Rinshō shinkeigaku = Clinical neurology
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Since April 1999, prion diseases in Japan have been surveyed with the field investigation system by the Creutzfeldt-Jakob Disease (CJD) Surveillance Committee under the Prion Disease and Slow Viral Infection Research Committee sponsored by the Ministry of Health, Labor, and Welfare of Japan. By March 2003, a total of 409 patients with prion diseases were reported, including 324 with sporadic CJD (79.2%), 49 with inherited prion diseases (12.0%), and 36 with infectious prion diseases (8.8%). About 15% of the sporadic CJD cases presented with atypical clinical features [long clinical course to akinetic mutism (> 9 months) or absence of periodic synchronous discharges (PSD) on EEG], including thalamic form of CJD (MM2 type) in the autopsy verified cases. ⋯ All the 36 patients with infectious prion diseases were iatrogenic cases due to cadaveric dura mater grafts. Taken together with the data by the previous surveillance system, a total of 97 dura mater cases has been reported in Japan; the latency period between receipt of a dura mater graft and onset of CJD was 122 +/- 53 months (mean +/- SD) including 275 months as the longest one. Fortunately, there was no case of variant CJD.
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In Japan, we have first alerted the necessity of critical care neurology (CCN) and neuro-ICU in 2000, however, CCN is still unrecognized. In the United States and Germany, CCN has become recognized as one of the important subspecialty in neurology, and also, there is progressive increase of neuro-intensive care unit (neuro-ICU) after 1990s. In this symposium, the present status, problems, and future direction of clinical study and practice in CCN were reviewed from the standpoints as follows; 1 Quantitative and sequential analysis of literatures in CCN: MEDLINE search disclosed marked increase in CCN-related literatures since 1990s. 2 Categorical analysis of important literatures selected by German Society of CCN. 3 Analysis of clinical practice in CCN through neuro-ICU simulation study: in 442 inpatients who admitted to our department during 1998. 4 Analysis of requisites in critical care in neurology practice both in diagnostic and therapeutic aspects. 5 Historical development of CCN. 6 Evaluation of the effect of neuro-ICU. 7 Short-term and long-term problems for the activation of CCN. In conclusion, 1) CCN is still immature in Japan, and needs early establishment, including neuro-ICU and educational system for developing neurointensivist, 2) Extraction of problems, decision-making, and rapid differential diagnosis are critical to prevent and manage life-threatening neurological conditions.
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Prion diseases include scrapie, BSE and CWD in animals, and spontaneous, familiar and infectious Creutzfeldt-Jakob disease(CJD) in human. Infectious prion diseases include kuru, variant CJD and iatrogenic CJD. CJD has been transmitted from human to human by contaminated cadaveric dura mater grafts and by cadaveric pituitary hormones. ⋯ Two groups of dura-related CJD are manifest in clinical course and pathological characteristics, such as rapidly progressive group and slowly progressive. Rapidly group was similar to cases with classical CJD in clinical features and shot duration to death from onset. Slowly progressive group developed akinetic mutism longer than 1 year, and characterized by florid plaques in the brain (Kitamoto).
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Therapeutic nihilism has been overcome by the results of phase III trials of intravenous t-PA with 3-hours time window. The integrated team approach can increase the number of patients treated rapidly, permit closer monitoring of patients, potentially increase the safety of thrombolysis, and streamline diagnosis and therapy. The results of randomized clinical trials and meta-analyses demonstrated that stroke unit treatment made mortality lower, hospitalization shorter, good outcome and discharge to the home greater as compared to treatment in general medical units. ⋯ In patients with moderate to severe neurological deficits at admission (NIHSS score 7 or more), good outcome (mRS score 0 to 2) was also more frequently observed in SCU than in other wards. We have to reestablish the systems of acute stroke management. It will be achieved by obtaining the approval of the use of t-PA, organizing stroke care units in major hospitals, and assessing their effects not only on patients' outcome but also on the quality and costs of care by their families, medical personnels and our public societies.
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Residency in the United States has been known as one of the most organized systems for postgraduate medical education in the world. However, recent changes in the social, ethical and economic environment in the healthcare have brought new demands to the residency education. ⋯ Additionally, residency program directors are facing new challenges of protecting healthcare private information, managed care, underrepresented minorities and visa acquisition by international medical graduates. Consequently, the workload of residency program directors has become more demanding.