Mad Cow-Like Illness Kills US Woman After 50-Year Dormancy, Know What It Is, How It Stayed Quiet For 50 Years

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In an astonishing medical case, a woman succumbed to a fatal prion disease, marking potentially the longest incubation period for such a condition ever recorded.

This case, highlighted in the journal Emerging Infectious Diseases, involved a 58-year-old who developed Creutzfeldt-Jakob disease (CJD) approximately five decades after receiving contaminated human growth hormone (HGH) treatments as a child. The incident underscores the perplexing and often insidious nature of prion diseases, which can lie dormant for years before manifesting.

Prion diseases, including CJD, are caused by the abnormal folding of normal prion proteins in the brain, leading to a cascade of neurodegenerative effects. These conditions are notoriously difficult to prevent or cure, given prions' resistance to conventional sterilization methods that effectively neutralize other pathogens. While prion diseases are exceptionally rare, their impact is devastating, often resulting in rapid cognitive decline and death shortly after symptoms emerge.

The unfortunate death of the woman, who first presented with tremors and balance issues, rapidly deteriorated into a comatose state from which she never recovered. Her autopsy confirmed CJD without any genetic predispositions, pointing to her childhood HGH treatments as the likely source. This revelation is alarming, as it suggests that prion diseases can incubate for an extended period, in this case, potentially over 50 years, before showing any clinical signs.

Medical History And The Spread Of Prion Diseases

Historically, CJD and other prion diseases have been transmitted through various means, including consumption of infected beef and medical treatments like HGH derived from human cadavers. The latter practice was ceased in the U.S. and other countries following the discovery of its link to CJD, shifting towards synthetic alternatives. Despite this change, over two hundred cases of iatrogenic CJD, resulting from medical intervention, have been documented globally, with most cases emerging within a decade of treatment. However, as evidenced by this report, the latency period can extend much longer.

The U.S. altered its HGH extraction method in 1977, significantly reducing the risk of prion contamination. Nonetheless, the woman's initial and final treatments spanned from 51.3 years to 42.1 years before symptom onset, with a probable infection timeline estimated at about 48.3 years prior. This case, therefore, not only sets a record for the latency period of HGH-related CJD but also raises concerns about potential future cases. Individuals who received HGH before the 1977 method change remain at risk.

The presence of genetic factors can influence one's susceptibility to prion diseases, as the woman in question had a genetic mutation associated with prolonged latency periods. This suggests that while the incidence of CJD due to contaminated HGH has dramatically declined, the risk persists for some, notably those treated before 1977. Researchers estimate that around 7,700 people in the U.S. received HGH treatments before the implementation of safer practices, indicating a need for continued vigilance.

Let us know more about Creutzfeldt-Jakob disease (CJD) and the causes, symptoms, precautions and treatments associated with it.
Creutzfeldt-Jakob Disease (CJD) is a rare, fatal neurodegenerative disorder that has puzzled scientists and clinicians for decades. Often likened to "mad cow disease" in humans, CJD is caused by misfolded proteins known as prions, which lead to rapid brain deterioration. Despite its rarity, the disease's swift progression and lack of effective treatment make it a significant concern in neurology and public health.
CJD is part of a group of diseases called transmissible spongiform encephalopathies (TSEs), characterized by sponge-like changes in brain tissue. The disease manifests in several forms:

• Sporadic CJD (sCJD): Accounts for approximately 85% of cases and arises spontaneously without known cause.
• Familial CJD (fCJD): Inherited through mutations in the PRNP gene, representing about 10-15% of cases.
• Iatrogenic CJD: Results from exposure to contaminated medical equipment or procedures, such as corneal transplants or hormone treatments.
• Variant CJD (vCJD): Linked to consuming beef products infected with bovine spongiform encephalopathy (BSE), commonly known as mad cow disease.

CJD Causes And Transmission

The primary culprit in CJD is the prion protein, which, when misfolded, induces other normal prion proteins to also misfold, leading to a chain reaction of brain damage. Unlike bacteria or viruses, prions lack nucleic acids, making them resistant to standard sterilization processes.
Transmission can occur through:

• Genetic inheritance: Mutations in the PRNP gene can be passed down, leading to familial CJD.
• Contaminated medical procedures: Use of infected surgical instruments or biological products.
• Consumption of infected meat: Particularly in cases of vCJD linked to BSE-infected beef.

CJD Symptoms And Progression

CJD is notorious for its rapid progression. Initial symptoms often include:
Cognitive decline: Memory loss, confusion, and impaired judgment.
Behavioral changes: Personality shifts, anxiety, and depression.
Physical symptoms: Muscle stiffness, involuntary movements (myoclonus), and coordination problems.

As the disease advances, patients may experience severe dementia, blindness, and eventually lapse into a coma. The median survival time after symptom onset is approximately one year.

CJD Diagnosis

Diagnosing CJD involves a combination of clinical evaluation and specialized tests:
Electroencephalogram (EEG): Detects characteristic brain wave patterns associated with CJD.
Magnetic Resonance Imaging (MRI): Identifies specific changes in brain tissue.
Cerebrospinal fluid tests: Detects proteins indicative of prion disease.

Definitive diagnosis often requires brain tissue examination, typically post-mortem.

CJD Treatment And Management

Currently, there is no cure for CJD. Treatment focuses on alleviating symptoms and providing supportive care:
Medications: Sedatives and antidepressants can help manage psychological symptoms, while drugs like clonazepam may reduce muscle jerks.
Palliative care: Ensures patient comfort, addressing pain and other distressing symptoms.

CJD Precautions and Prevention

Preventing CJD involves stringent measures, especially in medical settings:
Sterilization protocols: Ensuring surgical instruments are properly sterilized to eliminate prion contamination.
Screening: Careful screening of blood and tissue donors to prevent iatrogenic transmission.
Food safety: Regulations to prevent BSE-infected meat from entering the food supply have significantly reduced vCJD cases.

Disclaimer: The information provided in this article is for general informational and educational purposes only and is not intended as a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or a qualified healthcare provider with any questions you may have regarding a medical condition.