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Pick’s Disease Running head: PICK’S DISEASE Pick’s Disease in APA Style Mary Beth Moody Floyd E. Kellam High School 1 Pick’s Disease 2 Abstract This research paper is about Pick’s Disease, which is a form of frontotemporal dementia. It was documented in 1882 by neurologist Arnold Pick. It is a progressive dementia, and mainly affects people between the ages of forty and sixty. The disease affects the frontal and temporal lobes of the brain, as is found on brain scans such as an EEG. It focuses on many different facades of the subject, including its history, symptoms, causes, means of diagnosis, means of treatment, how to manage it, and its affect on the brain. Pick’s Disease 3 Pick’s Disease is a frontotemporal dementia documented in the late nineteenth century of Arnold Pick. Pick’s Disease is a very rare form of dementia, as most people only know of one type of dementia; Alzheimer’s. By affecting two lobes in the brain, Pick’s Disease can affect the entire bodies functioning, and will drastically alter a patient’s lifestyle. Although almost every case of Pick’s Disease occurs in middle age to elderly people, there are still rare incidents where Pick’s Disease is diagnosed to somebody younger than the typical age range of dementia. Frontotemporal dementia originates in the brain, and is a result of brain deterioration. Pick’s Disease was documented by Arnold Pick of Czechoslovakia in 1892. He discovered the disease after performing an autopsy on a patient who exhibited signs of dementia and speech loss. Arnold Pick stated that the dementia is most prominent in the frontal and temporal lobes, and is a result of brain cell destruction. It has obtained many other names, such as frontotemporal dementia, semantic dementia, lobar sclerosis, focal cerebral atrophy, primary progressive aphasia, and circumscribed brain atrophy (Pick's Disease: Frontal Lobe Dementia, 2008). Pick’s Disease is a progressive dementia, which makes it more difficult to have consistent symptoms. Some symptoms can include personality and socio-behavioral changes, an inability to plan and organize, and inappropriate social behavior. It is also characterized by obsessive behavior, hypochondria, a sleep pattern change, loss of muscle control, rigidity, and a speech loss (Pick's Disease: Frontal Lobe Dementia, 2008). The Diagnostic and Statistical Manual of Mental Disorders defines the symptoms as “changes in personality, deterioration of social skills, emotional blunting, behavioral disinhibition, and prominent language abnormalities” (First, 1994). University student Alisa Alexander writes that: Pick’s Disease 4 “There are three stages of the development of Pick’s disease. The first is usually psychological, behavior, and judgment problems. There may be a change in their social behavior. The second is the development of other symptoms i.e. lost of speech and obsessed behavior. The last is generalized dementia” (Alexander, 2002). In one case study, a 56 year old man diagnosed with Pick’s Disease eventually progressed to the state where he could only continually repeat six phrases. In another case study, a police officer had a slow deterioration over twelve years. It started with a hesitant speech, and then progressed to where he could no longer perform the simplest tasks without error. Eventually he got to the point where he could no longer add 3+2 or write his own name. Soon after that, the man became mute and progressed into a vegetative state (Penington, 1998). The behavior of a patient with Pick’s Disease changes in a different way for every patient. Sometimes the patient exhibits impulsiveness, or an obsessive/compulsiveness. They might want something extremely neat, or they might eat only one type of food. They can also become more aggressive, and become rude or impatient. A patient with frontotemporal dementia (FTD) might also become more promiscuous and have an increase in sexual exhibitionism. Emotionally, a patient with FTD could become extremely abrupt and insensitive. The patient may lose their sense of warmth and capacity for empathy (Rose & Russell, 2008). One of the main signs of Pick’s Disease is the loss or depreciation of speech ability. A victim of FTD will experience a reduction in vocabulary size and may have issues such as having difficulty finding a word. They have a hard time understanding speech or composing a sentence, which exhibits aphasia (“inability to use or understand language (spoken or written) because of a brain lesion” (Miller, 2006)). A patient with Pick’s Disease will be affected physically as well. They show problems with muscle rigidity, and have difficulty with movement and coordination (Rose & Russell, 2008). Pick’s Disease 5 It is not easy to be able to define who is at a high risk for getting frontotemporal dementia because it is so diverse. The disease is not found in any particular race, and it affects men and women alike (Alexander, 2002). Other researchers have found that it is slightly more common in women than men, though (Rose & Russell, 2008). The range of age of those affected with lobar sclerosis differs greatly between researchers as well, but the Diagnostic and Statistical Manual of Mental Disorders sets the range from the ages of fifty to sixty years (First, 1994). It has been looked at as a hereditary disease, and forty percent of cases of Pick’s Disease are thought to be hereditary (Rose & Russell, 2008). It is thought that approximately seven million Americans are afflicted with frontotemporal dementias, but only about five percent are diagnosed with Pick’s Disease (Rose & Russell, 2008). Not all cases are put definitely at the ages of fifty to sixty. In one case of Pick’s Disease, a young woman is diagnosed at the age of twenty-seven. She was evaluated as an extrovert as a child, but at age twenty-seven, she became withdrawn. Her productivity in the workplace declined, and she was eventually fired from her job as a secretary. She then started having incontinency of urine, couldn’t perform daily activities, fell often, and couldn’t perform simple tasks such as using silverware. Her appetite changed, and she suddenly was a voracious eater. She was admitted for testing after such changes, and findings were generally normal after brain scans such as an electroencephalogram. Only a few areas showed abnormal signals in the cerebral cortex, but those abnormalities were still unclear. Two months later, Pick bodies were found in testing in the cytoplasm of small and medium-size cortical neurons. Such ideas have been concluded about the presence of this kind of dementia in someone so young: “The terminological confusion of Pick disease makes the analysis of age at onset reported in the published literature very difficult. The use of the terms Pick disease and Pick complex to describe a clinical Pick’s Disease 6 syndrome of frontotemporal deficits with focal atrophy encompasses a variety of different diseases defined by their histological features., Some early ages at onset, for example, that described by Mowadat et al, were clinical diagnoses without histological confirmation. Pick disease needs to be considered in the differential diagnosis of behavioral and cognitive decline in young adults” (Rossor, 1999). The most major part of any mental disorder is how the brain is affected by the disease. Pick’s Disease occurs in the frontal and temporal lobes. The brain experiences cell degeneration, and in Pick’s Disease, that degeneration is most prominent in the thalamus, corpus striatum, and the subcortex. The thalamus is the part of the brain that processes information. The corpus striatum is the part of the brain that is involved with movement control, and the subcortex is “the nerve cells below the cerebral cortex or surface of the brain that control neurological functions” (Pick's Disease: Frontal Lobe Dementia, 2008). The degeneration in the corpus striatum is what primarily affects the body’s ability to move. In this form of dementia, the body moves more rigidly, an obvious result of deterioration of the corpus striatum. Alexander (2002), assesses that “Pick's disease affects the temporal lobes of the brain in 25%, frontal lobes in 25% and both frontal and temporal lobes in 50% of cases” (Alexander, 2002). Pick’s Disease is caused by the damage of five different types of nerve cells in a patient. Two out of those five nerve cells contain abnormal deposits of tau proteins (Pick's Disease: Frontal Lobe Dementia, 2008). Tau proteins are “Microtubule-associated proteins that are mainly expressed in neurons” and “Aggregation of specific sets of tau proteins in filamentous inclusions is the common feature of intraneuronal and glial fibrillar lesions in numerous neurodegenerative disorders” (Tau Proteins, 2009). The accumulation of tangles of these proteins in the frontotemporal lobes of the brain is one of the major causes of FTD. It is has also been proved that the inheritance of tau proteins puts a patient at an increased risk for Pick’s Disease (Pick's Disease: Frontal Lobe Dementia, 2008). Pick’s Disease 7 Another cause for Pick’s Disease is the presence of abnormalities in the nerve cells of the brain called Pick bodies. This is what distinguishes a frontal lobe dementia from other types of dementia. These Pick bodies are “fibrous tangles of tau proteins.” These tangles are different from those of the neurofibrillary tangles consistent with Alzheimer’s (Pick's Disease: Frontal Lobe Dementia, 2008). Pick bodies are also abnormally swollen (Alexander, 2002). Pick bodies lead to the changes in character common in Pick’s Disease (Rose & Russell, 2008). A diagnostic test for Pick’s disease can include a physical exam, clinical assessment, blood tests, neuro-psychology assessment, linguistic tests, or a CT or MRI scan (Pick's Disease: Frontal Lobe Dementia, 2008). Although all of the above tests can be proctored, the only conclusive way of determining the presence of Pick’s Disease is an autopsy of the brain. But without an autopsy, an EEG is also an effective way of finding a diagnosis (Rose & Russell, 2008). After a diagnosis has been determined, the life expectancy range varies widely from two to seventeen years, but averages at about eight years (Pick's Disease: Frontal Lobe Dementia, 2008). FTD is often misdiagnosed as depression in its early stages because of symptoms in the early stages of the disease. It is also often misdiagnosed as Alzheimer’s disease, as they are very similar (Rose & Russell, 2008). Pick’s Disease is very similar to and is often mistaken as Alzheimer’s disease. But there are some major differences that separate the two dementias. One big difference is what part of the brain they affect. Pick’s is only located in the frontal and temporal lobes. Alzheimer’s on the other hand can be located in various areas, including the posterior, temporal, parietal regions, and the hippocampus. Another difference is that Pick’s Disease has a range of about fifty to sixty while for Alzheimer’s it is extremely uncommon for there to be a diagnosis for a patient under sixty-five. Another difference is the symptoms. With Pick’s, the initial symptoms are Pick’s Disease those of behavior and a change in lifestyle. Alzheimer’s initial symptoms are memory loss (Pick's Disease: Frontal Lobe Dementia, 2008). There is no cure for the actual dementia of Pick’s Disease, but researchers are searching for a cure desperately. Current treatments for Pick’s include tranquilizers, antidepressants, serotonin-based supplements, and behavioral therapy (Pick's Disease: Frontal Lobe Dementia, 2008). Even with medication though, it is near impossible to go back to living a normal life. Medication is used primarily to treat the behavior symptoms (Alexander, 2002). 8 Pick’s Disease References Alexander, A. (2002, February 27). Pick's Disease. Retrieved March 11, 2009, from http://serendip.brynmawr.edu/bb/neuro/neuro02/web1/aalexander.html First, M. (1994). Diagnostic and Statistical Manual of Mental Disorders DSM-IV-TR Fourth Edition (Text Revision). Arlington, VA: American Psychiatric Publishing. Miller, G. (n.d.). WordNet Search - 3.0. Retrieved March 11, 2009, from http://wordnet.princeton.edu/perl/webwn Odawara, T. (2003). Alterations of muscarinic acetylcholine receptors in atypical Pick's disease without Pick bodies. Journal of Neurology, Neurosurgery, & Psychiatry, 74(2), 965. Penington, M. (1998, May 26). What Are The Symptoms of Picks Disease from Alzheimer's Outreach. Retrieved March 11, 2009, from http://www.zarcrom.com/users/alzheimers/odem/pk2.html Pick's Disease: Frontal Lobe Dementia. (n.d.). Retrieved March 11, 2009, from http://www.about-dementia.com/articles/about-dementia/dementia-causes/dementiapicks-disease.php Rose, A., & Russell, D. (2008, November 20). Pick's Disease: Symptoms and Prognosis. Retrieved March 11, 2009, from http://www.helpguide.org/elder/picks_disease.htm Rossor, M. (n.d.). Arch Neurol -- A Case of Sporadic Pick Disease With Onset at 27 Years, October 1999, Jacob et al. 56 (10): 1289. Retrieved March 11, 2009, from http://archneur.ama-assn.org/cgi/content/full/56/10/1289 9 Pick’s Disease Tau proteins. (2009, March 6). Retrieved March 11, 2009, from http://ghr.nlm.nih.gov/glossary=tauproteins 10