Causes and Symptoms (Magill’s Medical Guide, Sixth Edition)
Pick’s disease, also called frontotemporal dementia (FTD), is similar to, but much rarer than, Alzheimer’s disease. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia. Some medical researchers propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the whole group the Pick complex. The differing terms reflect different theoretical models of the disease, and specialists are likely to continue to debate these models.
In only 10 to 20 percent of cases of FTD is a specific cause, an autosomal dominant genetic trait, speculated. In these cases, there is a family history of someone showing symptoms of a frontal lobe dementia. In 60 to 90 percent of cases, there is no evident family (genetic) history and the cause is unknown. FTD is usually not inherited.
Onset is slow and insidious. Tissues shrink (atrophy) in the frontal and temporal brain lobes. FTD also causes some brain cells to develop abnormal fibers called Pick’s bodies. The cells in these bodies have an abnormal amount of a protein called tau. Tau appears throughout the body’s cells but exists in abnormally high amounts in Pick’s bodies and in Pick cells that exist inside normal brain cells (neurons). These form elsewhere in the brain and are not limited to the frontotemporal regions. These fibers are generally straight and single, as...
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Treatment and Therapy (Magill’s Medical Guide, Sixth Edition)
At present, there are no medications that can effectively treat FTD, although several can help treat many of its symptoms. Medications used in Alzheimer’s disease are not routinely prescribed because they often increase aggression in Pick’s disease patients.
Maximizing quality of life is the key treatment, and many of the more disturbing behaviors respond well to medication, including aggression and agitation. In addition to adding medications for symptom control, discontinuation of medications that promote confusion or that are not essential to the care of the person may improve cognitive function. It is common for anticholinergics, analgesics, and central nervous system depressants to be discontinued. Because many of the functions that FTD affects are also affected by low levels of thiamine, thiamine supplementation is often recommended.
Behavior modification is often the treatment of choice in controlling unacceptable or dangerous behaviors. Rewarding and reinforcing appropriate, positive behaviors while ignoring inappropriate, negative behaviors (within the bounds of safety) can significantly influence how patients act and interact. Formal psychotherapy is seldom effective because it overloads patients’ limited cognitive resources. Reality orientation, with repeated reinforcement of environmental and other cues, can reduce disorientation and agitation. Sensory functions, often overlooked, should be...
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Perspective and Prospects (Magill’s Medical Guide, Sixth Edition)
Pick’s disease affects about 1 out of 100,000 people, accounting for 0.4 percent to 2.0 percent of all cases of dementia. More common in women then men, it typically has an onset between ages forty and sixty, with a modal age of fifty-four, but it has been known to affect patients as young as twenty. A family history of FTD is considered a risk factor, although most Pick’s patients have no family history of the disease.
The first description of the disease was published in 1892 by Arnold Pick. Until recently, it was thought that Pick’s disease could not be distinguished from Alzheimer’s disease during life. In accordance with major research criteria of German neuropsychiatry, Pick’s atrophy was constructed as a full-blown disease entity in the 1920’s. This concept gained acceptance in the German and Anglo-American scientific community and was the starting point for further investigations in the 1950’s and 1960’s.
Initial diagnosis is mostly based on history and symptoms, signs, and tests and by ruling out other causes of dementia, especially those with metabolic causes. The development of neuropsychological assessment procedures and the use of electroencephalograms (EEGs), computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans are generally necessary in the prediagnostic workup of the disease. Functional brain imaging, such as single photon emission computed tomography...
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For Further Information: (Magill’s Medical Guide, Sixth Edition)
Frederick, Justin, “Pick Disease: A Brief Overview.” Archives of Pathology and Laboratory Medicine 130 (July, 2006): 1063-1066.
Kertesz, Andrew, and David G. Munoz, “Frontotemporal Dementia.” Medical Clinics of North America 86 (2002): 501-518.
National Institute of Neurological Disorders and Stroke (NINDS). Frontotemporal Dementia. Bethesda, Md.: National Institutes of Health, 2006.
“Non-Alzheimer’s Forms of Dementia.” In The Mayo Clinic Guide to Alzheimer’s Disease: The Essential Resource for Treatment, Coping, and Caregiving. Rochester, Minn.: Mayo Clinic, 2002.
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