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CJD CLEVELAND COUNTY NORTH CAROLINA Doctor Probable case of rare, fatal disease THIRD for area in recent months

Posted Mar 17 2013 12:17pm
Doctor: ‘Probable’ case of rare, fatal disease third for area in recent months




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.




Ben Earp





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, Raymond.




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 husband.




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 brain.




“He was unsteady when he walked. He had recent issues with memory,” Klein recalled.




The answers lied in the MRI scan, which shows detailed images of organs and tissues.




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.




Diagnosis




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 reports.




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 website.




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 husband.




“I told him I loved him,” she said. “His mouth moved and I read, ‘I love you, too.’”




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 from.”




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.




Finding closure




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 awareness.




“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 abanks@shelbystar.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 disease, including:




Dementia




Lack of coordination




Behavioral changes




Coma may occur




Blindness




Hallucinations




Muscle twitching




Sleepiness




Speech impairment Most die a few months to two years after symptoms surface.




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 Medicine














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 Prion


7:2, 99–108; March/April 2013; © 2013 Landes Bioscience












Thursday, February 21, 2013


National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013










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 report/CJD










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 studies










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 November 2012










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


IN CONFIDENCE


SCRAPIE TRANSMISSION TO CHIMPANZEES


IN CONFIDENCE










why do we not want to do TSE transmission studies on chimpanzees $





snip...




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.




snip...




R. BRADLEY











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










TSS
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