Charles City family says man died of human form of Mad Cow Disease - KWWL - Eastern Iowa Breaking News, Weather, Closings

Charles City family says man died of human form of Mad Cow Disease

CHARLES CITY (KWWL) -- Relatives of a north Iowa man tell KWWL the family member died at his home Wednesday from the human form of Mad Cow disease.

Paula Squier tells KWWL that doctors at the University of Iowa Hospitals and Clinics last week gave her husband Thomas a 90% diagnosis that he was suffering from Creutzfeldt-Jakob Disease. Thomas Squier was 60.

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Typically, onset of symptoms occurs at about age 60. There are three major categories of CJD:  sporadic CJD, hereditary CJD, and acquired CJD.

Squier says doctors told her that her husband had the sporadic form of the rare disease and that there probably isn't any threat to the general public.  While CJD can be transmitted to other people, the National Institute of Neurological Disorders and Stroke says the risk of this happening is extremely small.

Squier said her husband Thomas's symptoms started about 6-7 months ago with vision problems.  As they got worse, she took him to see doctors in Iowa City last week.

According to the National Institute of Neurological Disorders and Stroke, the only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. Squier's family confirms doctors are performing an autopsy to confirm the diagnosis.

Information on the Centers for Disease Control and Prevention website states Classic CJD has been recognized since the early 1920s. The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions.

This sporadic disease occurs worldwide, including the United States, at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual. The risk of CJD increases with age, and in persons aged over 50 years of age, the annual rate is approximately 3.4 cases per million. In recent years, the United States has reported fewer than 300 cases of CJD a year.

Whereas the majority of cases of CJD (about 85%) occur as sporadic disease, a smaller proportion of patients (5-15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.

The CDC says Classic CJD is not related to "mad cow" disease. Classic CJD also is distinct from "variant CJD", another prion disease that is related to bovine spongiform encephalopathy (BSE) or Mad Cow Disease.

Classic CJD characteristics, as compared to variant CJD, are presented in the table below.

Clinical and Pathologic Characteristics
Distinguishing Classic CJD from Variant CJD
Characteristic Classic CJD Variant CJD
Median age at death 68 years 28 years
Median duration of illness 4-5 months 13-14 months
Clinical signs and symptoms Dementia; early neurologic signs Prominent psychiatric/behavioral symptoms; painful dyesthesiasis; delayed neurologic signs
Periodic sharp waves on electroencephalogram Often present Often absent
"Pulvinar sign" on MRI* Not reported Present in >75% of cases
Presence of "florid plaques" on neuropathology Rare or absent Present in large numbers
Immunohitochemical analysis of brain tissue Variable accumulation Marked accumulation of protease-resistance prion protein
Presence of agent in lymphoid tissue Not readily detected Readily detected
Increased glycoform ratio on immunoblot analysis of protease-resistance prion protein Not reported Marked accumulation of protease-resistance prion protein
Source: Adapted from Belay E., Schonberger L. Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy. Clin Lab Med 2002;22:849-62.
*An abnormal signal in the posterior thalami on T2- and diffusion-weighted images and fluid-attenuated inversion recovery sequences on brain magnetic resonance imaging (MRI); in the appropriate clinical context, this signal is highly specific for vCJD.

Information of the National Institute of Neurological Disorders and Stroke's web site says there is no treatment that can cure or control CJD. Current treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks.  About 90% of patients die within 1 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.

KWWL tried contacting the Floyd County Health Department and the Iowa Department of Public Health, all of the offices are closed for the New Year's holiday.  KWWL also contacted the University of Iowa Hospitals and Clinics, the hospital would not confirm or deny the report citing HIPAA.  The Health Insurance Portability and Accountability Act is a national law protecting patient information.

"You won't see any statewide alert until test results confirm the disease," said Bruce Meisinger the Division Manager for Disease Surveillance for BlackHawk County.  "If test results come back positive, the state will likely send out an advisory on the HAN which is the Health Alert Network."

Visitation and funeral services for Thomas Squier are Saturday at Saint John Evangelical Lutheran Church in Charles City.

Tom owned EBBCO Construction for 18 years. His interests included racing, dart ball, woodworking and spending time with his family, especially his granddaughter.

KWWL will continue to follow this story and update it as we get new information.

Here are a few links with more details about Creutzfeldt-Jakob disease

Centers for Disease Control & Prevention

National Institute of Neurological Disorders and Stroke

Online Producer: JJ Murray

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