scores of MMSE due to aging (Figure 3).
Furthermore, the evaluation of ABS is done quickly compared to NPI scores (Figure 4). The ABS shows the high inter-rater reliability and the smaller score in secondary than main caregivers (Figure 5).
In conclusion, ABS provides a new simple and quick test for BPSD assessment with a shorter time. The ABS is useful for evaluating BPSD for mild to moderate dementia patients.






Ice Bucket Challenge by Koji Abe






Amyotrophic lateral sclerosis (ALS) information


The 3rd phase clinical trial of MCI-186 for ALS patients


The 3rd phase (2nd round) clinical trial of MCI-186 (Edaravone (Radicut)) for ALS patients, which had been started from July 2006 in Japan (Okayama University was the center hospital of this clinical trial), finished recently.
  The results of 1st round of this clinical trial showed the efficacy of Edaravone for ALS patints. Professor Abe reported the results as a first author of the below paper.
  The paper presented the safety of Edaravone and the efficacy in the pinch strength of ALS patients
  The results of 2nd round of this clinical trial have been analyzed by professor Abe et al. and will be published soon.
Abe K, Itoyama Y, Sobue G, Tsuji S, Aoki M, Doyu M, Hamada C, Kondo K, Yoneoka T, Akimoto M, Yoshino H. Confirmatory double-blind, parallel-group, placebo-controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener. 2014; 6:1-8.









The 2nd Asian Clinical Congress (ACC2) in Kyoto


We thank all of you for attending ACC2 in Kyoto at April 3-5, 2014. Because of your great contributions, our ACC2 successfully finished with making many Asian friends. Our photos are now uploaded online.

How to save pictures
   Right-click on the thumbnails below, and then select "Save Link As..." in the context menu.
   If you fail to see the photos, please contact us by email (shinnai@cc.okayama-u.ac.jp)

The same picutres can see also this site.





Overview of the Department of Neurology, Okayama University


Yasuuki Ohta, Senior member of staff


The young and attractive Department of Neurology, Okayama University, is in its 20th year of operation, and 16th year since Prof. Koji Abe assumed the position of director. There are 7 teaching staff members working under Prof. Abe, as well as 7 medical staff and 10 postgraduates and researchers working enthusiastically on medical care, education, and research. Including fellow students, young doctors undergoing training in internal medicine and neurology off campus, as well as doctors newly appointed as department and section heads at affiliated hospitals off campus, there is a total of 80 individuals involved in the laboratory. Our energy has long since been focused on student education and medical training, and as of 2004, postgraduate clinical training of doctors became compulsory. To achieve our goal of having ‘all doctors acquire a wide range of basic clinical skills,’ which is now required, we established an improved training program with the most appropriate training in the department of neurology that enables the patient to be thoroughly examined. The laboratory members work happily together every day to foster highly skilled neurologists and physicians.
Patients from the Chugoku, Shikoku, Kansai, Kyushu, and the more distantly located Kanto and Tokai regions are referred to the Department of Neurology at Okayama University hospital as general new outpatients of Prof. Abe. New patients are mainly taken care of by Prof. Abe and his staffs. Returning patients are received through the special outpatient system with stroke patients appointed to Dr. Deguchi, neurodegenerative diseases to Dr. Yamashita, dementia cases to Dr. Kurata and Dr. Hishikawa, Parkinson’s disease cases to Dr. Hatanaka, and neuroimmune diseases to Dr. Kawahara, who provide advanced medical care to the outpatients. At in-patients ward, many attending physicians play an active role under Dr. Kurata, the Department of Neurology ward’s medical director. The number of outpatients and inpatients per year in the Department of Neurology is rapidly increasing, and we have felt an increasing social demand for the Department of Neurology. The number of patients referred from affiliated hospitals within and outside of Okayama prefecture has also increased and cooperation between the departments of internal medicine, neurology, and brain surgery of regional medical institutions has grown increasingly closer.
Research activities are being actively expanded in addition to medical care and educational initiatives. The research system is divided into 3 groups of diseases with the most patients and highest social demand, namely stroke, neurodegenerative disease, and dementia, for which laboratory members happily engage in research every day. Many our laboratory members have the opportunity for studying abroad, including Harvard and Stanford University in the USA, Toronto University in Canada, and CR CHU in Quebec for international research activities and there is a great deal of collaborative research underway. In 2007, for the first time in 14 years, Japan (Osaka) held the 23rd International Symposium on Cerebral Blood Flow and Metabolism (world’s largest brain science-related conference) with Prof. Abe from our laboratory as the chairperson. The event was a great success with the most participants that have ever attended this symposium. Further, in 2009, Prof. Abe was appointed president of the International Society of Cerebral Blood Flow and Metabolism, and in Fall 2010, the first World Forum on Cerebral Blood Flow and Metabolism was hosted in Kyoto. In 2011, the 25th International Symposium on Cerebral Blood Flow and Metabolism was held in Barcelona, Spain. The 26th Japanese Symposium on Cerebral Blood Flow and Metabolism is scheduled for November 2014. These events have enabled the Department of Neurology at Okayama University to contribute to developments in neuroscience, and thus gain international recognition as a laboratory. In the 16 years that Prof. Abe has been in his position, 4 new professors in neurology at national universities have emerged, namely Dr. Mikio Shoji at Hirosaki University in 2006, and Dr. Tohru Matsuura at Jichi Medical University, Dr. Yoshio Ikeda at Gunma University, and Dr. Etsuro Matsubara at Oita University in 2013. Therefore, our laboratory is a place where medical staff members work together not only for Japan, but for the whole world.




Our Recent Scientific Papers


A prion disease causing peripheral neuropaghy


While human prion diseases have a wide range of clinicopathological phenotypes, they generally present as neurological disorders with acute multifocal degeneration of the central nervous system or neuropsychiatric disorders, most of which are include dementia and cerebellar ataxia.
We discovered a novel prion disease that causes pan-autonomic-sensory neuropathy, and found that the disease was caused by 2-bp deletion (CT) in the 178th prion protein codon. Prion protein deposits were found in the peripheral nerves of patients with this disease. These results were published in the European Journal of Neurology in 2013 (article. 1).
Recently, an English research group reported in the New England Journal of Medicine in 2013 that a prion disease causing very similar clinical symptoms (diarrhea and autonomic neuropathy) brought about by truncation mutation of the 163rd prion protein codon (ref. 1), and this similarity has gained much attention.
Matsuzono K, Ikeda Y, Liu W, Kurata T, Deguchi S, Deguchi K, Abe K (2013) A novel familial prion disease causing pan-autonomic-sensory neuropathy and cognitive impairment. Eur J Neurol 20:e67-9.
Reference 1 Mead S, Gandhi S, Beck J, Caine D, Gallujipali D, Carswell C, Hyare H, Joiner S, Ayling H, Lashley T, Linehan JM, Al-Doujaily H, Sharps B, Revesz T, Sandberg MK, Reilly MM, Koltzenburg M, Forbes A, Rudge P, Brandner S, Warren JD, Wadsworth JD, Wood NW, Holton JL, Collinge J (2013) A novel prion disease associated with diarrhea and autonomic neuropathy. N Engl J Med 369:1904-14.

In vivo imaging of cerebral infarction by optical imaging


Cerebral infarction is generally diagnosed using findings from magnetic resonance imaging and computed tomography; however, the development of new technology for diagnostic imaging is needed to enable early diagnosis and to determine treatment outcomes.
Our laboratory created a cerebral infarction model in LC3-GFP transgenic mice and took images of the mice brains in vivo with the scalp removed using a special high sensitivity camera. Autophagy was strongly induced in mice brains in an ischemic state and the emission of light was observed (figure below). These results were published in Autophagy in 2010 ().
Future developments in luminescent drugs and test equipment may enable for clinical application simpler and more sensitive detection of cerebral infarction lesions than current techniques.
Tian F, Deguchi K, Yamashita T, Ohta Y, Morimoto N, Shang J, Zhang X, Liu N, Ikeda Y, Matsuura T, Abe K (2010) In vivo imaging of autophagy in a mouse stroke model. Autophagy 6:1107-14.





Messages from Neurology Researchars and Residents


Fulfilling Life in Graduate School

Kazunori Miyazaki


After graduating agricultural department of Ehime University, I got interested in a neurodegenerating disease and entered the master's course of department of neurology of Okayama University graduate school. Furthermore, I would like to continue the study of neuroscience, and decided to enter the doctoral course.
  It was very glad that I wrote 2 English papers during the master's course. Also during a doctoral course, from scientific research to my own daily life, I have received many supports from the doctors and fellow in our laboratory. It encouraged me to continue research of ALS which is also a pronoun of a nerve incurable disease.
  In our laboratory, there are many doctors, of course Professor Koji Abe, working in cutting-edge research areas and having many research performances and experiences. Moreover, since I could have many opportunities of presentations in scientific conferences and submission papers, I learned many essential elements required for a researcher. As a result, I already have submitted 6 original articles in English journals below, and I have been able to spend a productive graduate student life so far. From now on, I’m preparing to submit two more papers in English.
  I think that the scientific and logical thinking, and the research technique, which could be obtained during 6 years in my graduate student life, will be helpful for a life as an advanced researcher in neuroscience and the job-hunting activities. Thus, I can spend fulfilling days under very stimulating environment.Moreover, I’m grateful for the generous support for the job-hunting activities from our laboratory.
Miyazaki K, Masamoto K, Morimoto N, Kurata T, Mimoto T, Obata T, Kanno I, Abe K. Early and progressive impairment of spinal blood flow-glucose metabolism coupling in motor neuron degeneration of ALS model mice. J Cereb Blood Flow. Metab. In press.
Miyazaki K, Ohta Y, Nagai M, Morimoto N, Kurata T, Takehisa Y, Ikeda Y, Matsuura T, Abe K. Disruption of neurovascular unit prior to motor neuron degeneration in amyotrophic lateral sclerosis. J Neurosci Res. 2011 May;89(5):718-28.
Miyazaki K, Nagai M, Morimoto N, Kurata T, Takehisa Y, Ikeda Y, Abe K. Spinal anterior horn has the capacity to self-regenerate in amyotrophic lateral sclerosis model mice. J Neurosci Res. 2009 Dec;87(16):3639-48.
Miyazaki K, Nagai M, Ohta Y, Morimoto N, Kurata T, Murakami T, Takehisa Y, Ikeda Y, Kamiya T, Abe K. Changes of Nogo-A and receptor NgR in the lumbar spinal cord of ALS model mice. Neurol Res. 2009 Apr;31(3):316-21.


Pleasure of Scientific Research

Hiromi Kawai


I graduated from department of biotechnological science Kinki University. I have been interested in brain disease and gone on to a master's course of department of neurology Okayama University graduate school. I do feel the passion for research and clinical works of the senior doctors. Although, I’m not a medical doctor, I want to contribute to patients and work hard to research.
  I wrote a paper in English the first year, and three papers have been published now. I am content to have a lot of opportunities for presentation at academic societies and seminars. I believe that it is a very precious experience for you to research connecting the clinical practice, because you can get not only the knowledge and the technique of the medicine but also the idea from clinical practice. I hope you get into our company if you might be interested in our works.
Kawai H, Deguchi S, Deguchi K, Yamashita T, Ohta Y, Omote Y, Kurata T, Ikeda Y, Matsuura T, Abe K. Protection against ischemic stroke damage by synergistic treatment with amlodipine plus atorvastatin in Zucker metabolic rat. Brain Res 2011;1382: 308-314.
Kawai H, Deguchi S, Deguchi K, Yamashita T, Ohta Y, Shang J, Tian F, Zhang X, Liu N, Liu W, Ikeda Y, Matsuura T, Abe K. Synergistic benefit of combined amlodipine plus atorvastatin on neuronal damage after stroke in Zucker metabolic rat. Brain Res, 2011; 1368: 317-323.
Kawai H, Yamashita T, Ohta Y, Deguchi K, Nagotani S, Zhang X, Ikeda Y, Matsuura T, Abe K. Tridermal tumorigenesis of induced pluripotent stem cells transplanted in ischemic brain. JCBFM 30; 2010:1487-1493.


Being a Researcher form China

Tian FengFeng


Two elements are indispensable to grow towering tree from a seed. First element is the gene of the seed and the second is the location where the seed was planted. Three years ago, I entered PhD course of Neurology in Okayama University as a foreign student from Luoayang in China. As a result of having continued my effort to research, I could have published 2 original papers in the International Journal of autophagy "Autophagy". Moreover, my study - i.e. " In vivo imaging of autophagy in a mouse stroke model "- was appeared in the Japanese newspapers and international version of it. My research life has entered its third year, now I’m writing other new papers. I think the reason why I could product such results is the supports from the members of our laboratory. Now I appreciate their kindness very much. I hope many other Chinese and foreign friends come to join our attractive projects.
Tian F, Morimoto N, Liu W, Ohta Y, Deguchi K, Miyazaki K, Abe K. In vivo optical imaging of motor neuron autophagy in a mouse model of amyotrophic lateral sclerosis. Autophagy. 2011 Sep;7(9):985-92.
Tian F, Deguchi K, Yamashita T, Ohta Y, Morimoto N, Shang J, Zhang X, Liu N, Ikeda Y, Matsuura T, Abe K. In vivo imaging of autophagy in a mouse stroke model. Autophagy. 2010 Nov;6(8):1107-14.


Neurology Residence Program

Kosuke Matsuzono M.D.


After graduating the medical school of Kagshima University 3 years ago, I worked a hospital in Akita prefecture as an intern for two years. Now I’m in neurology residency training in Okayama University.
  We aim for the realization of our motto, “Be gentle to the patients, be strict with myself, and provide intense and responsible medical care.”
  I believe neurology is truly worthwhile career, please come over to work with us.


Neurology Residence Program

Yoshiko Kouzai M.D.


I graduated the medical school of Ohita University 3 years ago. In order to be an intern, I came back to my home town Okayama city. Since I was interested in Neurology, I decided to work as a neurology resident of Okayama University.
  Neurological disorders always inspire me with various clinical features that patients present. I think that neurology is very interesting to think about what are our lives.


Scientific Research in PhD Course

Yoshio Omote M.D.


After training as a neurological resident in Okayama University, I entered the doctoral course of Neurology. Now I’m belonging to the basic and clinical research about cognition and vascular disease. Amid the heightening talk about aging society of Japan, patients with stroke, cognitive impairment and neurodegenerative will increase. If you are interesting in such diseases, let’s study and work with us.


Clinical Training and Scientific Research

Shoichiro Kohno M.D.


Last year, I began to work neurological resident in Okayama University, where multidisciplinary and comprehensive strategies are needed to diagnose, which are making me a strong and smart neurologist. Working in our University hospital is very appealing in terms of training as a neurologist through discussions on such cases with the attending doctors.
  From this year, I started about the clinical and scientific study in PhD course of the University with enjoying my work and research life.


Balancing of Work and Family

Noriko Hatanaka M.D.


After having got pregnant, I have been working as a neurologist in this department. However, I can make out work while raising two children now with a support from the doctors’ office. I am enjoying working as a neurologist here and hope to continue for a long period.





Contact Us


Address 2-5-1, Shikatacho, Kita-ku, Okayama-shi, Okayama, 700-8558, Japan
TEL +81-86-235-7365