A Form of Frontotemporal Dementia
“Pick Disease,” or “Pick’s Disease” is the name given to one form of a larger group of diseases now called the frontotemporal dementias. (FTD). It is the fourth most common cause of dementia, and thought to account for about 5 percent of dementias (currently called major neurocognitive disorders or MNDs). Among younger onset cases, those that begin before age 60, FTDs are the first or second most common cause of dementia. The FTDs differ from Alzheimer’s Disease (AD) in clinical presentation and pathology.
Alzheimer’s and Pick’s Disease: How They Differ
Clinically, Pick’s disease differs from AD in affecting personality before memory shows deterioration. Changes in personality can include poor social judgment, disinhibition, vulgarity, and restlessness. Some patients steal or show repetitive, compulsive behaviors. Many patients become irritable, agitated, or depressed. Others are more apathetic.
Self-awareness can be very limited. Language is affected early and eating behaviors are sometimes affected. Incontinence typically occurs earlier than in AD. A peculiar occurrence in some individuals is the development of artistic talents during the progression of dementia. Diagnosis is made on a clinical basis, although genetic testing can confirm some specific subtypes. Atrophy of the frontal and temporal lobes may be apparent on MRI.
The Progression of Pick’s Disease
Although some cases proceed slowly, Pick’s disease usually proceeds more rapidly than AD, on average taking only four to six years from diagnosis to death. Patients with behavioral changes tend to pursue a more rapid course.
Treatment of Pick’s Disease
Treatment using medications developed for AD sometimes aggravates the symptoms of FTDs. There is no specific medication for FTDs. Medications for FTD, therefore, are “off label” and symptom-oriented rather than disease-modifying or curative.
For challenging behaviors, non-pharmacologic therapy remains the most recommended approach. Antidepressant medications such as citalopram or sertraline are sometimes tried empirically. They have helped some patients but exacerbated the symptoms of others. Aricept® (donepezil) or Exelon® (rivastigmine), often the first medication for AD patients, have been reported to have inconsistent effects on people with FTDs. Sometimes they help, but sometimes they aggravate the symptoms. Similarly, the NMDA antagonist, Namenda® (memantine), has been reported helpful to some FTD patients and adverse to others.
Antipsychotic medications have sometimes been prescribed in an attempt to control impulsive or agitated behavior, but there is no consistent evidence to support their use in this way. Antipsychotic use should be clinically justified and combined with clear expectations regarding these medications’ possible risks and desired benefits.
When we think about dementia, we usually picture memory loss as the first sign. Pick's disease, and FTDs altogether, remind us that dementia has other faces as well. Language, personality, and behavior, affected early in Pick's disease and other frontal lobe dementias can deteriorate before prominent memory changes are noted. For clinicians and caregivers, this is a reminder that cognition is a broader term than memory, and that changes in personality or language, not just memory changes, require careful evaluation.
- Alzheimer’s Disease Toolkit (Helpful Information to Understand and Manage Alzheimer's Disease)
- Expert Information on Alzheimer's Disease (Articles)
- Frontotemporal Dementia (Article)
- What are the Stages of Frontotemporal Dementia (Article)
- Learn About Dr. Alois Alzheimer (Article)
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This content was last updated on: November 10, 2020
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