Under the Umbrella -- Pick's Disease - Frontotemporal D...

Under the Umbrella -- Pick's Disease

Overview

Pick's disease is a form of dementia characterized by a slowly progressive deterioration of social skills and changes in personality, along with impairment of intellect, memory, and language. Onset typically occurs sometime in the 50's, though it can occur as early as age 20 or as late as age 80. As with all FTDs, the course of Pick's disease will vary from one person to another. Not every symptom will be experienced by every person, nor will these symptoms develop in a pre-ordained sequence. All patients with Pick's disease will experience a gradual, steady decline in functioning.


Key Clinical Features

All Pick's disease patients suffer from behavioral symptoms; they may or may not also experience language and neurological difficulties.

Behavioral Symptoms
A progressive deterioration in the patients' ability to control or adjust his/her behavior appropriately in different social contexts is the hallmark of all of these behavior changes, and results in the embarrassing, inappropriate social situations that can be one of the most disturbing facets of FTD.
* Hyperoral behaviors include overeating, dietary compulsions, in which the person restricts himself to eating only specific foods (such as a certain flavor of Lifesaver, or eating food only from one fast food restaurant), or attempts to consume inedible objects. Patients may consume excessive amounts of liquids, alcohol and cigarettes.
* Stereotyped and/or Repetitive behaviors can include re-reading the same book multiple times, hand rubbing and clapping, humming one tune repeatedly, or walking to the same location day after day.
* Personal hygiene habits deteriorate early in the disease progression, as the person fails to perform everyday tasks of bathing, grooming, and appropriate dressing.
* Hyperactive behavior is exhibited by some patients, and can include agitation, pacing, wandering, outbursts of frustration, and aggression.
* Hypersexual behavior can range from a preoccupation with sexual jokes to compulsive masturbation.
* Impulsive acts can include shoplifting, impulsive buying, and grabbing food off of another person's plate.

Emotional Symptoms
* Apathy or an indifference toward events and the surrounding environment can be marked by reduced initiative, and lack of motivation.
* Lack of insight into the person's own behavior develops early. The patient typically does not recognize the changes in his or her own behaviors, nor do they exhibit awareness or concern for the effect these behaviors have on the people around them, including loved ones.
* Emotional blunting develops early in the course of the disorder, and is manifested as a loss of emotional warmth, empathy, and sympathy, and development of what appears to be indifference toward other people, including loved ones.
* Mood changes can be abrupt and frequent.

Language Symptoms There are some FTD subtypes (semantic dementia and progressive non-fluent aphasia) which have specific language deficits as their cardinal feature. Listed below are the general symptoms of language deterioration that can accompany any FTD diagnosis.
* Aphasia (reduced output of speech)
* Dysarthria (increased difficulty producing speech due to weakness or incoordination – speech sounds weak, imprecise and uncoordinated) Agrammatica refers to an increasing difficulty in remembering or understanding the rules of grammar that help us get meaning from a sentence.
* Decrease in Reading and Writing Comprehension occurs as the patient experiences problems with word meaning and recognition.
* Echolalia refers to a repetition of a word or phrase said by another person.
* Perseveration is the repetition of a word or phrase said by the patient himself.
* Mutism eventually develops in many FTD patients during advanced stages of the disease.

Neurological Symptoms
* Movement Dysfunction symptoms may be some of the first to develop, and are similar to those seen in Parkinson disease (in these patients the term “parkinsonism” is used to distinguish the fact that they do not have Parkinson disease, though they do exhibit some of these symptoms). These include: decreased facial expression, bradykinesia (slowness of movements), rigidity (resistance to imposed movement), and postural instability. Less commonly, patients may develop symptoms seen in Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD), including difficulty with eye movements, dystonia (abnormal muscle postures), or alien hand syndrome (in which the person does not recognize his hand as being part of his own body).
* Muscle Dysfunction may be similar to the muscular problems seen in ALS: weakness, muscle atrophy, and myoclonus (muscle jerks).

Neuroimaging Studies reveal atrophy and decreased blood flow in the frontal and temporal lobes of the brain. Patients with the most pronounced behavioral symptoms often have more extreme change on the right side of the brain.


Key Pathologic Features

Upon autopsy, patients are typically found to have atrophy of the frontal and temporal lobes of the brain. Patients with more severe behavioral symptoms are often found to have this damage focused in the frontal lobe.

When viewed under the microscope, some nerve cells in this area have characteristic abnormalities (inclusions called Pick bodies and swollen cells called Pick cells). These abnormalities are named for the neurologist Arnold Pick, who first identified them in 1892.


Cause

Scientists have recently determined that these affected cells contain deposits of an abnormal form of the protein tau.


Genetics

The majority of Picks disease is not hereditary.


Treatment

There is no cure for Picks disease.


Management and Prognosis

Although specific symptoms may vary from patient to patient, the progression of Pick's disease is an inevitable progressive deterioration. The length of progression varies, ranging from less than 2 years in some to more than 10 years in others. Pick's disease itself is not life-threatening. It does, however, predispose patients to serious complications such as pneumonia, infection, or injury from a fall. The most common cause of death is pneumonia.


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Material prepared by Jennifer M. Farmer, MS, CGC, Website Clinical Consultant and Susan L-J Dickinson, MS, CGC, Website Medical and Science Writer.