97
**** Jour. AVMA, 2002 ****** USDA-APHIS, 2001
***** Sigurdson et al., 2001
AREAS OF HIGHEST CONCENTRATION IN THE BRAIN
The primary site of pathogenic prions is the region of the obex
between the medulla oblongata and the spinal cord. The dorsal
vagal nucleus and other important nuclei here show typical spongi-
form changes. Other regions of the brainstem display lesions.
Spongiform encephalopathy of the cerebellar cortex explains the
locomotor disturbances and ataxia. Insoluble amyloid forms in the
nerve cells, with high concentration of pathogenic prions and
spongiform changes. Neighboring glia cells are also affected.
Another TSE, chronic wasting disease (CWD) of North American
deer and elk, discovered in Colorado in 1967, has been found in
wild or farmed deer and elk in Wyoming, Nebraska, South Dako-
ta, Oklahoma, Montana, Wisconin, and one case in Illinois. (The
North American elk, a misnomer, is Cervus canadensis,
not Alces
alces—the European Elch and the North American moose.) There
is no evidence that other species, including man, are infected
through contact with CWD.**** In experimental deer inoculated
orally with infective deer brain, pathogenic PrP were first found in
lymphoid tissues of the alimentary system and then in autonomic
nerves leading from the gut to the brainstem, where they appeared
first in the dorsal motor nucleus of the vagus. Other peripheral
nerves, such as the brachial plexus and sciatic nerve, were tested
and found negative.*****
The U. S. government has prohibited importation of live ruminants
and most ruminant products from Europe and Canada. The U. S.
Food and Drug Administration prohibits the feeding of most mam-
malian protein to ruminants. The U. S. Dept. of Agriculture has
prohibited importation of all rendered animal products of any
species. There is no evidence of BSE in the United States after a
decade of testing for it.******
REMOVAL OF THE BRAINSTEM FOR LABORATORY TESTS
After slaughter and decapitation, brainstem tissue can be removed
with a curette through the foramen magnum. If the head is bisect-
ed, the myelencephalon and metencephalon are separated from the
more rostral parts of the brain (see p. 51, below by a transverse cut
through 13 and 14, and by cutting the roots of cranial nn. V-XII
and the cerebellar peduncles to release the sample of the brainstem.
The material of the obex region is used for the BSE rapid test. If the
results are positive, histopathologic, immunohistochemical, and
E/M investigations follow, for which more rostral parts of the
brainstem are used.
TRANSMISSION OF BSE TO MAN
Human infection with the agent of BSE and consequent illnes with
the variant of Creutzfeld-Jacob disease (vCJD) is highly probable.
In vCJD the multiplication of the agent also occurs outside the
brain and spinal cord in the lymphatic organs (e.g. tonsils); where-
as in the sporadic (classical) CJD the pathological changes remain
restricted to the CNS. The likelihood of transmission from BSE-
infected cattle to man is supported by the fact that the agents of BSE
and vCJD are biologically and biochemically identical. The con-
nection of time and place between occurences of BSE and vCJD in
Great Britain supports this probability. Apparently a genetically
determined susceptibility plays a role in transmission because, so
far, only a few people have contracted vCJD, and only a few of the
cattle in a herd contract BSE.
DANGERS OF EATING MEAT AND MEAT PRODUCTS
FROM BSE-INFECTED CATTLE OR CATTLE SUSPECTED
OF EXPOSURE TO BSE
The risks increase with the amount of infective material consumed
and its concentration of pathogenic misfolded prions. Of the com-
ponents of nervous tissue, the perikarya and therefore the ganglia
and nuclei may present a greater danger than axons, and thus more
than nerves and fiber tracts. The perikarya occupy a much larger
volume and have a concentration of prions in the lysosomes, which
are not present in the processes. The danger is increased, the near-
er the ganglia lie to valuable cuts of meat; for example, the sympa-
thetic trunk and ganglia are closely associated with the tenderloin
(iliopsoas and psoas minor (see p. 81, upper fig.) The spinal ganglia
lie in the intervertebral foramina and are included with the bone in
steaks cut from the rib and loin regions (see text fig.). Regarding the
concentration of pathogenic misfolded prions the following list
presents the opinion of the European Union on the possible risk of
infectivity in various tissues (including experiments with scrapie).
1. Highly infectious tissues: brain and spinal cord together with
surrounding membranes, eyes, spinal ganglia.
2. Tissues of intermediate infectivity: intestine, tonsils, spleen, pla-
centa, uterus, fetal tissue, cerebrospinal fluid, hypophysis, and
adrenal gl.
3. Tissues of lower infectivity: liver, thymus, bone marrow, tubu-
lar bones, nasal mucosa, peripheral nerves.
4. Infectivity was not demonstrated in the following tissues and
organs: skeletal muscle, heart, kidneys, milk, fat (exept mesen-
teric fat), cartilage, blood, salivary gll., testis, and ovary.
Nervous tissue of the region of the obex in BSE.
Preparation: Prof. F. Ehrensperger, Inst. of Vet. Pathology, Zürich
Normal nervous tissue in the region of the obex (dorsal vagal nucleus).
Preparation: Prof. G. Boehme, Inst. of Vet. Anatomy, FU-Berlin
Gray matter
1 Dorsal horn
2 Ventral horn
3 Central canal
4 White matter
5 Dorsal root
6 Spinal ganglion
7 Ventral root
8 Dorsal br.
9 Ventral br.
Spinal cord and sympathetic trunk after
removal of the left side of the vertebral
arches and the musculature
Legend:
10 Communicating brr.
white and gray
11 Ganglia of sympathetic trunk
12 Sympathetic trunk
13 Denticulate lig.
14 Pia mater
15 Arachnoidea
16 Dura mater
a Psoas major
b Psoas minor
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