Doctor: ‘Probable’ case of rare, fatal disease third for area in recent
Ellen Horton holds a photo of her late husband, Raymond Horton, who passed
away in December from what one doctor says was Creutzfeldt-Jakob disease.
Raymond was one of three people whose life is believed to have been claimed by
this rare disease in Cleveland County in recent months.
By Alicia Banks
Published: Saturday, March 16, 2013 at 21:54 PM. It started with double
vision. That’s when Ellen Horton knew something was wrong with her husband,
The couple was en route back to Shelby from a car show in Pigeon Forge,
Tenn., in September. She described his driving as erratic.
“He was all over the road. He would run off the road and cross the center
lines,” she said. “I found out (that) to him, the line in the roads were curved
because of his eyes.”
The couple visited multiple doctors searching for answers. None surfaced.
With time, daily activities became a strain for the Marine Corps veteran.
His sight never came back. His arms stiffened.
“He couldn’t hold a sandwich. He forgot where his mouth was to feed him,”
Horton remembered. “He was fine in Pigeon Forge. He saw the ceiling fan
continuously turning, but the fan was off.”
Reading became impossible for Raymond. The avid reader had read the Bible
more than once. Horton said all Raymond wanted was his eyesight back. Tears sat
on the rim of her eyelids.
What Raymond is believed to have battled affects less than 400 people each
year, according to the Centers for Disease Control and Prevention.
It would be December before Horton discovered what plagued her
Searching for answers
Raymond saw Dr. Kevin Klein, head neurologist of Klein Neurology and Sleep,
in December. Klein used an EEG and MRI to gain a closer look inside Raymond’s
“He was unsteady when he walked. He had recent issues with memory,” Klein
The answers lied in the MRI scan, which shows detailed images of organs and
Parts of Raymond’s brain cortex appeared white on the MRI scan. Klein said
the discoloration, or abnormal diffusions, were similar to what doctors see in
stroke patients. Raymond never had a stroke.
“Most people who have what he did don’t lose their vision first, but he
did,” Klein said.
Klein diagnosed Raymond with Creutzfeldt-Jakob disease. The rare,
degenerative brain disease — not unlike mad cow disease — is marked by abnormal
proteins called prions that can develop in humans and animals, the CDC
It was previously reported two Cleveland County deaths with links to CJD,
one in December and another in January. Both deaths were labeled “probable
cases” of CJD.
CJD can come in various forms, including classic/sporadic, which develops
naturally in humans. It can lay dormant for many years. Symptoms usually surface
about age 65 in those affected, according to the National Library of Medicine’s
The disease isn’t contagious.
Horton found relief in her husband’s diagnosis.
But Raymond’s condition worsened.
He was admitted to Cleveland County Hospice in mid-December. Nurses bathed
and shaved him. Members from the N.C. Foothills Marine Corps League Detachment
1164 visited their former commandant while he was in Hospice.
Horton recalled a moment where she laid her head on her ailing
“I told him I loved him,” she said. “His mouth moved and I read, ‘I love
She sat calmly on her living room couch. A white tissue caught the falling
tears from her eyelids.
“He went through three months of hell,” Horton said.
Raymond died Dec. 28. He was 66 years old.
All forms of CJD are fatal. There is no known cure.
County and state cases
Klein diagnosed two other patients in Cleveland County with sporadic CJD in
2012. Both were between 60 and 70 years old.
Both passed away shortly after.
“That’s a high number,” Klein said. “It’s unknown where sporadic CJD comes
CJD can mirror other diseases such as dementia, Alzheimer’s and some
later-staged cancers. Symptoms include difficulty speaking and swallowing,
memory loss, blurred vision and jerky movements, according to the National
Library of Medicine’s website.
Raymond displayed all of them.
About 85 percent of CJD cases in the nation are sporadic, the CDC reports.
Since 2011, The N.C. Department of Health and Human Services reported 17
confirmed cases of CJD.
Nine were labeled probable.
Most with sporadic CJD live up to seven months before succumbing to the
disease, the MayoClinic reports.
CJD can only be confirmed through a brain biopsy or a cerebrospinal fluid
test, according to the National Institute of Neurological Disorders and Stroke’s
website. All three of Klein’s patients are called “probable cases” of CJD
because neither test was performed. Any tests to confirm CJD must be arranged by
the hospital handling the case, it was previously reported.
Horton said, because CJD was new to her, she didn’t realize a brain biopsy
could be done. Later, Horton requested a test on Raymond’s spinal fluids from
Wake Forest Baptist Medical Center in Winston-Salem. The results are pending.
Horton believes Raymond suffered from CJD after a doctor from Wake Forest
Baptist agreed with Klein’s diagnosis.
“I didn’t want to put his body through anymore,” Horton said about opting
out of a brain biopsy.
Horton described her husband as an “all-around kind of guy.” Those who
found a father figure in Raymond called him “paw-paw” or “Daddy Raymond.”
She doesn’t know if Raymond knew he had CJD.
“He never asked about it or mentioned it,” she said. “I still wonder if I’m
going to end up with it.”
Raymond’s case was the first time Horton heard of CJD. It could be the same
for many others.
She hopes sharing Raymond’s story not only leads to closure, but
“We don’t know what we’re walking around with in our bodies. We don’t,” she
said. “So little is known about it.”
Horton clutched a framed picture of Raymond smiling. He wore a Marines cap.
Horton said, she and Raymond lived to help others.
She wants his story to do the same.
Reach reporter Alicia Banks at 704-669-3338, email at email@example.com
and follow on Twitter @TheStarAlicia.
What is Creutzfeldt-Jakob disease?
A rare, degenerative fatal brain disorder that affects one person in every
1 million people per year worldwide.
Fewer than 400 cases are usually reported in the US each year.
CJD is not contagious and is always fatal.
What are the Symptoms? Signs of CJD can sometimes mirror Alzheimer’s
Lack of coordination
Coma may occur
Speech impairment Most die a few months to two years after symptoms
What are the forms?
Classic or sporadic: the disease appears even though the person has no
known risk factors for the disease. This accounts for 85 percent of cases.
Hereditary: Marked by a family history of the disease that can be tested
for a genetic mutation associated with CJD.
Variant: Linked to people eating infected beef with bovine spongiform
encephalopathy, or BSE, found in cows, also known as “mad cow disease.”
CJD can be transmitted through contact with contaminated brain tissue from
surgeries or medical procedures.
How many cases have been reported in North Carolina?
Year Confirmed Probable Total
2011 9 3 12
2012 8 6 14
Note: No cases of variant CJD, suspected to be related to mad cow disease,
have ever been reported or identified in North Carolina.
How can you get help?
Contact the Cleveland County Health Department at 704-484-5100 for any
questions about CJD or if you’re experiencing any of the symptoms, and contact
your health care provider.
Source: The National Institute for Neurological Disorders and Stroke, and
the N.C. Department of Health and Human Services, and the National Library of
POPULATION CLEVELAND COUNTY NORTH CAROLINA 2011 = 97,489.
3 CJD DEATHS RECORDED WITHIN MONTHS IN A COUNY WITH 97,489 ???
Tuesday, March 05, 2013
A closer look at prion strains Characterization and important implications
7:2, 99–108; March/April 2013; © 2013 Landes Bioscience
Thursday, February 21, 2013
National Prion Disease Pathology Surveillance Center Cases Examined January
16 YEAR OLD SPORADIC FFI ?
Monday, January 14, 2013
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
Sunday, February 10, 2013
Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection
Tuesday, March 5, 2013
Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening
of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION) FDA believes current
regulation protects the public from BSE but reopens comment period due to new
Thursday, February 14, 2013
The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and
TSE prion disease
Sunday, November 11, 2012
*** Susceptibilities of Nonhuman Primates to Chronic Wasting Disease
Friday, December 14, 2012
Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005
- December 14, 2012
Thursday, March 29, 2012
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
Wednesday, February 16, 2011
SCRAPIE TRANSMISSION TO CHIMPANZEES
why do we not want to do TSE transmission studies on chimpanzees $
5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
Friday, February 11, 2011
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5-May 2011
Sunday, April 18, 2010
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
Thursday, November 18, 2010
Increased susceptibility of human-PrP transgenic mice to bovine spongiform
encephalopathy following passage in sheep
Wednesday, January 19, 2011
EFSA and ECDC review scientific evidence on possible links between TSEs in
animals and humans Webnachricht 19 Januar 2011
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease