Pick’s Disease: Another form of Alzheimer?

Almost everyone has heard of Alzheimer’s disease. Even when an individual has no direct, personal connection to this neurodegenerative condition, they are aware of its effects portrayed in today’s media. Movies, books, and television shows fail to include a just as life-altering form of dementia known as Pick’s disease—a disease that literally shrinks the brain to result in changes in behavior and problems with language.

Pick's Disease: Another form of Alzheimer?
Magnetic Resonance Imaging for Pick’s Disease

What is Pick’s Disease?

Pick’s Disease is a neurodegenerative disease founded by Czech psychiatrist Arnold Pick in 1892. It is classified as frontotemporal dementia and also goes by the name of lobar degeneration. The disease process is set into motion by nerve cell proteins known as tau. Each of the brain’s neurons has microtubules to transport nutrients and various substances in-and-out of the cell. Tau protein bind to the microtubules to facilitate the cell function. In it, tau protein is in excess. These proteins aggregate in clumps called Pick bodies in the frontal and temporal lobes. The presence of Pick bodies deprives the brain of vital nutrients and damages the brain. Cells in the frontotemporal regions die off and neurons swell to induce changes of behavior, personality, and problems with language.

Who Can Develop Pick’s Disease?

In the United States alone, 50,000 to 60,000 citizens suffer from Pick’s disease. The most common age of diagnosis is between 40 and 75. However, some cases of Pick’s disease develop as early as age 20. Aside from age, there are risks that increase a person’s chances of the disease.

  • Male gender—Males are diagnosed with it more often than females.
  • Ethnicity—Certain ethnicities are prone to the disorder, particularly those of Scandinavian descent.
  • Genetics—There is a strong family connection to the formation of pick bodies in the brain. Having a parent or close relative with it is another risk factor. Researches have determined that 25% of cases of Pick’s disease are due to a genetic mutation inherited from a parent.

Symptoms of Pick’s Disease

Pick’s disease extensively compromises the frontal lobes. Each lobe has an important role in the skills of cognition and executive functions like attention, judgment, emotional control, multi-tasking, and planning. These go on to drastically influence everyday behaviors. The symptoms manifestations of Pick’s disease fall into two categories: behavior and language.

Behavior and Personality

Early signs of it are changes in behavior. Family and friends will notice that their loved one with it won’t act like their self. Subtle changes begin with depression-like symptoms, abrupt mood swings, and poor hygiene. They vary in severity and progress with age to include:

  • Compulsive behavior—Repetitive actions persistently performed to reduce anxiety. Examples are skin picking, hoarding, shopping, sex, gambling, exercise, etc.
  • Withdrawal from social interactions—Those with it are apathetic (indifferent) towards social interactions and activities they once derived pleasure from.
  • Paranoia and delusions—Unrealistic fears of feeling like people are “out to get you” accompany the belief in false ideas.
  • Loss of inhibition—Loss of inhibition is the inability to refrain from inappropriate actions. This leads to tension and rudeness in social settings.  
  • Restlessness—Easily bored and agitated
  • Child-like behavior—Temper tantrums, hyperactivity, and disregard for appearance are a few of the child-like behaviors associated with it.
  • Abnormal eating behavior—Patients engage in binge-eating, overeating, and craving sweets. Studies elaborate on the “oral exploration of inedible objects” (Manoochehri & Huey, 2012).
  • Memory lossThe memory loss occurs in later stages.
Pick's Disease
Pick’s Disease

Language

As previously mentioned, Pick’s disease affects every type of language or communication—meaning patients struggle to communicate via spoken words, writing, and sign language.

Patients lose the skills needed for reading and writing, which makes tasks involving those skills a challenge. When others are speaking, a patient cannot understand what is being said. They also struggle to respond or convey their ideas in speech. Aphasia, the failure to express and understand speech, manifests as an inability to speak, a diminishing vocabulary, and repeating words. Memory loss contributes to deficits in language, as patients cannot recall words for conversation.

Physical Symptoms

The physical symptoms of Pick’s disease are modest, as the frontal lobes do not regulate many physical functions. Patients can experience full-body weakness.

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Diagnosing Pick’s Disease

The only concrete diagnostic procedure to confirm is a biopsy of the brain. A neurosurgeon who operates on the brain surgically removes a small portion of tissue for examination under a microscope. The brain tissue in Pick’s disease should reveal swollen neurons and identifiable Pick bodies. Because brain surgery to obtain a biopsy is undoubtedly risky, medical professionals prefer a less invasive means of diagnosis. No single test or evaluation is a sole indicator of it. The diagnosis relies on a combination of medical tests and thorough evaluations by a doctor.

  • Genetic testing—The doctor may order exome sequencing to examine the patient’s DNA. This locates genetic mutations for it.
  • Neuropsychological testing—An assessment of cognitive functions by a neurologist, or psychologist. The tasks of a neuropsychological assessment check reasoning, abstract thinking, memory, attention, orientation, use of language, and evaluate the patient’s mood, presence of hallucinations or delusions, and overall mental status.
  • Blood work—Lab tests for liver disease, blood disorders, vitamin deficiencies, kidney problems, thyroid conditions, or hormonal disorders rule out potential causes for the dementia symptoms of it.
  • CT and MRI—Imaging studies such as computerized axial tomography (CT) scan or a magnetic resonance imaging (MRI) scan can reveal abnormalities in the frontal and temporal lobes, which are the parts of the brain impacted by it. They mainly look for atrophy of the brain.
  • Single-photon emission computed tomography (SPECT) scan—SPECT scans will show how active specific areas of the brain are.
  • Lumbar puncture—Abnormalities in the spinal fluid can cause symptoms of dementia induced by diagnoses other than Pick’s disease. A lumbar puncture takes a sample of cerebrospinal fluid for testing.

After the patient’s death, completing the brain biopsy during autopsy is helpful to give families closure through the confirmation of the diagnosis.

The Difference Between Pick’s Disease and Alzheimer’s

Pick’s disease and Alzheimer’s are two of several types of frontotemporal dementia, so they are similar in many ways. While they are both characterized by the presence of Pick bodies, Pick’s disease has distinct differences. Firstly, it causes a buildup of one form of tau proteins, whereas Alzheimer’s has multiple forms. Additionally, it does not affect the same parts of the brain. Alzheimer’s interferes with the frontal, parietal, temporal, and occipital lobes, yet the damage is confined to the frontal and temporal lobes.

Next, the disease follows different patterns of cognitive decline. For example, a study published in Arch Neurol concludes that personality change and language impairment is more prevalent in Pick’s disease, but memory loss was more common in Alzheimer’s (Binetti et al., 2000). Patients with Alzheimer’s exhibit memory loss in the early stages of the disease and behavioral problems in its later stages. However, memory loss is a symptom that arises in later stages, while behavioral changes occur early on in the disease.

Treatment for Pick’s Disease

Pick’s disease is a progressive, irreversible condition. There is no cure and no intervention capable of slowing progression. Treatment focuses on symptoms management and quality of life measures. As the disease progresses, individuals with Pick’s disease require continual assistance completing daily activities.

A beginning approach to treatment should include treating medical conditions that advance dementia symptoms like heart failure, infections, kidney failure, nutritional deficiencies, thyroid disorders, decreased oxygen, and anemia.

Behavior Modification

Since behavior and personality changes are defining symptoms of Pick’s disease, behavior modification therapy is a helpful treatment. Behavior modification is a therapeutic approach based on operant conditioning. Operant conditioning incorporates reinforcement and punishment to control behavior. Encouraging behaviors through positive reinforcement is a successful intervention for Pick’s disease. The therapist implements an award system to reduce unwanted behavior and to promote desired behavior. Traditional psychotherapy, however, is not conducive for Pick’s disease because it causes further confusion.

Speech Therapy

Whether mutism (the inability to speak), difficulty understanding speech, and other symptoms of aphasia, speech problems are a significant component of Pick’s disease. Speech therapy seeks to overcome these barriers to communication. A speech therapist applies exercises to improve language skills. Depending on the patient, speech therapy can include group therapy to strengthen communication in interactions.

Medication

  • Selective serotonin reuptake inhibitors—SSRIs are a class of antidepressants that increase the levels of the neurotransmitter serotonin in the brain. In Pick’s disease, SSRIs target behavioral symptoms like depression, anxiety, and compulsive behavior.
  • Antipsychotics—Although used with caution due to dangerous side effects, physicians prescribe antipsychotics for acute episodes of intense agitation and aggressive behavior.

Cholinesterase inhibitors are frequently used for dementia. They block the breakdown of acetylcholine, another neurotransmitter in the brain. Current research does not lend evidence of cholinesterase inhibitors improving the symptoms of Pick’s disease.

Pick’s Disease Progression

Pick’s disease progresses in a series of stages. The prognosis of the condition is poor. Average life expectancy is approximately 7 years after symptom onset. This average can be as little as 2 years or as great as 10 in some patients.

Mild Pick’s Disease

In the beginning stage of Pick’s disease, symptoms are subtle. Mild behavioral changes appear in this stage. The patient might have frequent mood swings, strange eating habits, and their relationships suffer from social withdrawal. When they interact with others, they are apathetic and rarely express sympathy and empathy. They engage in impulsive behavior. Their lack of inhibition might even drive them to break laws. Planning and organization become erratic.

Moderate Pick’s Disease

Symptoms progress from the mild stage to moderate within the span of a couple of years. The patient grows more unorganized and executive functions like memory and attention decline. Compulsive and repetitive behaviors are present. If the patient suffers from binge eating, weight problems and health issues occur. Pick’s disease drastically impedes language skills in the moderate stage.

Severe Pick’s Disease

After 5 to 10 years, severe Pick’s disease leaves the patient disabled and dependent on a caregiver for 24-hour assistance. The patient might be mute, unable to express themselves to communicate. Profound lack of empathy, apathy, and impulsive behaviors accompany memory loss. The disease transforms the individual’s personality, which is emotionally painful for loved ones. Preserving the patient’s dignity is vital in severe or late stage Pick’s disease. As science evolves, there is the hope of increasing the lifespan that has been shortened by this unfortunate disease.

References

Binetti G, Locascio JJ, Corkin S, et al. Differences between Pick disease and Alzheimer disease in clinical appearance and rate of cognitive decline. Arch Neurol 2000;57:225–32.

Manoochehri, M., & Huey, E. D. (2012). Diagnosis and management of behavioral issues in frontotemporal dementia. Current neurology and neuroscience reports, 12(5), 528–536. doi:10.1007/s11910-012-0302-7