According to Arnold Pick, who first described the disease in 1892, Pick's disease causes an irreversible decline in a person's functioning over a period of years. Although it is commonly confused with the much more prevalent Alzheimer's disease, Pick's disease is a rare disorder that causes the frontal and temporal lobes of the brain, which control speech and personality, to slowly atrophy. It is therefore classified as a frontotemporal dementia, or FTD.
Causes of Pick's disease
Like Huntington's disease and Lewy Body disease, Pick's disease is the result of a build-up of protein in the affected areas of the brain. The accumulation of abnormal brain cells, known as Pick's bodies, eventually leads to changes in character, socially inappropriate behavior, and poor decision making, progressing to a severe impairment in intellect, memory and speech.
Pick's disease usually strikes adults between the ages of 40 and 60, and is slightly more common in women than in men. Unfortunately, the cause for Pick's disease is unknown. A few studies suggest that Pick's disease may have a genetic component, but most family members are unaffected. Other risk factors are unknown.
In contrast to Alzheimer’s disease, where memory loss is the predominate early sign, the first symptoms of Pick’s disease usually involve personality changes or a decline in basic functioning.
While the progression of symptoms in Pick's disease is fortunately slow, symptoms do worsen over time. The following symptoms are typical of patients with Pick's disease. More severe symptoms appear in later stages of the illness.
Behavioral signs and symptoms of Pick’s disease
- Impulsivity and poor judgment
- Extreme restlessness (early stages)
- Overeating or drinking to excess (when this was not previously a problem)
- Lack of attention to personal hygiene
- Sexual exhibitionism or promiscuity
- Withdrawal or decreased interest in activities of daily living
- Decline in function at work and home
- Repetitive or obsessive behavior
Emotional signs and symptoms of Pick’s disease
- Abrupt mood changes
- Lack of warmth, concern, or empathy
- Rudeness, impatience, or aggression
- Easily distracted; poor attention span
- Unaware of the changes in behavior
Language signs and symptoms of Pick’s disease
- Loss of vocabulary
- Trouble finding the right word
- Difficulty speaking or understanding speech
- Repeating words others say
- Weak, uncoordinated speech sounds
- Decrease in ability to read or write
- Complete loss of speech
Physical signs and symptoms of Pick’s disease
- Increased muscle rigidity or stiffness
- Difficulty moving about
- Lack of coordination
- General weakness
- Memory loss
- Urinary incontinence
Pick's disease can only be conclusively determined by a post-mortem examination of the brain, but there are ways to reach a probable diagnosis when symptoms set in. Once diagnosed, patients and caregivers can use practical strategies and professional support to treat the symptoms of the disease.
How is Pick’s disease diagnosed?
Currently, the best methods for reaching a probable diagnosis involve careful symptomatic evaluation, together with brain scans and EEGs. These techniques can help ascertain whether the condition is likely to be Pick's disease or a related disorder such as Alzheimer's disease. Although these dementias may be similar, there are clear symptoms that set them apart.
If at least three of the following five distinguishing characteristics are present in the early stages, the diagnosis is likely to be Pick's rather than Alzheimer's. Also, as compared with Alzheimer's disease, obvious mental impairment and memory loss occur later in Pick's disease patients than in Alzheimer's patients.
The five distinguishing features of Pick’s disease
Doctors look for at least three of the following to diagnose Pick’s disease.
- Onset before age 65
- Initial personality changes
- Loss of normal controls, e.g., gluttony, hypersexuality
- Lack of inhibition
- Roaming behavior
Treatment for Pick’s disease is usually similar to treatment for Alzheimer’s: symptom management that is aimed at maximizing quality of life. This may include medications to manage particular symptoms, regular supervision, and assistance. Treatment should also include emotional and substantive support for the caregiver. If you or a loved one has Pick’s disease, the following may help control symptoms.
- Sensory function aids. Eyeglasses or hearing aids can bolster failing senses.
- Behavior modification. Systems that reward positive behaviors can help reinforce appropriate behavior when symptoms set in.
- Professional therapy. Speech therapy and/or occupational therapy can improve communication and movement.
- Medication to control behaviors that can be dangerous to oneself or others. Antidepressants known as selective serotonin reuptake inhibitors (SSRIs) may offer some relief from apathy and depression and help reduce food cravings, loss of impulse control and compulsive activity
Focusing on the positive aspects of dealing with a terminal disease might seem like an exercise in futility, and yet, there can be unexpected bright spots for patients with Pick's disease. For instance, at the University of California/San Francisco Medical Center's Memory and Aging Center, doctors discovered a small group of frontotemporal dementia patients who developed new creative skills in music and art. The artistic talents emerged when the brain cell loss occurred predominantly in the left frontal lobe, which controls functions such as language.
As the ability to communicate through words declined, these patients' brains somehow accessed other realms of self-expression. So exploring and encouraging the development of latent skills is one way in which Pick's disease patients can maintain their quality of life and possibly slow the progress of mental deterioration.
Advice for family members
Caring for someone with dementia can be a life-changing experience. People with Pick’s disease may need daily or even around-the-clock care, and taking this on is a huge responsibility. You may feel alone, and the kind of daily challenges you face can be tough on your physical and mental health. If you are a caregiver, remember that it’s important to take measures to prevent burnout and garner your own support—in order to better care for your loved one.
Coping with the loss of personality
Often, the hardest thing about seeing someone you love develop Pick’s disease is witnessing the loss of or change in former personality. If the patient is living at home, you may remember the way he or she was before the disorder—a tragic and daily realization. In an assisted facility, the fact that those around the patient did not know him or her before the disease may be equally heartbreaking. Either way, this aspect of the Pick’s disease is extremely distressing for loved ones; seeking support and taking time for yourself can help you cope.
The National Institute of Neurological Disorders and Stroke (NINDS), and other branches of the National Institutes of Health, conduct regular research related to frontotemporal dementia. They also support additional research through grants to major medical institutions across the country. Their goal is to learn more about the causes, diagnosis, treatment, and possible prevention of Pick's disease and other FTDs.
Resources and References
NINDS Frontotemporal Dementia Information Page – Defines frontotemporal dementia (which includes Pick's disease), including patient prognosis, treatment protocols and research. (National Institute of Neurological Disorders and Stroke)
Pick's Disease: An Explanation (UK) – Detailed description of symptomatology and suggestions for managing the illness. (Pick's Disease Support Group)
Frontotemporal Dementia – Explains the distinction between FTDs (which includes Pick's disease) and other dementias, including background on genetics, evaluation and treatment, and a section for caregivers. (University of California, San Francisco)