Recognizing Rapid Onset Dementia: Signs, Causes and How To Seek Help

When we think of dementia, we usually think of a disease that starts slowly with a few signs of memory decline and progresses slowly over several years. While that is true for most dementias, it can be shocking to discover that some dementias develop over weeks or months and can quickly be fatal. 

When dementia begins quickly and progresses rapidly, it is called rapid onset dementia or rapidly progressive dementia (RPD). Since early diagnosis is essential in managing RPDs, we’ve created this guide to help you recognize the signs early and be ready to seek help.  

What is rapid onset dementia?

Rapid onset dementia, most commonly referred to in the medical community as rapidly progressive dementia, is dementia that progresses much more quickly than slower, more typical types of dementia.

You may also hear it called sudden onset dementia. It is common for RPDs to progress within weeks or months, while other dementias may progress over the course of a few years. 

What RPD means for your loved one depends on the underlying disease that is causing it. 

Uncovering the roots: what can cause rapid onset dementia?

Rapidly progressive dementia has many possible causes. One of the most commonly observed causes of RPD is a type of dementia called Creutzfeldt-Jakob disease (CJD). However, there are other causes of RPD. Here is a breakdown of some possible causes of rapid onset dementia, or rapidly progressive dementia symptoms, using the word VITAMINS as a helpful mnemonic. 

• Vascular: Strokes, or other vascular diseases and vasculitis. 
• Infectious: HIV, HSV, fungal infections, syphilis, parasites, Lyme disease, Whipple’s Disease.
• Toxic-Metabolic: Vitamin deficiencies, exposure to toxic substances like mercury or arsenic, metabolic disorders, and electrolyte imbalance. 
• Autoimmune Disorders: Hashimotos, Lupus, etc. 
• Metastases: Cancers like primary CNS lymphoma, small cell lung cancer, and other malignancies. 
• Iatrogenic: Medicinal toxicity or illegal drugs. 
• Neurodegenerative disease: Some neurodegenerative diseases may be part of it, such as CJD, Alzheimer’s disease, Lewy Body Dementia, Frontotemporal dementia, etc.
• Systemic: Mitochondrial diseases. 

Prion diseases and rapid onset dementia

Prion diseases are rare, progressive, neurodegenerative disorders that affect humans and animals. One form in humans is Creutzfeldt-Jakob disease (CJD). These diseases lead to rapid cognitive decline, often described as rapid onset dementia. The abnormal prion proteins damage brain cells, destroy brain tissue, leading to rapid deterioration of cognitive and motor skills. 

Recognizing the signs of rapid onset dementia, or rapidly progressive dementia

Rapid onset dementia begins suddenly and progresses quickly. Recognizing the symptoms of cognitive decline early can mean getting proper treatment and possibly even a cure, depending on the cause of the RPD. In other words, if the cause of your loved one’s rapid onset dementia is treatable, the associated dementia may be, too. 

In some cases, RPD may also be the first sign of the underlying cause. For example, if someone experiences RPD that results from an issue with the medication they’re taking, the signs of rapidly progressive dementia may be the first warning sign that something is wrong. Acting fast can prevent further damage or symptom progression.  

If your loved one is showing sudden warning signs of dementia that appear over few weeks or months and seem to be worsening quickly, they may have rapidly progressive dementia. Common symptoms include:

• Difficulty performing activities of daily living  (ADL)
• Inability to problem solve
• Confusion about time or place
• Driving issues
• Speech impairment
• Personality changes
• Withdrawal from social activities

Diagnosing rapid onset dementia: what to expect

As with all types of dementia, the only way to get an accurate diagnosis of rapid onset dementia is from a licensed medical doctor. A doctor who specializes in dementia will conduct various tests. Here are some texts and examinations to expect. The clinician will also exclude other potential diseases that may have similar symptoms, such as cerebroretinal angiopathy.

Physical exam 

• Assessment of medical history 
• Blood labs
• Urinalysis
• MRI, EEG, and CT (neurological tests)

RPD can be challenging to diagnose because, in addition to identifying the rapid progression of dementia, the underlying cause must be identified, and many possible causes have similar symptoms. As an advocate for your loved one, don’t give up until you get answers and an understanding of the path ahead. 

Now what? How are rapidly progressive dementias treated?

RPD is treatable in some cases, and in others, it is not. For example, if your loved one’s RPD results from a vitamin deficiency, a hormonal imbalance, medicinal toxicity, or other treatable causes, treating the underlying condition can also help take care of the RPD. 

In contrast, if the rapid dementia progression has an underlying cause like Creutzfeldt-Jakob disease for which there is no cure, the associated RPD cannot be cured either.

In these situations, treatment consists of medications, like antidepressants and antipsychotics, to help treat the symptoms and improve your loved one’s quality of life.

Here is a checklist of things you can do to get ahead once you’ve gotten an official RPD diagnosis.

5 Steps to take if you've received a diagnosis of rapid onset dementia

• Learn about RPD or prion disease: Understanding the condition and what to expect from it will help you better prepare for the future.
• Learn about the underlying cause: Diagnosing the underlying cause of RPD will help ensure proper treatment and care and possibly even assist in eliminating the RPD.
• Select your support team: This includes physicians, therapists, other healthcare professionals, and other friends and loved ones you can rely on. A solid support team will be vital. 
• Consider care options: Whether your loved one will need in-home care, assisted living, or memory care, starting the journey with an understanding of the options will reduce frustration if that time comes.
• Make a plan for self-care: As a caregiver, don’t underestimate the power of caring for yourself. This may include joining support groups, focusing on regular exercise and a healthy lifestyle, or anything else that helps to keep your physical and mental health strong. Make a plan.

Caregiver resources for rapid onset dementia and prion diseases

Stepping into the role of caregiver for a loved one with rapid onset dementia is both a significant responsibility and a privilege. Understanding the condition and the resources available will help you embrace your loved one's needs at every stage.

At Seniorly, we exist to help you in your caregiving journey by empowering you with the resources, knowledge, and connections necessary to ensure your loved one the best care possible. Take a look below to find suggestions for additional reading.