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Articles by M Nishizawa
Total Records ( 2 ) for M Nishizawa
  M Tada , A Kakita , Y Toyoshima , O Onodera , T Ozawa , T Morita , M Nishizawa and H. Takahashi

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by prominent autonomic failure with ataxia and/or parkinsonism. The leading cause of death in MSA is sudden death. We have shown that the early development of autonomic failure is an independent risk factor for sudden death. The depletion of sympathetic preganglionic neurons in the spinal intermediolateral cell column (IML) and its afferent medullary catecholaminergic and serotonergic neurons has been proposed to be partly responsible for autonomic failure in MSA. In this study, we investigated whether the depletion of neurons in any of these autonomic neuron groups contributes to sudden death in MSA. Out of 52 autopsy-proven patients with MSA, we selected 12 individuals who had died within 3.5 years after disease onset to define the accurate levels of slices and identify early neuropathological changes of autonomic nuclei in MSA. Four patients succumbed to sudden death and eight patients died through established causes. Serial 10 µm sections were obtained from the 8th segment of the thoracic cord and the rostral medulla oblongata. Sections from the medulla oblongata were immunostained for thyrosine hydroxylase and tryptophan hydroxylase. The total cell number in the five sections was computed for comparison. Compared with the control, the MSA group showed a marked depletion of neurons in the IML (38.0 ± 7.1 versus 75.2 ± 7.6 cells, P < 0.001), thyrosine hydroxylase-immunoreactive neurons in the ventrolateral medulla (VLM) (17.4 ± 5.1 versus 72.8 ± 13.6 cells, P < 0.01) and tryptophan hydroxylase-immunoreactive neurons in the VLM (15.6 ± 9.2 versus 60.8 ± 17.0 cells, P < 0.01), nucleus raphe obscurus (19.3 ± 4.4 versus 75.3 ± 8.6 cells, P < 0.001), nucleus raphe pallidus (2.1 ± 2.7 versus 9.0 ± 3.4 cells, P < 0.03), and arcuate nucleus (0.4 ± 0.8 versus 2.3 ± 1.5 cells, P < 0.05). Moreover, in patients who succumbed to sudden death, when compared with patients who had established causes of death, we found a marked depletion of tryptophan hydroxylase-immunoreactive neurons in the VLM (7.3 ± 3.5 versus 21.8 ± 6.5 cells, P < 0.02) and nucleus raphe obscurus (15.0 ± 2.0 versus 22.5 ± 2.1 cells, P < 0.01). The results indicate that the spinal IML and medullary catecholaminergic and serotonergic systems are involved even in the early stages of MSA, and the dysfunction of the medullary serotonergic system regulating cardiovascular and respiratory systems could be responsible for sudden death in patients with MSA.

  I Nozaki , T Hamaguchi , N Sanjo , M Noguchi Shinohara , K Sakai , Y Nakamura , T Sato , T Kitamoto , H Mizusawa , F Moriwaka , Y Shiga , Y Kuroiwa , M Nishizawa , S Kuzuhara , T Inuzuka , M Takeda , S Kuroda , K Abe , H Murai , S Murayama , J Tateishi , I Takumi , S Shirabe , M Harada , A Sadakane and M. Yamada

We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999. We obtained information on 1685 Japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n = 180, 14.7%) and probable (n = 1029, 84.2%) cases, except for dura mater graft-associated Creutzfeldt–Jakob disease which also included possible cases (n = 13, 1.1%). They were classified into 922 (75.5%) with sporadic Creutzfeldt–Jakob disease, 216 (17.7%) with genetic prion diseases, 81 (6.6%) with acquired prion diseases, including 80 cases of dura mater graft-associated Creutzfeldt–Jakob disease and one case of variant Creutzfeldt–Jakob disease, and three cases of unclassified Creutzfeldt–Jakob disease (0.2%). The annual incidence rate of prion disease ranged from 0.65 in 1999 to 1.10 in 2006, with an average of 0.85, similar to European countries. Although methionine homozygosity at codon 129 polymorphism of the prion protein gene was reported to be very common (93%) in the general Japanese population, sporadic Creutzfeldt–Jakob disease in Japan was significantly associated with codon 129 homozygosity (97.5%), as reported in western countries. In sporadic Creutzfeldt–Jakob disease, MM1 type (Parchi’s classification) is the most common, as in western countries. Among atypical sporadic Creutzfeldt–Jakob disease cases, the MM2 type appeared most common, probably related to the very high proportion of methionine allele in the Japanese population. As for iatrogenic Creutzfeldt–Jakob disease, only dura mater graft-associated Creutzfeldt–Jakob disease cases were reported in Japan and, combined with the data from previous surveillance systems, the total number of dura mater graft-associated Creutzfeldt–Jakob disease was 138, comprising the majority of worldwide dura mater graft-associated Creutzfeldt–Jakob disease patients. Regarding genetic prion diseases, the most common mutation of prion protein gene was V180I (41.2%), followed by P102L (18.1%), E200K (17.1%) and M232R (15.3%), and this distribution was quite different from that in Europe. In particular, V180I and M232R were quite rare mutations worldwide. Patients with V180I or M232R mutations rarely had a family history of prion diseases, indicating that a genetic test for sporadic cases is necessary to distinguish these from sporadic Creutzfeldt–Jakob disease. In conclusion, our prospective 10-year surveillance revealed a frequent occurrence of dura mater graft-associated Creutzfeldt–Jakob disease, and unique phenotypes of sporadic Creutzfeldt–Jakob disease and genetic prion diseases related to the characteristic distribution of prion protein gene mutations and polymorphisms in Japan, compared with those in western countries.

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