From: Champion Expanding Encyclopedia Of Mortuary Practices Number 625. pp 2514-17, 1995
ABSTRACT: An in-depth discussion and explanation of Creutzfeldt-Jakob disease along with several related prion driven disorders are covered for the elucidation and education of the embalmer. Early history of the various diseases is outlined with the interrelationships to kuru, scrapie, BSE and other similar neuro-degenerative diseases. The bizarre nature of the causative agent is discussed in detail along with its remarkable survivability. The dangers and concerns that embalmers have are delineated and placed in perspective. A suggested protocol to minimize risk during embalming is presented. A summation and suggestions for embalmers completes the article.
INTRODUCTION: Creutzfeldt-Jakob disease is a disease of bizarre nature
that almost every embalmer or funeral director has heard of but has virtually
no idea concerning the facts and realities of the disease itself. There is a
very small amount of information that has been available to the embalming profession
concerning Creutzfeldt-Jakob disease and unfortunately it is either incomplete,
misleading or outright incorrect. There is more conjecture and rumor involved
with this disease than almost any other disease and there is virtually no valid
information available to help the embalmer. This has resulted in a definite
uncertainty and even panic in most situations involving embalmers when they
encounter this disease during the course of their professional career. Nothing
is more fearful than complete rack of information concerning a potentially deadly
disease.
The intent of this article is to introduce you, the embalmer, to the strange
world of the Human Transmissible Spongiform Encephalopathies, such as Creutzfeldt-Jakob
disease and supply you with the information you need to make intelligent, informed
choices when you are faced with dealing with Creutzfeldt-Jakob disease and how
best to protect yourself in the situation when it arises.
EARLY HISTORY: The strange and convoluted story begins with a curious
dementia case reported by Dr. Creutzfeld in 1920 in Germany. The case involved
a 22 year old woman with a curious neurodegenerative disease of insidious onset
and short duration followed by death. In 1921, Dr. Jakob further elaborated
on this type of unusual dementing disorder by describing 4 dementia cases, all
of middle-aged patients with remarkably similar symptoms and all rapidly resulting
in death. These cases and others were followed through the 1930's to 1950's
and were left essentially as an enigma or causation by an unknown agent or agents.
Interestingly, on a bizarre note, the original case by Dr. Creutzfeldt may not
have actually been the famed Creutzfeldt-Jakob disease that bears his name.
There is general agreement that most if not all of the cases elucidated by Dr.
Jakob were the syndrome and sophisticated analysis of some remaining tissue
samples of the cases confirm Creutzfeldt-Jakob disease as the culprit.
KURU, A CANNIBAL DISEASE: The real breakthrough for understanding Creutzfeldt-Jakob
disease occurred in 1957 with the discovery of a curious degenerative brain
disease of cannibals. Certain Fore tribes of Papua, New Guinea were inflicted
with a disease called Kuru - the trembling with fear. This disease manifested
itself as a fatal disease with an onset and duration that lasted usually 1 year.
To date there have been 2500+ cases reported in these relatively small villages
with an occurrence of at least 1% of the population. It was prevalent in women
and children but rarely affected adult males. The terminal stage of this brain
disease was marked by total dementia, muteness, inability to move, ataxia (jerkiness
of the muscles), dysarthria, dysphagia and death.
Kuru was determined to be linked to the ritual cannibalism that was practiced
by these tribes. The brains of the elders were ritually prepared and eaten by
the women and children, but seldom by the men. This endocannibalism was outlawed
by the authorities of New Guinea and now the disease is slowly disappearing.
There are still cases appearing due to the extreme incubation periods of the
disease -exceeding 30 years in some instances. Kuru was successfully passed
to chimpanzees in 1966 by intracerebral injection of brain specimens. This disease
however, was not passed maternally or by mother's milk which was determined
by very careful epidemiological studies. Further studies have even determined
the exact cannibal feast that resulted in the transmission to certain victims.
A suggested link to a bizarre disease of sheep (scrapie) was proposed in 1959
and ultimately lead to the path of understanding for Creutzfeldt-Jakob disease.
SCRAPIE - AN ANIMAL TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY: This brain
degenerative disease of sheep has been known for over 200 years and usually
develops in breeding ewes that are 2-4 years old. The disease takes its name
from the scraping against trees and fence posts by the sheep to alleviate an
intense itching with brain degeneration and death as the final result. In the
1930's a massive outbreak of scrapie was apparently caused by the use of formalin-treated
louping-ill virus vaccine that destroyed entire flocks and diseased over 1500
sheep. Formaldehyde, in this situation, was proven inadequate in eliminating
or neutralizing the causative agent of scrapie.
Scrapie has been transmitted to several animals by various routes such as intracerebral
injection, parenteral injection and orally (with the oral route being the most
unlikely, yet very possible). The agent causing scrapie has been shown to survive
in soil for years and consequently, no ploughing under of sick sheep is now
allowed. At first, this disease was thought to be related to visna (a retroviral
true slow virus disease of sheep) but such is not the case. Visna is essentially
a sheep counterpart to an AIDS-type dementia complex of humans. Despite years
of research, it is still not known how scrapie spreads in sheep flocks. It is
quite obvious though, that scrapie does indeed spread very insidiously and easily
through sheep.
CREUTZFELDT-JAKOB DISEASE: This disease is a neurodegenerative disease
that evokes no apparent immune or inflammatory response and results in dementia,
cortical blindness, motor disorders, rigidity and myoclonus (muscle spasms)
and some uncontrollable trembling with the result being death usually within
six months of onset of symptoms. There are no documented recoveries, the disease
is fatal. EEG (electroencephalographic) traces appear normal but do exhibit
abnormalities later in the course of the disease. Creutzfeldt-Jakob disease
occurs on a worldwide basis of 1 per million population and the peak age of
occurrence is 60-65 years of age. There are, however, several examples of much
earlier onset. It occurs twice as often in an urban environment as in a rural
setting. There are at feast 250+ diagnosed cases of Creutzfeldt-Jakob disease
every year in the United States.
Approximately 15% of the cases appear to be inherited and demonstrate an autosomal
dominant pattern of gene expression. The rest are sporadic with uncertainty
as to the cause. There appears to be some clustering in certain ethnic groups
such as Libyan Jews and Czechoslovakian herdsmen with the implication that contact
with infected sheep herds and the eating of sheep brains and eyeballs may contribute
to the incidence of the disease. This connection is not certain and is now suspect
with some researchers who believe it is more likely due to a genetic susceptibility
to the disease. Exactly how sporadic cases of Creutzfeldt-Jakob disease do occur
is not known with certainty.
There has been one other unfortunate aspect of the disease - in iatrogenic cases
(medically induced by treatment). There are numerous proven transmissions of
the disease resulting from neurosurgical contamination of stereotaxic electrodes
that were disinfected with formaldehyde, 12 cases of disease from corneal transplants,
over 45 cases from pituitary extract hormone and gonadotropin from cadavers
(unfortunately in most cases transmitted to children) and several cases of transmission
from dura mater grafts from infected cadavers. The most bizarre case involving
neurosurgery infection was the successful transmission of Creutzfeldt-Jakob
disease from electrodes that were confirmed to have transmitted the disease
to patients despite sterilization with formaldehyde. The electrodes were stored
for three years then cleaned and sterilized with formaldehyde/ethanol three
times then implanted into the brain of a chimpanzee. After eighteen months,
the animal was positively diagnosed with the disease. The use of cadaver derived
pituitary hormone extracts was banned in 1985.
There is no direct correlation, but approximately 30 hearth care workers (such
as neurosurgeons, autopsy pathologists, etc.) so far have contracted the disease.
This, however, does not appear to be greater than expected from the normal population
but caution due to the lack of understanding of the exact causes of sporadic
infection is advised. The causative agent of Creutzfeldt-Jakob disease has been
found in brain, spinal cord, cerebrospinal fluid and less in lymph and lymph
tissues, liver, kidney, lung, cornea and finally blood. It is not certain if
it is possible to transmit by mothers milk due to two conflicting reports, but
it does appear possible, but very inefficiently, at best. It was first not thought
possible to transmit by blood transfusion but it now appears likely that this
has occurred. Four cases of probable blood transfusion Creutzfeldt-Jakob disease
have appeared in Australia where females that were treated with cadaver derived
pituitary hormone for infertility subsequently donated blood. It appears quite
possible from animal studies that transmission by placenta, milk and umbilical
cord exists at least in lower animals. Maternal transmission in animal studies
are uncertain but it is positively determined that there is fetal sharing of
maternal erythrocytes.
Definite diagnosis of Creutzfeldt-Jakob disease has been uncertain until very
recently where two-dimensional electrophoresis is now possible in some cases
with reasonably accurate results ante-mortem. Previously, a definitive diagnosis
was only possible at autopsy or at best by a dangerous brain biopsy procedure.
There have been several misdiagnoses of Creutzfeldt-Jakob disease as Alzheimer's
disease. Some researchers feel that due to misdiagnoses, Creutzfeldt-Jakob disease
is underreported and occurs more frequently than it appears. Alzheimer's disease
is also 85% sporadic and 15% familial (linked to inheritance) with an incidence
of 1 out of 4 by the age of 85. The duration is typically longer with 5-10 years
being the average. Alzheimer's disease is predicted to become of epidemic proportions
in the coming decades as the elderly live longer and longer. Alzheimer's disease
also displays numerous beta-amyloid plaques with numerous neurofibrillary tangles
(these are distinct however from the plaques associated with Creutzfeldt-Jakob).
GERSTMANN-STRAUSSLER-SCHEINKER SYNDROME: This is an unusual variant of
a Creutzfeldt-Jakob type disease that was first diagnosed in 1928 by Gerstmann
and followed with seven additional cases in 1936 by all three researchers. It
has a very similar appearance to Creutzfeldt-Jakob but differs in several important
ways. It is very rare with an occurrence of 1 in 10 million of population and
has an earlier onset of symptoms at approximately the 4th decade of life with
a longer duration of typically 5 years. The symptoms are dementia, cerebellar
ataxia and pyramidal signs (of the cerebral cortex) with death being the final
result. Gerstmann-Straussler-Scheinker syndrome appears to be an inherited form
of a Creutzfeldt-Jakob type disease affecting only certain families (of which
several kindreds have been identified throughout the world).
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