What are the other Names for this Condition? (Also known as/Synonyms)

• Dementia due to Creutzfeldt-Jakob Syndrome
• Dementia due to Gerstmann Straussler Syndrome
• Dementia due to Jakob-Creutzfeldt Syndrome

What is Dementia due to Creutzfeldt-Jakob Disease? (Definition/Background Information)

• Dementia is a type of disorder accompanied by a collection of symptoms that indicate brain impairment and loss of brain function. It leads to a progressive decline of all cognitive aspects, such as behavior, memory, attention, analytical ability, thinking, language skills, etc.
• There can be a gradual deterioration of the brain’s abilities, due to age factor; or a sudden event, like an accident or illness might cause dementia
• Creutzfeldt-Jakob disease (CJD) is a rare, progressive, degenerative disease of the brain that takes place following infection of a type of naturally-occurring protein in the central nervous system, called prions. The condition has no cure and the disease prognosis is also poor
• Many individuals develop the disease spontaneously, for no evident reason. In a few cases, it may be an inherited condition. Infrequently, consuming contaminated beef or exposure to diseased human tissue can also cause CJD
• Creutzfeldt-Jakob disease leads to dementia, involuntary muscle jerks, loss of motor coordination, and psychiatric symptoms. The condition does not respond to any treatment, and is generally fatal, within 1-2 years of CJD onset
• Several types of CJD exist. All types involve an abnormal form of a naturally occurring protein in the central nervous system. These include:
  • Sporadic Creutzfeldt-Jakob Disease (sCJD)
  • Familial Creutzfeldt-Jakob Disease (fCJD)
  • Iatrogenic Creutzfeldt-Jakob Disease (iCJD)
  • New Variant (or Variant) Creutzfeldt-Jakob Disease (nvCJD or vCJD)

Who gets Dementia due to Creutzfeldt-Jakob Disease? (Age and Sex Distribution)

• Creutzfeldt-Jakob disease is extremely rare, and found worldwide with an incidence of approximately 1 case per 1,000,000 populations. Both, males and females are equally affected
• CJD generally occurs past the age of 50 years (average age 65 years). It is uncommon in individuals below this age range
• However, variant CJD (vCJD, a type of CJD) may affect individuals in their 20s (and older) too
• No race or ethnic preference has been observed

What are the Risk Factors for Dementia due to Creutzfeldt-Jakob Disease? (Predisposing Factors)

There are a host of factors that could potentially increase the risk for Creutzfeldt-Jakob disease, and hence for dementia associated with it. Some may be controlled, while others are unchangeable and often relentless:

• Age - this factor may augment the risk
• Genetic causes, having a family history of CJD
• Exposure to infectious prions, during:
  • Consumption of contaminated beef
  • Medical treatments involving tissues or blood products from an infected donor with unknown CJD (e.g., organ transplant/graft, treatment with a hormone preparation, etc.)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dementia due to Creutzfeldt-Jakob Disease? (Etiology)

Most cases of dementia have not been understood; particularly, as to how certain disorders/inherited conditions influence dementia-development. Nevertheless, the causal factors of Dementia due to Creutzfeldt-Jakob Disease may include:

• Transmission of prions from an infected individual or animal product (such as beef)
• Spontaneous genetic mutation that creates abnormal prion protein, which may be responsible for both sporadic and familial cases of CJD
• Accidental transmission of prion proteins after organ transplants (e.g. corneas, brain-part grafts), EEG electrode implantation, and other surgeries. It is believed that improperly decontaminated equipment could cause an infection, in such cases

Creutzfeldt-Jakob disease causes a type of rapidly progressive dementia, where a sudden onset and advancement of the condition, leads to serious complications.

What are the Signs and Symptoms of Dementia due to Creutzfeldt-Jakob Disease?

Dementia occurs inevitably due to Creutzfeldt-Jakob disease, the presentations of which may be mild initially. But, the signs and symptoms may deteriorate quickly. These include:

• Initial experiences include fatigue, sleep disturbances, hallucinations, weight loss, headache and pain, seizures
• Loss of basic motor (physical coordination) skills, such as difficulty walking, maintaining balance, swallowing difficulties, speech and vision difficulties
• Sudden, loud noises or bright light may trigger involuntary body spasms (myoclonus); a condition that may also occur during sleep
• Progressive deterioration of the senses, memory loss, confusion, and impaired judgment; these lead to profound dementia that is observed after some time
• Changes in overall personality - regression or absence of reasoning/rationale, agitation, delusions, depression
• Extremely irrational mental/emotional/physical behavior (these may be completely inappropriate for the situation)
• Loss of social abilities, social awkwardness

How is Dementia due to Creutzfeldt-Jakob Disease Diagnosed?

To examine for Dementia due to Creutzfeldt-Jakob Disease, a variety of tests are usually conducted. The prescribed course of evaluation is usually based on the signs and symptoms. Symptoms of dementia, myoclonus, and an abnormal EEG in a middle-aged patient, suggest a diagnosis of CJD. The main diagnostic tools include:

• Physical exam with a comprehensive evaluation of medical history
• Neurological, motor skills, and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
• Imaging studies that may be performed include: MRI scan of the CNS (brain and spine), CT scan (head)
• Electroencephalography (EEG)
• Blood analysis
• Cerebrospinal fluid analysis, where a spinal tap test may be performed
• CJD may be confirmed by brain tissue biopsy and assays that detect abnormal prion proteins.  If a biopsy is negative, the diagnosis of CJD may not be ruled out, because prion proteins may be absent from a small tissue sample. In this case, a final diagnosis cannot be made until an autopsy is conducted
• A differential diagnosis may be considered, in order to eliminate other similar conditions

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dementia due to Creutzfeldt-Jakob Disease?

Complications of Dementia due to Creutzfeldt-Jakob Disease are:

• The main complication, which occurs on account of memory loss and neurological function impairment, is that institutionalized care might be required for prolonged periods, or for the rest of the individual’s life. There is a progressive inability of the individual to take care of themselves, due to a complete degeneration of their mental and emotional health
• Individuals frequently develop pneumonia during the later stages, which is often fatal
• Loss of inhibition
• Spontaneous incontinence (involuntary bowel or bladder movement)
• Loss of sleep
• Decreased level of personal safety principles (lack of awareness)
• Complete loss of personality; paranoia, depression, irrational aggression/anxiety, delirium
• The individual remain susceptible to opportunistic infections, due to a reduced sense of hygiene, decreased intake of food/nutrition
• Pain and its persistence, that could remain untreated due to poor communication skills
• Treatment medication side effects

How is Dementia due to Creutzfeldt-Jakob Disease Treated?

There is no cure or standard treatment for Dementia due to Creutzfeldt-Jakob Disease. Patients receive supportive care and may be given medications to control myoclonus and pain, if present. Dementia management (due to CJD) is undertaken on a case-by-case basis.

Some of the treatment methods include:

• Ensuring that the individual gets a nutritious diet with medical supplements (include proteins, fiber-rich fruits and vegetables, milk shakes, juices, and plenty of water), is well-cared for in a safe environment (whether at home or outside), and is under regular/constant care and support
• Use of medications to slow progression of CJD, such as interleukins
• Use of painkillers, if required
• Treatments in development include the use of antibodies against prions and several drugs with potential therapeutic efficacy

How can Dementia due to Creutzfeldt-Jakob Disease be Prevented?

• Dementia due to Creutzfeldt-Jakob Disease is not preventable. Nevertheless, the progression of the condition may be delayed to some extent
• Creutzfeldt-Jakob disease is not a ‘readily’ contagious condition, but it may be transmitted to others, in the absence of due care, when handling brain tissue. For example, prions may be transmitted to healthcare workers through accidental needle sticks or stab wounds. It is very important to sterilize by autoclave any equipment that has had a contact with brain tissue from patients with CJD

What is the Prognosis of Dementia due to Creutzfeldt-Jakob Disease? (Outcomes/Resolutions)

• Early detection and aggressive management of Creutzfeldt-Jakob disease (and the dementia associated with it) is essential, in all forms of the disease. This may prevent a rapid deterioration of the condition and help sustain the quality of life, for some more additional time
• Patients diagnosed with CJD may live anywhere between 6 months to 5 years (in rare cases), following the onset of symptoms
• Death usually results in less than 1 year (normally in about 7-8 months), while 5-10% of patients survive more than 2 years
• Patients with familial CJD tend to live longer than those with sporadic CJD

Additional and Relevant Useful Information for Dementia due to Creutzfeldt-Jakob Disease:

There are voluntary organizations and support groups that provide counsel, help, and understanding to the individuals and families of the Creutzfeldt-Jakob disease affected.