Creutzfeldt-Jakob Disease (CJD) in Grand Rapids, Michigan

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive brain disorder. It belongs to a group of conditions called transmissible spongiform encephalopathies (TSEs), or prion diseases, which affect both humans and animals.

In people living in Grand Rapids and across West Michigan, CJD leads to progressive destruction of brain tissue, often causing:

  • Rapidly worsening dementia
  • Problems with balance and walking
  • Muscle stiffness and involuntary movements

Most people with CJD die within six months of symptom onset, and almost all die within two years.

CJD Has No Known Cure

At this time, there is no treatment or cure for Creutzfeldt-Jakob disease. Care in Grand Rapids focuses on:

  • Managing symptoms (for example, with neurologists at Spectrum Health or Trinity Health Grand Rapids)
  • Supporting families and caregivers
  • Ensuring safe infection-control practices in hospitals and surgical settings

Because CJD is so rare and serious, suspected cases are usually managed by specialists in neurology and infectious disease at major health systems such as Spectrum Health, Metro Health, Mercy Health, or Trinity Health Grand Rapids.

How Common Is CJD?

CJD affects about one person per million people per year worldwide.

  • In a population of 10 million, there are typically around 10 cases each year.
  • It most often affects people between 50 and 70 years of age.

In the Grand Rapids metropolitan area (population 200,000+ in the city and many more in the surrounding region), CJD remains extremely rare, but local hospitals are prepared to evaluate and manage suspected cases.

Types of Human Prion Diseases

Human prion diseases include:

  • Sporadic CJD – the most common form (85–90% of cases)
  • Genetic or familial CJD – caused by inherited mutations (10–15% of cases)
  • Variant CJD (vCJD) – linked to bovine spongiform encephalopathy (BSE or “mad cow disease”)
  • Medically acquired CJD – from certain medical procedures or tissue products (now extremely rare)
  • Kuru – historically seen only in Papua New Guinea, related to ritual cannibalism (no longer occurring)

In the United States, including Michigan, sporadic CJD is by far the most common form.

Sporadic CJD: Cause Is Still Unknown

Sporadic CJD arises without a clear trigger. The exact cause is unknown.

Scientists believe that, in some people, the normal prion protein in the brain spontaneously changes shape into an abnormal form. This abnormal protein then acts like a “template,” causing other normal prion proteins to misfold and accumulate, leading to brain damage and cell death.

Factors that may play a role include:

  • Genetic susceptibility in some individuals
  • Random (spontaneous) changes in prion protein structure

No environmental, lifestyle, or seasonal factor related to West Michigan’s climate (such as cold winters or lake-effect weather) has been shown to cause CJD.

Genetic (Familial) CJD

Genetic prion disease is very rare. It is caused by inherited changes (mutations) in the prion protein gene.

Key points:

  • It is passed from parent to child at conception.
  • Each child of an affected parent has a 50% chance of inheriting the mutation.
  • It is often recognized because multiple family members (siblings, parents, children) develop similar symptoms.

Families in the Grand Rapids area who have a history of genetic CJD may be referred to a clinical geneticist (often through Spectrum Health or University of Michigan-affiliated specialists) for genetic counseling and testing.

Variant CJD and “Mad Cow Disease”

Variant CJD (vCJD) was first recognized in 1996 in the United Kingdom and is linked to an epidemic of bovine spongiform encephalopathy (BSE) in cattle, often called “mad cow disease.”

Important points for residents of Grand Rapids and Michigan:

  • Variant CJD is associated with eating BSE-contaminated beef products.
  • People with variant CJD tend to be younger than those with sporadic CJD.
  • The illness often lasts longer (up to a year or more) and has some distinct symptoms and brain changes on autopsy.

In the United States:

  • BSE has not occurred in American cattle in the way it did in the UK.
  • Variant CJD remains extremely rare and has not been found to be acquired from U.S. beef.
  • Federal and state food safety regulations, including those overseen by the USDA and Michigan Department of Agriculture & Rural Development, help protect the food supply.

Medically Acquired CJD

Medically acquired CJD has occurred worldwide due to certain medical treatments in the past. These are now almost entirely preventable due to modern safety standards.

Documented sources have included:

  • Contaminated neurosurgical instruments
  • Dura mater grafts (tissue used to repair the membrane covering the brain)
  • Corneal transplants (very rare – only a few cases worldwide)
  • Pituitary hormone extracts from human cadavers used in older treatments for infertility or short stature

Once the risk was recognized:

  • Human-derived pituitary hormones were replaced by synthetic alternatives.
  • Sterilization procedures for neurosurgical instruments were strengthened.
  • Organ and tissue transplant guidelines were tightened.
  • Patients are screened for CJD risk before certain neurosurgical procedures.

In the United States, there have been a small number of medically acquired CJD cases, but no new cases from these sources have been reported for many years. Hospitals in Grand Rapids follow national infection-control standards to minimize any risk.

CJD and Blood Transfusions

For sporadic and medically acquired CJD, there is no evidence that routine blood products cause disease transmission.

However, variant CJD has been shown to be transmissible by blood transfusion. This has been demonstrated in:

  • Laboratory experiments
  • Real-world cases in the United Kingdom

Because of this, U.S. blood banks, including those serving Grand Rapids and West Michigan, follow strict donor deferral guidelines. People may be deferred from donating blood if they:

  • Lived in the United Kingdom for six months or more between 1980 and 1996
  • Received a blood transfusion in the UK since January 1, 1980
  • Have certain medical histories suggesting increased CJD risk (e.g., some neurosurgical procedures with dura grafts, or specific family histories of prion disease)

Local blood collection centers in West Michigan (such as Versiti Blood Center of Michigan) follow these national and international safety standards.

How CJD Is Diagnosed

CJD is difficult to diagnose, especially in the early stages.

Challenges include:

  • Early symptoms can be vague and resemble other conditions (such as Alzheimer’s disease, stroke, or other neurologic disorders).
  • There is no single blood test that can definitively diagnose CJD in the early stages.
  • Many other treatable conditions must be ruled out first.

In Grand Rapids, evaluation usually involves a neurologist and may include:

  • Detailed neurological exam and medical history
  • MRI of the brain
  • EEG (electroencephalogram)
  • Cerebrospinal fluid (CSF) tests, sometimes including specialized prion protein tests
  • Blood tests to exclude other causes

Definitive Diagnosis

A definite diagnosis of CJD can only be made by examining brain tissue under a microscope, usually:

  • At autopsy, after death

This confirms the characteristic spongiform (sponge-like) changes and abnormal prion protein deposits.

Symptoms of Creutzfeldt-Jakob Disease

Symptoms result from progressive damage to brain cells. They often worsen quickly over weeks to months.

Common symptoms include:

  • Confusion or disorientation, rapidly progressing to severe dementia
  • Memory loss
  • Personality and behavior changes
  • Mood changes, including depression or anxiety
  • Weakness and loss of muscle control
  • Loss of balance and coordination, causing difficulty walking and frequent falls
  • Muscle spasms and jerking movements (myoclonus)
  • Visual problems, such as double vision or loss of vision

As the disease advances, people typically become:

  • Unable to walk or care for themselves
  • Unable to speak clearly
  • Bedbound and completely dependent on caregivers

In West Michigan, patients with suspected CJD are often cared for in collaboration between hospital teams (neurology, palliative care) and community-based services such as home health or hospice.

How Prion Proteins Cause Brain Damage

In a healthy brain, normal prion protein is produced, but its exact function is not fully understood.

In CJD:

  • The normal prion protein changes shape into an abnormal form.
  • This abnormal protein is resistant to breakdown and accumulates in brain tissue.
  • It triggers a chain reaction, converting more normal protein into the abnormal form.
  • Over time, this leads to cell death, loss of brain tissue, and the “spongy” appearance seen on microscopic examination.

The abnormal prion protein is also extremely resistant to:

  • Heat
  • Standard chemical disinfectants
  • Routine hospital sterilization procedures that kill bacteria and viruses

Because of this, special sterilization protocols are recommended for instruments exposed to high-risk tissues (such as brain and spinal cord), but these cannot be used routinely on all instruments because they can damage equipment.

Is CJD a Virus?

CJD has sometimes been called a “slow virus” disease because of its long incubation period and progressive course. However:

  • No virus has ever been identified as the cause.
  • The prion protein itself is believed to be the infectious agent.
  • The viral theory remains unproven.

How CJD Is (and Is Not) Transmitted

Not Spread by Casual Contact

CJD is not spread through everyday social or household contact. It is not transmitted by:

  • Hugging, touching, or caring for someone with CJD
  • Kissing
  • Sharing cups, utensils, or dishes
  • Casual contact at work, school, or in the community

Family members and caregivers in Grand Rapids can safely provide physical and emotional support without fear of catching CJD through routine contact.

Possible Routes of Transmission

Transmission has been documented through:

  • Certain medical procedures (now tightly controlled)
  • Contaminated surgical instruments used on brain or spinal cord
  • Human-derived tissue products (such as older dura grafts or pituitary hormones)
  • Blood transfusion in the case of variant CJD (not sporadic CJD)

Standard infection-control practices at Grand Rapids hospitals are designed to minimize any theoretical risk.

Public Health and CJD in Michigan

In the United States, CJD is a notifiable disease, meaning:

  • Doctors and hospitals must report newly diagnosed cases to local and state health departments.

For residents of Grand Rapids and Kent County:

  • Suspected or confirmed cases are reported to the Kent County Health Department.
  • Public health officials may coordinate with the Michigan Department of Health and Human Services and the Centers for Disease Control and Prevention (CDC) to monitor and investigate cases.

This reporting helps:

  • Track how often CJD occurs
  • Identify any unusual patterns
  • Ensure appropriate public health responses

Why Hospitals May Ask About CJD Risks

Before certain surgeries or procedures, especially involving the brain, spine, or eyes, hospitals in Grand Rapids may:

  • Ask about any personal or family history of CJD
  • Review past neurosurgical procedures or tissue grafts
  • Screen for blood donation deferrals related to time spent in the UK or other risk factors

This screening helps:

  • Protect other patients by ensuring proper sterilization or instrument management
  • Guide decisions about tissue and organ donation

Living in Grand Rapids: What Residents Should Know

For people in Grand Rapids, Michigan:

  • CJD is extremely rare.
  • There is no evidence that CJD is linked to local environmental factors, Great Lakes water, or typical West Michigan foods.
  • Routine activities—school, work, healthcare visits, outdoor recreation along the Grand River or Lake Michigan—do not pose a CJD risk.

However, because CJD is so serious, anyone experiencing rapidly worsening memory loss, confusion, or difficulty walking should see a doctor promptly. Early evaluation can:

  • Rule out more common and treatable conditions (such as stroke, vitamin deficiencies, medication effects, or other dementias)
  • Allow referral to a neurologist if needed

Where to Get Help in Grand Rapids

If you are concerned about CJD or rapidly progressive dementia, consider:

  • Your primary care doctor or GP
    They can perform an initial evaluation and refer you to a neurologist.

  • Local neurology clinics and hospitals

    • Spectrum Health (Corewell Health) – Neurology and Neurosciences
    • Trinity Health Grand Rapids – Neurology services
    • Metro Health – University of Michigan Health
    • Mercy Health facilities in West Michigan

  • Kent County Health Department
    For public health information and disease reporting support.

  • National CJD Support Organizations
    These groups offer information, support, and resources for families affected by prion diseases.

If you have a strong family history of CJD or other prion diseases, ask your doctor about a referral to a clinical geneticist for counseling and possible genetic testing.

Key Points About CJD for Grand Rapids Residents

  • CJD is a rare, rapidly progressive brain disease that usually leads to death within six months to two years.
  • There is no cure or specific treatment, but supportive and palliative care are available locally.
  • The cause of sporadic CJD is unknown; most cases are not inherited or linked to medical procedures.
  • Genetic CJD is inherited and gives each child of an affected parent a 50% chance of carrying the mutation.
  • Variant CJD is linked to BSE (“mad cow disease”) and has been mainly a concern in the UK, not in U.S.-raised cattle.
  • CJD is not spread by casual contact such as touching, hugging, or sharing dishes.
  • U.S. and Michigan blood donation and surgical guidelines help protect against any potential transmission.
  • CJD is a notifiable disease, and suspected cases in Grand Rapids are reported to the Kent County Health Department and state authorities.

If you or a loved one in Grand Rapids is facing a possible diagnosis of CJD, work closely with your healthcare team, ask about available support resources, and consider connecting with national CJD support organizations for additional guidance.