Frontotemporal dementia (FTD), originally known as Pick’s disease, is a rare form of dementia that is associated with shrinking of the frontal and temporal anterior lobes of the brain . The name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior; and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Spatial skills and memory remain intact. Although the exact etiology of Pick’s diseases is not known, there is a strong genetic component to the disease; FTD often runs in families.
Pick’s disease was first described by Arnold Pick, a Czechoslovakian physician who was trained in clinical neurology, psychiatry, and neuropathology. In 1892, Pick reported on a 71-year-old man with progressive loss of language and mental deterioration. After the man died, autopsy revealed asymmetrical atrophy of the frontal cortex of the brain. In 1911, Alois Alzheimer confirmed the pattern of atrophy found in brains of patients with Pick’s disease. The term Pick’s disease was coined by A. Gans in 1922.
The cortical atrophy seen in Pick’s disease is different from Alzheimer’s disease , although there are
major overlaps with Alzheimer’s presenile dementia. In Pick’s disease, shrinkage is greatest in the frontal and temporal lobes. One of the characteristics of Pick’s disease is microtubule-associated tau proteins, which are the main cytoskeletal components modified during the neurodegenerative changes associated with this disease. In Alzheimer’s disease, on the other hand, any area of the brain may be affected. Abnormalities called Pick bodies and Pick cells, abnormally swollen nerve cells, are also found in the brains of individuals with Pick’s disease. Pick bodies are found inside nerve cells and contain the abnormal form of tau protein that is associated with Pick’s disease.
Researchers continue to debate how to classify Pick’s disease. Today, few researchers use the term Pick’s disease, although it is still used by patients, care-givers, and some health practitioners. Currently, Pick’s disease is considered to be part of a syndrome that includes not only Pick’s disease but also primary progressive aphasia and semantic dementia, which are two related disorders. The syndrome is known as FTD. Some researchers have suggested that some cases of frontotem-poral dementia in which Pick bodies or Pick cells are absent may also represent a form of Pick’s disease.
Pick’s disease is rare, affecting less than 1% of the U.S. population. It accounts for about 2–5% of all cases of dementia. Although it sometimes appears in younger or older people, it typically begins in middle age, between the ages of 50 and 60 years. The average age of onset is 54 years and it tends to occur more often in women than in men.
The symptoms of Pick’s disease vary among individuals, but changes in behavior, emotions, and language are frequently associated with neurological problems related to movement and memory. Behavioral changes include disinhibition, inappropriate behavior, compulsions such as a tendency to overeat or eat a particular kind of food, repetitive behavior, social withdrawal, inability to keep a job, difficulty initiating tasks and following through, difficulty maintaining personal hygiene, and a short attention span. Emotional changes include mood swings, inappropriate mood, lack of concern for the feelings of others, apathy , and indifference to behavioral changes. Language changes include decreased ability to read, write, speak, and understand language. Speech difficulties may range from difficulty finding words and diminished vocabulary to a complete inability to speak. Patients also sometimes display echolalia, or a tendency to repeat the words of others. Patients may also experience difficulty with movement and coordination, muscle weakness or rigidity, and progressively worsening memory loss. Urinary incontinence may also occur.
In the early stages of Pick’s disease, patients frequently demonstrate personality changes that are manifested as inappropriate behavior. This is in contrast to Alzheimer’s disease, which, in its early stages, is characterized mainly by memory loss. As Pick’s disease progresses, patients become aphonic and apathetic. They eventually lapse into a vegetative state and become completely disabled. Death occurs because of malnutrition, infections, or general failure of body systems.
Diagnosing Pick’s disease is difficult, because symptoms overlap with those of other disorders, such as Alzheimer’s disease and other dementias that affect the frontal lobes of the brain. According to the National Institutes of Health, at the present time, a definitive diagnosis can only be made with a brain biopsy, which is an invasive procedure in which a small sample of brain tissue is surgically removed for examination. Other diagnostic methods are more commonly used, which allow a diagnosis to be made by ruling out other causes of dementia.
Diagnostic procedures include a detailed clinical evaluation to assess personal and family health history, other medical conditions, overall health status, use of prescription or non-prescription drugs, current symptoms, and changes in daily functioning. Blood tests may be done to detect problems in organ function, hormone levels, and vitamin deficiencies. Neurologic exams may be performed to determine which areas of the brain are affected, which can include electroencephalography (EEG), computerized tomography (CT) scans, and magnetic resonance imaging (MRI) scans. A psychiatric evaluation may be carried out to determine whether the patient suffers from disorders suchas depression , which can mimic or worsen the symptoms of Pick’s disease.
There is no cure for Pick’s disease, and currently, there are no known medications that slow the progression of the disease. Medications that are used to treat Alzheimer’s disease should not be used to treat Pick’s disease, because they may increase aggression in patients.
Analgesics —Drugs that reduce pain.
Anticholinergics —Drugs that block the action of acetylcholine, a naturally occurring chemical that is involved in communication between nerve cells.
Atrophy —Shrinkage or deterioration.
Cimetidine —A drug that decreases the amount of acid in the stomach, and that is used to treat conditions such as ulcers, gastroesophageal reflux disease, and heartburn.
Computerized tomography (CT) scan —An imaging technique in which x rays are taken of the brain from several different angles and combined through a computer to provide an image of the brain.
Electroencephalography (EEG) —A recording of the electric potentials of the brain from electrodes attached to the scalp.
Frontal lobe —A part of the brain that is involved in processes such as muscle movement, speech production, working memory, planning, reasoning, and judgment.
Hypoxia —Oxygen deficiency.
Lidocaine —A local anesthetic.
Magnetic resonance imaging (MRI) scan —An imaging technique in which magnetic fields, radio waves, and computer enhancement are used to create an image of brain structure.
Primary progressive aphasia —A disorder in which there is progressive loss of language skills.
Semantic dementia —A disorder in which there is progressive loss of knowledge about words and word meanings.
Temporal lobe —A part of the brain that is involved in processing auditory and visual information, emotion and motivation, and understanding language.
Treatments for Pick’s disease are designed to manage its symptoms. Behavior modification strategies, which involve rewarding appropriate behavior, may help to decrease unacceptable or dangerous behaviors. Speech therapy may be helpful for increasing language use. Occupational therapy may be used to help patients improve performance of daily living tasks. Encouraging new hobbies may help to relieve boredom in patients and decrease behavior problems.
Disorders that exacerbate confusion, such as heart failure, hypoxia, thyroid disorders, anemia, nutritional deficiencies, infections, and depression, should be treated. Medications that increase confusion, such as anticholinergics, analgesics, cimetidine, central nervous system depressants, and lidocaine, should be stopped if they are not clearly needed. In some cases, medications may be prescribed to treat aggression, agitation, or dangerous behavior.
In the early stages of Pick’s disease, legal advice may help families make ethical decisions about caring for a patient.
As the disease progresses, patients may require constant monitoring and care, either at home or in an institutionalized setting. Help from visiting nurses and aides, volunteer workers, and adult protective services may be needed. Families may benefit from counseling, to help them deal with the difficulties of caring for patients. Support groups can also be a helpful resource for families.
The prognosis for Pick’s disease is poor. It is a rapidly progressing disease. Death commonly occurs between 2 to 10 years after the onset of the disease.
There are currently no known ways of preventing Pick’s disease.
See also Elder care.
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“NINDS Frontotemporal Dementia Information Page.” National Institute of Neurological Disorders and Stroke. 2006 http://www.ninds.nih.gov/disorders/picks/picks.htm
Association for Frontotemporal Dementias (AFTD),100 North 17th Street, Suite 600, Philadelphia, PA 19103. Telephone: (267) 514-7221, (866) 507-7222.http://www.FTD-Picks.org
NIH Neurological Institute, P.O. Box 5801, Bethesda, MD 20824. Telephone: (800) 352-9424, (301) 496-5751.TTY (for people using adaptive equipment): (301) 468-5981
Ruvanee Pietersz Vilhauer, PhD