Pick's Disease:

What is Pick's disease?

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.

According to the National Institute of Neurological Disorders and Stroke, the following conditions are currently grouped together as frontotemporal dementias:

• Pick's Disease
• Primary progressive aphasia
• Semantic dementia

Causes and risk factors 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. While the cause is still unknown, there is a strong genetic component: FTDs tend to run in families, and approximately 40% of Pick's Disease cases are believed to be hereditary.

Signs and symptoms of Pick's Disease

Because the frontal lobes affect behavior and emotional response, people with Pick's Disease will usually show signs of changes in personality before they manifest evidence of dementia. This may begin as impulsiveness or a lack of inhibition. 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 will appear in later stages of the illness.

Symptoms of Pick’s Disease
Behavioral changes	Impulsivity Obsessive/compulsiveness (for example, overeating or only eating one type of food) Drinking alcohol to excess (when this was not previously a problem) Rudeness or impatience, leading to aggression Poor judgment Inability to function or interact in social situations Inability to hold a job Lack of attention to personal hygiene Sexual exhibitionism or promiscuity Withdrawal or seclusion
Emotional changes	Abrupt mood changes Lack of warmth, concern, or empathy Indifference to events or to one's environment Easily distracted; difficulty maintaining a line of thought Unaware of the changes in behavior Decreased interest in activities of daily living
Language changes	Reduced quality of speech: shrinking vocabulary, difficulty finding a word Difficulty speaking or understanding speech (aphasia) Repeating words others say (echolalia) Weak, uncoordinated speech sounds Decreased ability to read or write Complete loss of speech (mute)
Neurological and physical problems	Increased muscle rigidity or stiffness Difficulty moving about Lack of coordination General weakness Memory loss Urinary incontinence

Diagnosis and treatment of Pick's Disease

How is Pick’s Disease diagnosed?

Pick's Disease can only be conclusively determined by a post-mortem examination of the brain. Careful symptomatic evaluation, together with brain scans and EEGs, are the best methods currently available for reaching a probable diagnosis. These techniques can help ascertain whether the presenting condition is likely to be Pick's Disease or a related disorder, such as Alzheimer's disease.

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.

Managing Pick’s Disease

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.

In addition, consider the following steps to help manage the symptoms of Pick's Disease:

• Sensory function aids, such as eyeglasses, hearing aids, etc.
• Behavior modification that rewards positive behaviors
• Speech therapy and/or occupational therapy
• 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

Research and clinical trials on Pick's Disease

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.

The NINDS is currently recruiting patients for a frontotemporal dementia research study that specifically address Pick's Disease patients, and there are eight additional clinical trials addressing frontotemporal dementia that are also seeking participants.

Related links for Pick’s Disease

NINDS Frontotemporal Dementia Information Page – Defines Frontotemporal Dementia (also known as 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)

Amara Rose and Douglas Russell, LCSW, contributed to this article. Last modified on: 12/19/07.