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Pick's Disease: Frontal Lobe Dementia

Pick's disease is a progressive type of dementia involving atrophy of the frontal and temporal lobes of the brain.

The onset of Pick's disease is most common between the ages of 40 and 65 with an average onset of 54 years. Pick's disease mainly affects behavior and language and is characterized by gradual personality changes, speech impairment and eventual dementia.

The life expectancy following diagnosis ranges between two and seventeen years, sometimes longer, with an average of eight years.

Areas of the Brain Affected by Pick's Disease

Frontal lobe dementia is distinguished from other types of dementia by the presence of abnormalities called Pick bodies, found in the frontal and temporal lobes of the brain.

Pick's disease occurs in the cerebral cortex, the outer layer of the brain that controls behavioral and cognitive functions.

Cell degeneration is predominant in:

the thalamus, the area of the forebrain that processes information
the corpus striatum, the part of the brain involved with movement control
the subcortex, the nerve cells below the cerebral cortex or surface of the brain that control neurological functions.

Arnold Pick was a Czechoslovakian neurologist and psychiatrist, born in Moravia, Czechoslovakia on July 20, 1851. Pick died on April 4, 1924.

Pick first documented the disease in 1892 following the autopsy of a patient who had exhibited symptoms of dementia and progressive speech loss during his later years. Pick established that brain atrophy in the deceased had been caused by brain cell death in specific areas of the brain, the frontal and temporal lobes.

Other Common Names for Pick's Disease

The name "Pick" refers to only one aspect of the disease: the deterioration in the brain cell tissue caused by the presence of Pick bodies. Other names for Pick's disease include:

frontotemporal dementia (FTD)
frontotemporal lobar degeneration
semantic dementia
lobar sclerosis
focal cerebral atrophy
primary progressive aphasia
circumscribed brain atrophy
Arnold Pick's Disease.

Overview of Frontal Lobe Dementia Symptoms

Early symptoms are dictated by which areas of the brain are first affected by the diseased pick bodies.

For example, if the temporal lobes that control language degenerate first, then early symptoms are more characteristic of semantic dementia. When the frontal lobes that control behavior are involved, the first symptoms are usually behavioral changes.

In addition, symptoms often seem contradictory in the early- and mid-stages of frontal lobe dementia. Some patients, for instance, may behave in an erratic and hyperactive manner during the early stages, whereas others appear withdrawn and unresponsive. Symptoms, however, tend to become more uniform, in late-stage FTD.

Symptoms of Pick's disease include:

personality and socio-behavioral changes (for example, disregard for home, job or financial commitments)
inability to plan and organize
judgmental, inappropriate social behavior
apathy, negativity, poor motivation
inability to empathize and communicate emotionally
repetitive speech and behavior
glazed facial expressions
obsessive behavior
hypochondria
over eating and excessive alcohol consumption
sleep pattern changes and increased drowsiness
impaired motor response to verbal commands
rigidity
loss of muscle control (akinesia)
speech loss
dementia
death (usually caused by complications).

Tau Proteins

Recent studies have distinguished five or more different types of nerves cells that become damaged in patients with FTD. Two of the five nerve cells contain abnormal deposits of tau proteins.

The accumulation of tangles of abnormal tau proteins in the nerve cells of the frontotemporal lobes of the brain is a major cause of Pick's disease.

Researchers have found that an inherited mutation in the tau gene increases the risk of developing Pick's disease.

Pick Bodies

The presence of abnormalities in the nerve cells of the brain, called Pick bodies, distinguishes frontal lobe dementia from other types of dementia.

Pick bodies are fibrous tangles of tau proteins. The straight, fibrous appearance of Pick bodies differs markedly from the paired and coiled construction of the neurofibrillary tangles associated with Alzheimer's disease.

Diagnosis and Treatment for FTD

Diagnostic tests for Pick's disease include:

physical exam
clinical assessment
blood tests
neuro-psychology assessments
linguistic tests
CT and MRI scans.

Treatment for Pick's Disease

No treatment exists for the prevention of Pick's disease. Research and clinical trials are continuing to search for a cure for frontal lobe dementia.

Current treatments for the symptoms of FTD include tranquilizers, antidepressants, serotonin-based supplements and behavioral therapy.

How Does FTD Differ From Alzheimer's Disease?

Differences between FTD and Alzheimer's include the following:

Pick's disease is typically localized in the frontal and temporal cerebral lobes; Alzheimer's attacks various areas of the brain including the posterior, temporal and parietal regions and the hippocampus.
The age of onset for FTD is between 40 and 65 years; Alzheimer's is uncommon under the age of 65.
Severe behavioral problems are usually the first symptoms to manifest with FTD, whereas Alzheimer's sufferers tend to show early symptoms of memory impairment.
Although neurofibrillary tangles may also be present with FTD, the distinguishing factor for Pick's disease is the absence of amyloid plaques (aluminum silicate and amyloid peptides protein deposits).

Resources

ADAM Healthcare Center. (2005). Pick's disease.

Casey, B. (2001, February). FTD/Pick's stages and progression. Pick's Disease Support Group Newsletter, 7(1).

Cleveland, J. & Longsdon, R. (1999). UW researchers discover gene associated with frontotemporal dementia. Dimensions, Autumn 1999 (University of Washington Alzheimer's Disease Research Center).

Family Caregiver Alliance. (2005). Frontotemporal dementia.

Godbolt, A. (nd). Pick's disease (frontotemporal lobar degeneration).

Kennard, C. (nd). Diagnosis, treatment & prognosis of Pick's disease.

Kennard, C. (nd). Pick's disease: A form of dementia named after Arnold Pick.

Morris, H. (2000). Understanding the cause of fronto-temporal dementia.

Yancopoulou, D., Crowther, R.A., Chakrabarti, L., Gydesen, S., Brown, J.M. & Spillantini, M.G. (2003, August). Tau protein in frontotemporal dementia linked to chromosome 3 (FTD-3). Journal of Neuropathology & Experimental Neurology 62( 8), 878-882.

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Posted by Saras

November 20, 2008 at 07:02 AM

my husband is 46 and recently has become anxious, paranoid, lax at work and home. Everything has become difficult for him. No motivation. Also with noted short term memory problems. I am a nurse & I am very worried. What are the first steps to take?

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Posted by corinne

November 17, 2008 at 08:01 PM

quite helpful in understanding symptoms. Would appreciate what steps one can take to support someone with Picks disease.

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Posted by Fran R

August 20, 2008 at 03:12 PM

This has really given me some useful information into the various forms and symptoms of FTD, and a much more positive outlook on what is known about the disease and what research is being done.

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