[7] Texas CJD patient
Date: Mon 29 Mar 2010
Source: Recordandolinda.com [edited]
<http://recordandoalinda.com/index.php?option=com_content&view=article&id=19&It emid=8>
Physician Discharge Summary, Parkland Hospital, Dallas Texas General
Neurology Team: General Neurology Team
A Hispanic female with no past medical history presented with 14
months of increasing/progressive altered mental status, generalized
weakness, inability to walk, loss of appetite, inability to speak,
tremor and bowel/bladder incontinence. She was in her usual state of
health up until February 2009, when her husband noted that she began
forgetting things like names and short term memories. He also noticed
mild/vague personality changes such as increased aggression. In March
[2009], she was involved in a hit-and-run motor vehicle accident,
although she was not injured. The police tracked her down and
ticketed her. At that time, her son deployed to Iraq with the Army
and her husband assumed her mentation changes were due to stress over
these 2 events. Also in March 2009, she began to have weakness in her
legs, making it difficult to walk. Over the next few months, her
mentation and personality changes worsened, getting to a point where
she could no longer recognize her children.
She was eating less and less. She was losing more weight. In the last
2-3 months, she reached the point where she could not walk without an
assist, then one month ago, she stopped talking, only making
grunting/aggressive sounds when anyone came near her. She also became
both bowel and bladder incontinent, having to wear diapers. Her
tremor and body jerks worsened, and her hands assumed a sort of
permanent grip position, leading her family to put tennis balls in
her hands to protect her fingers. The husband said that they had
lived in Nebraska for the past 21 years. They had seen a doctor there
during the summer time who prescribed her Seroquel and Lexapro,
thinking these were signs of a mood disorder. However, the
medications did not help, and she continued to deteriorate
clinically. Up until about 6 years ago, the patient worked at Tyson
foods, where she worked on the assembly line slaughtering cattle and
preparing them for packaging. She was exposed to brain and spinal
cord matter when she would euthanize the cattle. The husband says
that he does not know any fellow workers with a similar illness. He
also says that she did not have any preceding illness or travel. The
patient died at 38 years old on 6 Feb 2010 in Mesquite Texas.
--
Communicated by:
Terry S Singeltary Sr <flounder9@verizon.net>
[Terry S. Singeltary Sr. has added the following comment:
"According to the World Health Organisation, the future public health
threat of vCJD in the UK and Europe and potentially the rest of the
world is of concern and currently unquantifiable. However, the
possibility of a significant and geographically diverse vCJD epidemic
occurring over the next few decades cannot be dismissed
<http://whqlibdoc.who.int/publications/2003/9241545887.pdf>. The key
word here is diverse. What does diverse mean? If USA scrapie
transmitted to USA bovine does not produce pathology as the UK c-BSE,
then why would CJD from there look like UK vCJD?"
Attention has been drawn to this case on account of the occupation of
the of the deceased patient and her prolonged exposure to cattle
brain and spinal cord tissue fragments. Creutzfeldt-Jakob disease
(CJD) is a rare, degenerative, invariably fatal brain disorder. It
affects about one person in every one million people per year
worldwide; in the United States, there are about 200 cases per year.
CJD usually appears in later life and runs a rapid course. Typically,
onset of symptoms occurs about age 60, and about 90 percent of
patients die within one year. In the early stages of disease,
patients may have failing memory, behavioral changes, lack of
coordination and visual disturbances. As the illness progresses,
mental deterioration becomes pronounced, and involuntary movements,
blindness, weakness of extremities, and coma may occur. There are 3
major categories of CJD.
CJD cannot be transmitted through the air or through touching or most
other forms of casual contact. Spouses and other household members of
sporadic CJD patients have no higher risk of contracting the disease
than the general population. However, exposure to brain tissue and
spinal cord fluid from infected patients should be avoided to prevent
transmission of the disease through these materials. In some
situations, CJD has spread to other people from grafts of dura mater
(a tissue that covers the brain), transplanted corneas, implantation
of inadequately sterilized electrodes in the brain, and injections of
contaminated pituitary growth hormone derived from human pituitary
glands taken from cadavers (all usually designated as iatrogenic
cases). Since 1985, all human growth hormone used in the United
States has been synthesized by recombinant DNA procedures, which
eliminates the risk of transmitting CJD by this route.
The appearance of the new variant of CJD (nv-CJD or v-CJD) in several
younger than average people in Great Britain and France has led to
concern that BSE may be transmitted to humans through consumption of
contaminated beef. Although laboratory tests have shown a strong
similarity between the prions causing BSE and v-CJD, there is no
direct proof to support this theory.
Nonetheless, the occupation of the deceased patient in Texas has
raised concerns regarding the origin of this woman's illness, and
further investigation is merited. - Mod.CP]
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