Recognizing rapid onset dementia: signs, causes and how to seek help

A dementia diagnosis can create a lot of unknowns. When you think of dementia, you might picture a condition that begins with a few signs of memory loss and progresses slowly over several years. While that’s true for some types of dementia, other forms can develop over weeks or months.

When dementia begins suddenly and progresses quickly, it’s called rapid onset dementia or rapidly progressive dementia (RPD). Early diagnosis is essential for managing RPD, so this guide can help you recognize possible signs and know when to seek help for your loved one.

What is rapid onset dementia?

Rapid onset dementia, more commonly called rapidly progressive dementia in the medical community, progresses much faster than more typical forms of dementia.

You may also hear it called sudden onset dementia. RPD commonly progresses within weeks or months, while other dementias may progress over several years.

What RPD means for your loved one depends on the underlying disease or condition causing it.

What causes rapidly progressive dementia?

Rapidly progressive dementia has many possible causes. One of the most observed causes is Creutzfeldt-Jakob disease (CJD). Other conditions can also cause RPD. The word VITAMINS offers a helpful way to remember some possible causes of rapidly progressive dementia symptoms:

• Vascular: Strokes, other vascular diseases, and vasculitis.

• Infectious: HIV, HSV, fungal infections, syphilis, parasites, Lyme disease, and Whipple disease.

• Toxic-metabolic: Vitamin deficiencies, exposure to toxic substances such as mercury or arsenic, metabolic disorders, and electrolyte imbalances.

• Autoimmune disorders: Hashimoto’s disease, lupus, and other autoimmune disorders.

• Metastases: Cancers such as primary CNS lymphoma, small cell lung cancer, and other malignancies.

• Iatrogenic: Medication toxicity or illegal drugs.

• Neurodegenerative disease: Neurodegenerative diseases such as CJD, Alzheimer’s disease, Lewy body dementia, and frontotemporal dementia.

• Systemic: Mitochondrial diseases.

Prion diseases and rapid onset dementia

Prion diseases are rare, progressive neurodegenerative disorders that affect humans and animals. One form that affects humans is Creutzfeldt-Jakob disease (CJD).

These diseases can lead to rapid cognitive decline, often described as rapid onset dementia. Abnormal prion proteins damage brain cells and destroy brain tissue, causing cognitive and motor skills to deteriorate quickly.

Signs of rapidly progressive dementia

Rapid onset dementia begins suddenly and progresses quickly. Recognizing signs of cognitive decline early may lead to proper treatment and possibly even a cure, depending on the cause. If the underlying cause of your loved one’s RPD is treatable, the associated dementia may be treatable, too.

In some cases, RPD may be the first sign of an underlying condition. For example, if RPD results from a medication problem, rapidly progressing dementia symptoms may be the first warning that something is wrong. Acting quickly can help prevent further damage or symptom progression.

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

• Difficulty performing activities of daily living (ADLs)

• Difficulty solving problems

• Confusion about time or place

• Difficulty driving

• Speech impairment

• Personality changes

• Withdrawal from social activities

Diagnosing rapid onset dementia: What to expect

As with all types of dementia, only a licensed physician can provide an accurate diagnosis of rapid onset dementia. A physician who specializes in dementia may conduct several tests and examinations. A clinician will also rule out other conditions that may cause similar symptoms. One condition referenced in connection with similar symptoms is Cerebro retinal angiopathy.

Tests and examinations

• Medical history assessment

• Blood tests

• Urinalysis

• MRI, EEG, and CT neurological tests

RPD can be challenging to diagnose. In addition to confirming that dementia is progressing rapidly, the physician must identify the underlying cause, and many possible causes have similar symptoms. As an advocate for your loved one, keep asking questions until you understand the diagnosis and the path ahead.

How is rapidly progressive dementia treated?

RPD is treatable in some cases, but not in others. If your loved one’s RPD results from a vitamin deficiency, hormonal imbalance, medication toxicity, or another treatable cause, treating the underlying condition may also help address the RPD.

In contrast, if the underlying cause is Creutzfeldt-Jakob disease, which has no cure, the associated RPD can’t be cured either.

In these situations, treatment may include medications such as antidepressants and antipsychotics to manage symptoms and improve your loved one’s quality of life.

Once your loved one has received an official RPD diagnosis, these steps can help you prepare for what comes next.

5 steps to take after a rapid onset dementia diagnosis

• Learn about RPD or prion disease: Understanding the condition and what to expect can help you prepare for the future.

• Learn about the underlying cause: Identifying the underlying cause of RPD can help your loved one receive appropriate treatment and care. In some cases, treating the cause may help eliminate the RPD.

• Choose your support team: Your support team may include physicians, therapists, other health care professionals, friends, and loved ones you can rely on.

• Consider care options: Your loved one may eventually need a type of long-term care. You can compare these settings with other available types of care. Memory care may fit your loved one’s needs. Understanding these options now may reduce frustration if your loved one’s care needs change.

• Make a plan for self-care: If you’re a family caregiver, don’t underestimate the importance of caring for yourself. Self-care may include joining a support group, exercising regularly, maintaining a healthy lifestyle, or choosing another activity that supports your physical and mental health. Make a plan that works for you.

Caregiver resources for rapid onset dementia and prion diseases

Becoming a family caregiver for a loved one with rapid onset dementia is both a significant responsibility and a privilege. Understanding the condition and the available resources can help you respond to your loved one’s needs at every stage.