What is Pick Disease?
Frontotemporal dementia (FTD) is a condition that worsens over time, resulting in changes in behavior and language abilities. It occurs due to the deterioration of the front and side areas of the brain. This illness can vary quite a bit in how it appears in different people because of differences in clinical signs, genetics, and causes, all of which contribute to a person’s specific form of the disease. It’s also often called Pick’s disease and usually starts affecting people before they reach old age.
There are different types of this dementia, one of which affects behavior mainly (behavioral variant or bvFTD), while another primarily affects language abilities (primary progressive aphasia or PPA). The language-affecting subtype is further divided into three categories: nonfluent/agrammatic variant (nfvPPA), which involves difficulty constructing sentences; semantic variant (svPPA), which involves difficulty understanding words; and logopenic aphasia, which affects a person’s ability to retrieve words and form sentences.
There are also a few other conditions related to FTD, including a motor neuron disease (FTD-MND), progressive supranuclear palsy (PSP-S), and corticobasal syndrome (CBS) which have overlapping symptoms and fall within the range of possible FTD diagnoses.
The progression of frontotemporal dementia usually begins subtly and then worsens steadily. From the time of diagnosis until it finally causes death, this condition causes a great deal of physical, mental, social, and financial strain on patients, their families, and caregivers.
What Causes Pick Disease?
Most of the time, frontotemporal dementia just happens without a specific reason. However, the illness can run in families, and research shows that up to 40% of people who have it have had a relative with dementia. Additionally, 10% to 20% of patients have certain gene changes that are passed down from parents to children.
The main genes linked to frontotemporal dementia are microtubule-associated protein tau (MAPT), progranulin (GRN), and C9orf72 plus a few others that aren’t as common.
Not much is known about what might increase a person’s risk of developing frontotemporal dementia, but some studies suggest factors like having diabetes, suffering a head injury, or having an autoimmune disease might contribute. Other things that have been looked at include high blood pressure, smoking, obesity, and a history of strokes.
More research is needed to confirm these links and offer advice on lifestyle changes that might reduce the chance of getting frontotemporal dementia.
Risk Factors and Frequency for Pick Disease
Frontotemporal dementia is the second most commonly diagnosed form of dementia in people younger than 65, accounting for 10% of confirmed cases. It represents 3% of dementia cases across all ages. Although it’s more common in people in their 50s and 60s, it can also affect people in their thirties or over seventy.
- 60% of frontotemporal dementia is the behavioral variant, while a subtype called primary progressive aphasia occurs less often.
- Estimates of how common it is range from 15 to 22 cases per 100,000 people, and 2.7 to 4.1 new cases per 100,000 people annually.
- These numbers may be lower than the actual rate of the disease due to challenges in diagnosing it.
- The rates of frontotemporal dementia are similar globally, but there’s not much information available about its prevalence in developing countries or among non-white populations.
- Studies have found no difference in how often the disease occurs in men versus women.
Signs and Symptoms of Pick Disease
: Frontotemporal dementia refers to a group of disorders that cause changes in behavior and language abilities. These changes occur due to the deterioration of the brain’s frontal and temporal lobes. The signs and symptoms usually differ based on the type of dementia, but they tend to overlap as the disease progresses. To diagnose this condition, doctors need a detailed account of the symptoms from the patient and their family.
The behavioral type of frontotemporal dementia, or bvFTD, manifests through six main categories of behavioral changes or cognitive decline, which include:
- Inappropriate social behavior, loss of manners, or reckless actions
- Apathy – which is often misinterpreted as depression
- Indifference or insensitivity towards others’ feelings
- Recurrent movements or speech, obsessive routines
- Changes in eating habits, including consuming more sweets and alcohol, or exploring objects orally
- Difficulties with high-level thinking tasks while memory and visual abilities often remain unaffected
Diagnosing bvFTD requires the manifestation of at least three out of these six symptoms.
In contrast, primary progressive aphasia (PPA), a language-focused type of frontotemporal dementia, divides into three categories: nonfluent primary progressive aphasia (nfvPPA), semantic variant primary progressive aphasia (svPPA), and logopenic aphasia. Their symptoms include:
- nfvPPA: Struggling speech, mispronunciation, and grammar mistakes. Ability to understand words and knowledge of objects is usually preserved, but understanding complex sentences might be challenging.
- svPPA: Trouble naming things and understanding single words. Diagnosing requires experiencing at least three out of four additional symptoms: trouble recognizing objects, difficulty reading aloud, writing issues, ability to repeat phrases, and unaffected speech flow.
- Logopenic variant PPA: Difficulty finding words, repeatedly saying sentences and expressions. Mispronunciations may occur while understanding of words, object knowledge, speech motor control, and basic grammar tends to remain intact.
However, these symptoms often mix as the disease progresses and affect different areas of the brain.
Further complexity comes with syndromes related to frontotemporal dementia such as the motor neuron disease, corticobasal degeneration, and progressive supranuclear palsy. These conditions lead to movement impairments and can also feature the behavioral and language-related symptoms found in frontotemporal dementia. The diagnosis also involves the understanding of these overlapping elements.
Testing for Pick Disease
Medical professionals evaluating suspected cases of frontotemporal dementia primarily rely on imaging techniques and fluid markers in the body. Currently, standard imaging such as a structural magnetic resonance imaging (MRI) scan or a computed tomography (CT) scan can be used to check for any shrinkage in the frontal and temporal lobes of the brain, which is common in frontotemporal dementia.
Moreover, doctors are keen on employing some advanced imaging techniques. These include diffuse tensor imaging that examines loss of white matter (the part of your brain responsible for learning and function), fluorodeoxyglucose positron emission tomography (FDG-PET) that monitor’s the brain’s metabolism, amyloid and tau PET traces that look for abnormal protein build-up in the brain, arterial spin labeling (ASL) that measures blood flow in the brain, and resting-state functional MRI (RS-fMRI) that investigates the connectivity between different parts of the brain.
The application of these techniques to diagnose frontotemporal dementia and monitor its progression are still under study, and also figuring out whether it’s Alzheimer’s or frontotemporal dementia a patient is suffering from.
Along with imaging techniques, fluid markers found in the body can also deliver important insight into diagnosing frontotemporal dementia. These involve testing cerebrospinal fluid (the clear fluid in the spinal cord and brain) and serum protein (a component of blood) to exclude Alzheimer’s disease. In particular, elevated T-tau proteins and decreased beta-amyloid 42 protein levels in cerebrospinal fluid are strong indicators of Alzheimer’s disease and can help rule out frontotemporal dementia.
Neurofilament light chain (NFL) proteins found in cerebrospinal fluid and serum samples show promise in diagnosing frontotemporal dementia and other neurological disorders. Scientists are also exploring the use of biomarkers related to specific genes, such as progranulin and poly (GP), to examine unique mutations related to frontotemporal dementia. As with the new imaging techniques, more research is needed to develop a comprehensive strategy for diagnosing and monitoring frontotemporal dementia using fluid biomarkers.
Treatment Options for Pick Disease
At present, there is no cure for frontotemporal dementia and no specific treatments that can halt its progression. Therefore, treatments focus on managing the symptoms and offering support to patients, their families, and caregivers.
Medication may be used to help manage some of the behavior symptoms associated with this condition. For instance, certain types of antidepressants known as selective serotonin reuptake inhibitors (SSRIs) can help. Low-dose atypical antipsychotics, a kind of medication used to control aggressive or disruptive behaviors, might be recommended for severe cases. Care must be taken with these drugs especially in elderly patients, as they come with a strong caution related to heart problems.
Medications typically used for Alzheimer’s disease, called cholinesterase inhibitors, do not work for frontotemporal dementia and may cause behavioral problems to worsen.
Non-drug interventions also play a crucial role in managing symptoms and slowing the progression of the disease. Physical therapy and exercise can help slow cognitive decline. Speech therapy may help improve language deficits for patients who have trouble with communication. As patients’ daily functions decrease, they will need support to maintain a safe home environment. Consistent application of non-drug behavioral management strategies may also ease the physical, mental, and social impacts of the disease.
Education for both the patient and caregiver is extremely important. It can help remove feelings of guilt and the stigma associated with the behavior changes caused by the disease. Caregivers should be directed towards support groups and community resources to help them manage their responsibilities. Their mental health should be considered and included in the patient’s treatment strategy. Legal and financial arrangements, such as appointing a legal guardian, can be made to alleviate potential distress related to these aspects of the disease.
What else can Pick Disease be?
Frontotemporal dementia often shows similar symptoms to other disorders affecting the brain, so it can be easily confused with them. It’s common for this type of dementia to be misidentified as conditions like depression, bipolar disorder, or schizophrenia, especially when the symptoms first start to show. However, there are some differences that can help doctors figure out if it’s frontotemporal dementia or not. For example, frontotemporal dementia usually starts affecting people over the age of 45, while schizophrenia tends to appear at a younger age. Additionally, frontotemporal dementia gets progressively worse over time, whereas the progression of schizophrenia can vary and sometimes even pause.
Differentiating frontotemporal dementia from other types of dementia can also be difficult. If the person’s memory and ability to understand spatial relationships are affected more than anything else, it could be a sign of Alzheimer’s dementia, the most common form of dementia. If there are signs like visual hallucinations and symptoms similar to Parkinson’s disease, it might be Lewy body dementia.
There are also several other conditions that can show symptoms similar to those of frontotemporal dementia, including:
- Infections
- Poisoning
- Certain types of tumors
- Bodily injuries
- Paraneoplastic disease, a rare disorder triggered by cancer
- Autoimmune disorders
- Thyroid conditions
- Hepatic encephalopathy, a loss of brain function due to liver disease
- Various genetic disorders
What to expect with Pick Disease
Frontotemporal dementia is a diverse condition, making it difficult to predict exactly how it will progress or determine the typical life expectancy. This type of dementia is less common than Alzheimer’s disease, which adds to the challenge. Survival rates can vary widely depending on the specific form of the disease.
For example, patients with a form that also involves motor neuron disease (a condition affecting the nerves that control muscles) usually have the shortest time from diagnosis to death, typically two to three years. On the other hand, people with what’s known as the ‘behavioral variant’ of frontotemporal dementia typically live for around nine years after diagnosis.
Patients with semantic dementia, a form that mainly affects understanding of language and meaning, have a survival rate of twelve years, similar to the generally slower progression of Alzheimer’s disease.
Possible Complications When Diagnosed with Pick Disease
Frontotemporal dementia often leads to a decline in both mental and physical functionality, which can cause physical, emotional, and social problems for those suffering from the disease and their caregivers. One of the key issues is dysphagia, or difficulty swallowing, which can result in increased cases of pneumonia and respiratory failure. These can be a significant cause of death in people with frontotemporal dementia.
Associated Complications:
- Dysphagia
- Pneumonia and respiratory failure
- Weight loss
- Protein-calorie malnutrition
- Failure to thrive
A lack of movement, common in patients with frontotemporal dementia, can result in heart disease. This is one of the leading causes of death for these individuals.
Additional Risks:
- Cardiac disease
- Falls and traumatic injury due to difficulties with walking
Preventing Pick Disease
Teaching patients and their caregivers is a key part of managing frontotemporal dementia. Understanding what’s going on with the disease – especially how it can change behavior – can help lessen feelings of stress and guilt that may arise if it seems the patient is behaving poorly. It’s important to prepare families and caregivers for the ways this diagnosis might affect them – physically, emotionally, socially, and financially. By doing this, we can create a steady care environment and make the healthcare team’s work more effective overall.
