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A story of Pick's disease: a rare form of dementia.

Abstract: Pick's disease is a progressive illness that affects brain function, eventually causing loss of verbal skills and problem-solving abilities. Pick's disease accounts for 5% of all dementias. The most common symptoms involve personality, behavior, and language changes. Diagnosis can be difficult; a differential diagnosis often requires several years. Frequently, the disorder is initially diagnosed as stress, depression, or Alzheimer's disease. A magnetic resonance imaging scan will show atrophied portions of the frontal and temporal lobes. One of the most difficult aspects of care for someone with Pick's disease is communication. The communication styles of nurses can alleviate the anxiety experienced by a person with Pick's disease. Nurses should try different strategies as an approach to communicating and caring for someone with Pick's disease, remembering that each person suffering from dementia is different. Pick's disease can be emotionally devastating to the families of those who develop this disease. Working with families confronting the disability of a loved one is perhaps the greatest challenge for nurses. Nurses have the responsibility of educating the primary caregiver about nutrition, skin protection, incontinence care, safety, and end-of-life decisions.

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Dementia is defined by short- and long-term memory loss and the presence of other cognitive deficits that progressively worsen and impair activities of daily living. According to the United Nations Department of International Economics and Social Affairs, more than 7 million people in North America and Europe currently have been diagnosed with dementia (Litvan, 2001).

Pick's disease is an often-overlooked form of dementia. It is a progressive dementia that usually begins midlife, at 50-60 years of age. It is characterized by slowly progressive changes in character and social deterioration, leading to impairment of intellect, memory, and language (Roquer, n.d.). The disease is a rare disorder similar to senile dementia of the Alzheimer's type. Alzheimer's disease accounts for 50%-60% of dementia cases, and Pick's disease accounts for about 5% of cases (Alzheimer's Association, 1995). Pick's disease eventually causes loss of verbal skills and problem-solving abilities.

Diagnosing Pick's disease can be difficult, and require several years. Frequently; the disorder is initially diagnosed as stress or depression and sometimes as Alzheimer's disease (Hodges, 2001). Pick's disease can be emotionally devastating to the families of those persons who develop the disease. This article first describes the course of a patient diagnosed with Pick's disease and the effect of the disease on his family, and then outlines the pathophysiology, symptoms, diagnosis, and management of Pick's disease.

Case Presentation

No one knows quite when R.K. began changing. Five years ago? Seven? The scenes surface in the minds of friends and families like goldfish in a murky pond. R.K. would forget familiar routes to friends' homes. Names escaped him although he had always remembered them in the past. His remarks began to sound out of place ... not quite sensible.

In May 1993, R.K. was working as a registered mechanical engineer. He was 58 years old and lived with his wife of 33 years. They had two daughters who lived out of town. Then he was laid off by the company for which he had worked for more than 17 years. He became withdrawn and during the next year, more and more depressed. R.K. always had made all the family's financial decisions and handled the money affairs, but he began using poor judgment and made several bad financial decisions. R.K.'s wife noticed that he had begun to repeat his own statements as well as statements made by others. She forgave his mistakes out of love and even covered up for him.

His wife insisted that he consult their internist. He finally agreed to do so, but complained only of an ingrown toenail and hair loss. In December 1994, again at his wife's insistence, R.K. returned to the internist. A magnetic resonance imaging (MRI) scan done at that time showed generalized atrophy of the brain. The internist sent him to a psychologist for memory testing. The results were inconsistent, but the psychologist suggested that his wife take over their finances. R.K. agreed but insisted on continuing to drive.

During the next 6 months, R.K. was tested four or five times, by a psychologist, a neurologist, and a psychiatrist, but all tests were inconclusive. R.K. became more stubborn and independent. He began staying away from home in his truck for hours at a time. On one occasion, after R.K. was gone for more than 6 hours, his wife demanded that he give her the keys.

By July 1995, R.K. and his wife were forced to sell their home of 19 years due to his unemployment and her inability to work. They moved to an apartment closer to their families. Another memory test in May 1996 still was inconclusive. R.K. was placed on a multitude of medications from the antidepressant, antipsychotic, and anxiolytic classes. However, these medications caused side effects such as lethargy, increased verbal repetition, and unresponsiveness, so they eventually were discontinued.

R.K.'s repetitions became worse. His wife was becoming very tired and needed help caring for him, as she was constantly watching him as one might watch a child. She hired someone to take R.K. for rides in his truck, hoping to obtain respite. R.K., normally a very quiet and introverted person, now had few inhibitions. He wandered outside and needed constant attention. One night his wife found him in his closet buttoning every button on every shirt he owned. R.K. became less verbally responsive. All he said was "No, No, No."

The turning point came when R.K.'s wife found him huddled in a corner of the shower, unaware of how to turn the water off. When the children came for Thanksgiving, they discussed placing him in a long-term-care facility.

R.K. was taken to the geropsychiatric unit of a nearby hospital, where the diagnosis of Pick's disease finally was established. He was placed on fluphenazine hydrochloride (Prolixin). Because of the drug's toxicities, he was taken to intensive care unable to speak or swallow, immobile, and with arrhythmias. R.K. was transferred to a rehabilitation unit where he again became ambulatory and able to tolerate foods. He was started on diazepam (Valium), which seemed to be the drug of choice for him. From the hospital, R.K. was placed in a long-term-care facility. Thereafter came a succession of transfers to other facilities because of behavioral problems such as grabbing other patients, aggressiveness, setting off the fire alarms, taking telephones apart, and difficulties with medications. Finally, at his wife's insistence, he was transferred to a secure unit for confused and demented patients that was closer to his home.

Today R.K. is ambulatory, but is mute and without any facial expression. He recognizes his wife but not always his children or grandchildren. He is incontinent, but with encouragement he can feed himself. His daily activities include walking the halls of the secure unit, checking door locks, straightening papers on the desk, and picking up objects and inspecting them. The prognosis for R.K. is grim. His illness will eventually leave him in a vegetative state, unable to feed himself, walk, or acknowledge anyone.

Pathophysiology of Pick's Disease

In 1892, Dr. Arnold Pick first identified Pick's disease through an autopsy of a 71-year-old man who had had progressive mental deterioration. The autopsy demonstrated unusual shrinkage of the frontal cortex, the area involved in reasoning and other higher mental functions. This type of cortical shrinkage is different from changes found in the brain of an Alzheimer's patient. In Pick's disease the frontal and temporal lobes are the most atrophied (Brown, 1992; National Institute of Neurological Disorders and Stroke [NINDS], 2001). Brain cells in these areas often take on a "ballooned" appearance; they generally are filled with an abnormal form of protein tau. These cells, the hallmark of Pick's disease, are called Pick bodies. They can be detected only upon neuropathological examination (Noble, 1996). In Alzheimer's disease, pathology shows an abnormal protein beta-amyloid, which is disposed in the center of the senile plaques.

Research has been inconclusive about familial causes of Pick's disease. According to findings by Brown (1992), Pick's disease does appear to be inherited in some families, but the majority of cases are sporadic. Rosser (2001), on the other hand, found that genetic factors are strongly implicated; in half of the cases, there is a family history of similar diseases. More research is needed to establish these facts and to develop specific interventional studies that focus on behavior modification and communication with the person experiencing Pick's disease.

Symptoms

The frontal lobes of the brain function to plan and monitor performance as well as control behavioral and emotional responses. Damage to the frontal lobes can cause symptoms that differ greatly from one individual to another. Pick's disease varies greatly in the way it affects people, but there is a core of symptoms focused around personality, behavior, and language changes. Table 1 displays the symptoms most commonly associated with Pick's disease.

Numerous changes in personality may be seen in someone with Pick's disease. Individuals may lose their inhibitions. They may become either extroverted or withdrawn. They may talk to strangers, particularly children, make inappropriate remarks in public, and be rude or impatient. They may appear selfish and unable to see someone else's point of view; this change is caused by a loss of mental flexibility. Personality change can take the form of apathy and indifference toward customary interests or total disregard for the feelings of others (Alzheimer's Disease Society, 1997). The person may become aggressive, exhibit childlike behavior, or develop repeated routines and behaviors. Changes in sexual behavior may be linked to apathy or loss of inhibition.

Function declines because the person simply does very little, is confused, and uses poor judgment. Many times, someone with Pick's can perform a task when told what to do but cannot undertake the same task independently. The ability to initiate, organize, or follow through on a simple activity is lost (Therapeutic Advances, 1999).

Speech problems can range from reduction in the quantity or quality of speech to total loss of speech. Understanding speech and being able to write also are affected. One common symptom is the repetition of sentences, which is known as echolalia. Later, patients become very quiet or do not speak at all. They may sit for hours doing nothing unless prompted. At the other extreme, they can become restless, pacing constantly and easily distracted by anything they see or hear. Their attention span is short. Often they have difficulty sustaining a line of thought or maintaining a conversation for any length of time.

Individuals often exhibit changes in eating habits (e.g., overeating) or dietary preferences, particularly for sweet foods. An increase in sugar consumption leads to weight gain. Excessive alcohol intake may also occur. In the later stages, people with Pick's disease may compulsively put objects in their mouth. Swallowing may be difficult, and the person may choke on food or develop aspiration pneumonia.

In the early stages of Pick's disease, the person usually is aware of time and able to recognize people and faces. This is unlike the usual presentation of Alzheimer's disease. However, failure to recognize faces and incorrect use of objects do sometimes occur. Mathematical skills usually are relatively well preserved. In later stages of the disease, people usually become mute and apathetic, barely responding to the outside world. Eventually, they enter a permanent vegetative state. Death typically occurs either from malnourishment and debilitation, resulting in pneumonia, or from the aging process.

Diagnosis

The average course of Pick's disease is 5 years, but it can range from 2 to 15 years (NINDS, 2001). The diagnosis is difficult during life, because the symptoms vary and often so greatly overlap with those of Alzheimer's or Parkinson's disease. The cause of this form of neuro-degenerative disease is uncertain. Genetic studies are making some progress in revealing a matrix of genes that may contribute to development of Pick's disease. One theory is that a mutation on a gene on Chromosome 17 is the cause in some cases (Rosser, 2001).

Initially, an in-depth clinical evaluation should be conducted, including a complete personal and family health history. The purpose of a detailed history is to uncover the person's symptoms, changes in functioning and abilities, and other medical conditions. A physical examination helps to determine the overall health status of the patient. Laboratory tests, including blood tests, can detect liver, kidney, thyroid, or other problems (e.g., vitamin deficiencies) that can cause dementia. A thorough neurological examination should include an electroencephalogram; a computed tomography (CT) scan, and an MRI scan. The CT and MRI scans can reveal the frontal and temporal lobe atrophy characteristic of Pick's disease. Other evaluations can add information about possible causes for the dementia and explore the person's abilities and strengths. A psychiatric evaluation can determine the presence of an illness such as depression, the symptoms of which can mimic dementia, either as the cause or a contributing factor of symptoms (Robinson, 2001). The person's use of prescription and nonprescription drugs, especially those affecting the central nervous system, should be assessed. Psychometric tests can be useful in determining the person's areas of impairment as well as remaining areas of strength and independence. Tests to determine the person's remaining abilities to perform routine tasks (i.e., activities of daily living) can provide healthcare team members with information for future planning. Table 2 displays common characteristics of various forms of dementia with specific criteria for assessing differences.

Treatment

Unfortunately, there is no cure for Pick's disease, and there are no drugs that slow disease progression. Drugs designed for treatment of Alzheimer's disease are contraindicated in frontotemporal dementia, because they may increase aggression. Management of the patient lies in coping strategies, such as sidestepping issues rather than confronting them, and working around obsessions rather than trying to change them (Kelman, Thomas, Kennedy, & Cheng, 1994). Speech therapists and occupational therapists may be able to help by stimulating speech or swallowing or providing repetitive activities based on level of ability. Boredom is a serious problem. Caregivers have found it helpful to encourage new hobbies such as art, music, rug making, walking, and jigsaw puzzles (Mintzer, Hoemig, & Mirski, 1998). These activities focus on the individual, and they can reduce the incidence of behavior problems.

Patients with Pick's disease must be kept in a safe and supervised environment, particularly as the disease advances. Medications such as antidepressants can help in managing some behaviors, such as aggression and agitation (Petersen, Smith, Waring, Ivnik, Tangalos, & Kokmen, 1999). Providing emotional support for caregivers and other family members also is important. Support groups, including the Alzheimer's Association, are an excellent resource for patients and their families.

The Nurse's Role in Communicating

One of the most difficult aspects of care for someone with Pick's disease is communication. Nurses communicate on many levels, both verbally and nonverbally. When a patient's behavior doesn't fit expectations, nurses often become frustrated. They may behave in ways intended to reduce their own anxiety, almost without thinking.

There are four common reactions to the anxiety nurses feel when caring for someone who does not seem "to make sense." Nurses need to consider substituting these, with more constructive reactions (Morris, 1999).

One reaction is to conduct a monologue. The person with Pick's disease and the nurse carry on 'parallel' conversations, but neither is listening or responding to what the other is saying. Communication is therefore one-sided, and there is no exchange of information. The person is effectively living in his or her own private world.

When caring for a person with Pick's disease, the nurse should avoid resorting to a monologue and instead use a form of two-way communication. Individuals experiencing dementia often are socially isolated and deprived of normal sensory input; because of brain damage they are not hearing, seeing, feeling and perceiving the world as we are (Robinson, 2001). The content, structure, and accuracy of what the person says are not as important as the nurse's response. The key is to establish two-way communication between the patient and nurse. The nurse can do that by choosing part of a patient's phrase and using it as the basis for a response.

A second reaction is that nurses may speak and make decisions for the person (e.g., "Does he take sugar?" "Yes, he does."). Often nurses use their own frame of reference in deciding what is best for the patient. The decisions nurses make for patients often emerge from their own preferences. For example, the nurse's preference for sleeping with two pillows does not mean that is what will make the patient comfortable.

Third, nurses may attempt "reality orientation," in which the patient is corrected and attempts are made by the nurse to reorient the patient to the "real world." In reality orientation, the person with dementia is asked to remain in the nurse's "reality" (i.e., see things and interpret events as the nurse does), and yet the person often is unable to do so. The inability of the patient to remain in the nurse's reality makes the patient feel inadequate and can give rise to responses ranging from anger to withdrawal. It is important to consider how much patients with Pick's disease feel they are on "shifting sands." At any moment they may lose track of what is being said or thought (Morris, 1999).

Finally, nurses may find collusion easier than reality orientation. In collusion, the nurse agrees with the patient's disoriented communications. Collusion can be very tempting, as it is an easy mechanism for engaging the patient in conversation. However, people with Pick's disease often have unpredictable lucid moments--good days as well as bad days. Collusion may weaken their faith both in the nurse and in "reality," causing further confusion. It may represent an immediate solution, but does little to help the person with dementia. Being honest but perhaps economical with the truth, rather than judging the truth or accuracy of what the patient says, can be a constructive communication style (Petersen et al., 1999).

To achieve two-way communication, the nurse must be creative, attempting to put herself or himself in the shoes of the person with Pick's disease. The nurse must use previous experience and what is known or anticipated about the patient's experience (e.g., what the patient likes, dislikes, believes in; the patient's life history). The nurse should become familiar with the patient's life stories to better interpret what he or she is trying to say (Mintzer et al., 1998).

It is more important to keep in mind possible impairments that might explain why a patient is behaving in a particular way and to appreciate the abilities that are preserved. Examples include the various forms of visual difficulties that can occur in Pick's disease--difficulty with perceiving how near or far away something is; inability to see everything in view because of partial vision with sections missing, difficulty in distinguishing something against its background, and difficulty tracking a moving object. Each of these difficulties can give rise to a wide range of behaviors, e.g., a supposedly blind patient recognizing someone across the room, believing that a caregiver is attacking or abusing him or her because of inability to see the caregiver approaching; withdrawing because of an inability to make sense of what is seen, even helping himself or herself to food from the wrong plate. Perhaps the most important visual problems are "illusions" and "visual agnosias." A child, in the half-light, may think that clothes on a bedroom chair are a monster or an intruder. Similarly, the person with Pick's disease may misinterpret what she or he sees. These misperceptions or illusions can lead to a simple mistake or result in strong emotions such as fear, anxiety, or anger. Quite separate are visual agnosias in which the object is seen clearly but not understood; it may be either perceived as being something else or not recognized.

The emphasis of effective communication is on trying out different strategies as an experimental approach. This is a creative process unique to the people involved. The tools needed may change over time. Every person suffering from dementia is different, so there are no hard and fast rules as to what to say or do. There are only guidelines and the courage and support to enable creativity in the interaction. Nurses should try things out and discuss their strategies with other nurses.

Summary

Nurses should be aware of Pick's disease and the long-term interventions required to care for people with dementia. Nurses have the skills to coordinate community services, evaluate safety issues in the home, plan respite, provide support-group information for the family, and coordinate care across settings. Providing information on adult day care facilities in the geographic area can be a key to balancing the stress level of primary caregivers. Providing assistance and support to families is an integral aspect of nursing care throughout the disease process.

Working with families who are confronting the disability of a loved one is perhaps the greatest challenge for nurses. Eventually, the patient will lose the ability to perform activities of daily living. Nurses are responsible for educating the primary caregiver. The nurse has a unique opportunity to help families during their initial adjustment to the diagnosis and in their decision making as the disease progresses.

Continuing Education Credit

The Journal for Neuroscience Nursing is pleased to offer the opportunity to earn neuroscience nursing CE for this article online. Go to www.aann.org, and select "Continuing Education." There you can read the article again or go directly to the posttest assessment. The cost is $15 for each article. You will be asked for a credit card or online payment service number.

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Table 1. Signs and Symptoms of Pick's Disease

Symptom Area Manifestations

Coordination Loss of skilled movement abilities

Personality Loss of concern
 Loss of initiative
 Flat affect
 Mood swings
 Impaired judgment
 Lack of spontaneity
 Indecision

Behavior Excessive exploration of environment
 Withdrawal
 Inability to function socially
 Inability to maintain employment
 Decreased ability to perform self-care activities
 Inappropriate behavior for situation
 Inability to solve problems
 Memory loss

Language Echolalia
 Aphasia
 Poor enunciation
 Decreased ability to speak and write
 Inability to name objects
Table 2. Characteristics of Common Dementias

Type of Dementia Onset Progression

Alzheimer's Insidious Slow
 Disease

Vascular Disease Abrupt Stepwise

Depression Insidious Progression
 with remission

Lewy-Body Insidious Progressive and
 Dementia rapid

Pick's Disease Early age Progressive
 (frontotemporal
 dementia)

Steele- Insidious Progressive
 Richardson-
 Olszewki
 Syndrome
 (progressive
 supranuclear
 palsy)

 Laboratory
Type of Dementia Clinical Features Features Neuro Imaging

Alzheimer's Deficit in cognition Increase tau Cortical and
 Disease No problem with and decreased hippocampal
 consciousness beta-amyloid atrophy
 Illusions, in cerebral
 delusions, and spinal fluid
 hallucinations
 Increased muscle
 tone
 Seizures and gait
 disturbance
 (late stage)

Vascular Disease Emotional Hyperlipidemia Multiple
 disturbance vascular
 Evidence of focal lesions
 neurological
 disease
 Evidence of vascular
 disease
 Patchy cognitive
 defects

Depression Subjective None None
 complaints
 Sadness
 Lack of motivation

Lewy-Body Difficulty with None Transient
 Dementia social functions slow-wave
 (early stage) activity in
 Memory impairment the temporal
 (late stage) lobe on EEG
 Attention defects
 Problems with
 alertness
 Visual hallucination
 Parkinsonism
 History of falls
 Delusions

Pick's Disease Apathy None Frontal and
 (frontotemporal Uninhibited behavior temporal
 dementia) Antisocial behavior lobe atrophy
 Poor insight
 Poor decision making
 Language deficits
 Memory deficits

Steele- Altered balance, None Frontal
 Richardson- vision, atrophy
 Olszewki swallowing, speech
 Syndrome Forgetfulness, lack
 (progressive of interest, or
 supranuclear loss of
 palsy) enthusiasm,
 irritability,
 impaired
 concentration


Acknowledgments

The authors thank William McAuley, PhD, professor and Linda Moore, PhD RN, associate professor, at the University of North Carolina at Charlotte, for their careful review of an earlier draft of this manuscript.

References

Alzheimer's Association. (1995, Fall). Alzheimer's disease. Advances.

Alzheimer's Disease Society. (1997). What is Pick's disease and frontal lobe dementia? London: Author.

Brown, J. (1992). Pick's Disease. Bailliere's Clinical Neurology, 1(3), 534-557.

Hodges, J.R. (2001). Frontotemporal dementia (Pick's disease): Clinical features and assessment. Neurology, 56(11, Suppl. 4), S6-S10.

Kelman, H.R., Thomas, C., Kennedy, G.J., & Cheng, J. (1994). Cognitive impairment and mortality in older community residents. American Journal of Public Health, 84, 1255--60.

Litvan, I. (2001). Therapy and management of frontal lobe dementia patients. Neurology, 56(11, Suppl. 4), S41-S45.

Mintzer, J. E., Hoernig, K.S., & Mirski, D.F. (1998). Treatment of agitation in patients with dementia. Clinical Geriatric Medicine, 14(10), 147.

Morris, Clare. (1999). Communication Problems in Dementia. Factsheet retrieved April 16, 2002, from Pick's Disease Support Group Web site: www.pdsg.org.uk.

Noble, J. (1996). Textbook of primary care medicine. St Louis: Mosby.

National Institute of Neurological Disorders and Stroke. (2001, May). Pick's disease information page. Retrieved from www.ninds.nih.gov/health and medical/disorder index/pick's disease.htm.

Petersen, R.C., Smith, G.E., Waring, S.C., Ivnik, R. J., Tangalos, E. G., & Kokmen, E. (1999). Mild cognitive impairment: Clinical characterization and outcome. Archives of Neurology, 56, 303-308.

Robinson, K.M. (2001). Rehabilitation applications in caring for patients with Pick's disease and frontotemporal dementias. Neurology 56(11, Suppl 4), S56-8.

Roquer, P. (n.d.). Pick's disease--An explanation. Factsheet retrieved April 5, 2002, from Pick's Disease Support Group Web site: www.pdsg.org.uk.

Rossor, M.N. (2001) Pick's disease: A clinical overview. Neurology, 56(11, Suppl. 4), S3-S5.

Therapeutic advances for Alzheimer's disease and other dementias. Neurology Treatment Updates. Retrieved 1999 from http://pri marycare.medscape.

Questions or comments about this article may be directed to: Sonya Hardin, PhD RN CCRN, University of North Carolina at Charlotte, Adult Health Nursing Department, 9201 University City Boulevard, Charlotte, NC 28078. She is an assistant professor in the department of adult health nursing and coordinator of the MSN/MHA program at the University of North Carolina at Charlotte, as well as a part-time staff nurse in the critical care unit at Davis Regional Medical Center in Statesville, NC.

Brenda Schoooley, MSN RN, is a nurse manager for the Center for Medicine at Presbyterian Hospital located in Charlotte.
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Author:Hardin, Sonya; Schooley, Brenda
Publication:Journal of Neuroscience Nursing
Geographic Code:1USA
Date:Jun 1, 2002
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