> But Gambetti said the risk to those patients is slight.
> "The chances that this disease is transmissible are very, very low," he
1: Brain. 1990 Dec;113 ( Pt 6):1891-909.
Spongiform encephalopathy transmitted experimentally from
Creutzfeldt-Jakob and familial Gerstmann-Straussler-Scheinker diseases.
Baker HF, Duchen LW, Jacobs JM, Ridley RM.
Division of Psychiatry, MRC Clinical Research Centre, Harrow, Middlesex, UK.
A comparison was made of the effects of experimental intracerebral
inoculation into marmosets of brain homogenates from a case of
Creutzfeldt-Jakob disease (CJD) and from a member of the Wo. family with
cerebral amyloid and spongiform encephalopathy--the
Gerstmann-Straussler-Scheinker (GSS) syndrome. All the inoculated
marmosets developed spongiform encephalopathy (SE) after incubation
times of 20-23 months in the CJD group and 25-32 months in the GSS
group. Subsequent passage from 1 affected animal in each group resulted
in SE developing after 17 months incubation. In every animal inoculated
with CJD or GSS material and in the 2 passage experiments the most
severely affected region of the brain was the thalamus which in all
cases was almost totally occupied by vacuoles. Other grey matter masses
were less severely and less consistently affected. Vacuolation was
observed in the cerebellar granule cell layer as well as in the
molecular layer and the brain stem was finely vacuolated in all cases.
There were only minor and inconsistent differences between the disease
transmitted from CJD compared with GSS and some differences between the
original transmissions and the SE caused by passaged inocula. Severe
astrocytic gliosis accompanied the spongiform changes but no amyloid was
identified in any of the marmosets with experimentally transmitted
disease. The pathogenesis of the spongiform change in the thalamus was
studied in a series of marmosets by light and electron microscopy 3-22
months after the intracerebral inoculation of CJD or GSS homogenates and
was compared with controls. Dilated irregularly-shaped cisternae and the
large complex vacuoles typical of SE, present in abundance after 18 and
22 months incubation, were considered most probably to be derived from
cisternae of neuronal smooth endoplasmic reticulum.
* Case Reports
Volume 43 Issue 12 Page 1687 - December 2003
Similar levels of infectivity in the blood of mice infected with
human-derived vCJD and GSS strains of transmissible spongiform
Larisa Cervenakova, Oksana Yakovleva, Carroll McKenzie, Svetlana
Kolchinsky, Lisa McShane, William N. Drohan, and Paul Brown
The possible transmission of variant CJD (vCJD) through blood
transfusion or use of plasma-derived products prompted this study
comparing infectivity in murine models of vCJD and
Gerstmann-Sträussler-Scheinker (GSS) disease, a non-vCJD form of
transmissible spongiform encephalopathy (TSE).
STUDY DESIGN AND METHODS:
RIII/Fa/Dk (RIII) or Swiss-Webster (Swiss) mice were inoculated
intracerebrally (IC) with mouse-adapted strains of vCJD or GSS
(Fukuoka-1) of similar infectivity. Groups of RIII mice were euthanized
17 weeks after inoculation (during the incubation period), and another
23 weeks after inoculation (when symptomatic). Blood was collected,
separated into components, and inoculated into groups of healthy mice;
brains and spleens from all mice were harvested and tested for the
presence of PrPres by Western blot using 6H4 MoAb.
Levels of 20-30 infectious doses per mL were present in buffy coat and
plasma during both the incubation and symptomatic stages of disease; PLT
pellet infectivity was lower (10 ID/mL) and RBCs were not infectious.
The disease was transmitted more efficiently by IV than IC inoculation
of plasma, but there was no difference observed with inoculation of
buffy coat. The incubation period was shorter after IC inoculation of
GSS- than vCJD-brain inocula. The amount of PrPres in spleens was
similar for both TSE agents, but was slightly lower in brains of vCJD
than GSS mice.
Infectivity was detected in blood components of mice infected with a
human-derived strain of vCJD during both the preclinical and clinical
phases of disease in a similarly low range of concentrations as in mice
infected with a human-derived nonvariant strain (GSS, Fukuoka-1). Other
measures of virulence, including brain infectivity titers, incubation
periods, and the accumulation of PrPres in spleens and brains, were also
comparable in both experimental models.
Terry S. Singeltary Sr. wrote:
> Wednesday, August 18, 2004 - Page updated at 12:00 A.M.
> Nation's experts work to identify mystery disease
> By Sandi Doughton
> Seattle Times staff reporter