Rinshō shinkeigaku = Clinical neurology
-
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.
-
Neurological manifestations of chemical and biological weapons are reviewed. Nerve agents in current use, storage, or production include tabun, sarin, soman and VX. The initial effects of exposure to a nerve agent depend on the dose and on the route of exposure. ⋯ In autopsy, it is really confirmed in the characteristic findings of the meningeal abnormality. The potential weaponization of variola virus continues to pose a military threat because the aerosol infectivity of the virus and the development of susceptible populations. A high rate of lethality, a staunch resistance to treatments and a rapid onset of severe generalised muscle weakness make botulinum toxin a suitable agent for biological warfare particularly by oral administration.