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Introduction and InstructionsWelcome to the Continuing Medical Education course, Early Recognition and Treatment of Dementing Disorders. To begin, print or write down the following instructions. Complete these steps in order to register for and receive CME credit.1. Go to http://cme.uwisc.org/index.pl?iid=274518 (opens new browser window). 2. Click on and complete the "Account Sign-up" (top right) to set up your password. You only need to do this once. 3. Click on "Register for Credit" under Course Materials. 3. Come back to this page (browser window should still be open) and read the course content below. 4. Complete and submit the post-test and evaluation online (http://cme.uwisc.org/index.pl?iid=274519&isa=Eval&op=show). A passing score of 70% or higher is required to receive credit. If you do not pass, you must take the post-test again until you achieve that score. 5. Print credit letter immediately after successful completion of the post-test and evaluation. This educational activity is part of the Memory Screening Initiative, funded by the Wisconsin Department of Health and Family Services, and supported by an educational grant from Forest Laboratories, Inc.
At the end of this course, you will
be able to:
Course content begins below. Dementia is UnderdiagnosedThere is significant data from numerous sources to suggest that 30-50% of persons with dementia are never diagnosed and as a result, are never treated. In addition, most persons who are treated for dementia are treated inappropriately. Although current treatments have been shown to result in statistically significant improvements in cognition, these benefits are small for individual patients. As a result, treatments are often stopped because of the absence of clinically meaningful cognitive benefits. As we will show, there is evidence to suggest that one benefit of treatment, especially with acetylcholinesterase inhibitors, is to increase the odds that a person will have a slow rather than a rapidly progressive course. Studies have shown that acetylcholinesterase inhibitors slow hippocampal atrophy and in animals, reduce the rate of beta amyloid deposition in amyloid plaques. Both of these are pathologic hallmarks of progressive AD.A study illustrating the extent of and the potential consequences of the failure to diagnose AD comes from Nebraska (see Figure 1). This was a study of 230 residents living in 7 assisted living facilities in Nebraska. The study was very simple and researchers administered the Mini Mental State Exam defining cognitive impairment or dementia as a score below 24. The researchers found that 63% of the residents in the assisted living facilities were cognitively impaired. Of those, 63% had no diagnosis and 75% were untreated. Twenty-two percent of these cognitively impaired persons were being asked to self-medicate themselves. In addition, only 11% had a surrogate decision-maker (see Figure 2). This study is one of many documenting not only the under-recognition of cognitive impairment, but also the potential consequences of failing to identify persons with dementing disorders. Figure 1
Figure 2
To determine the extent of unrecognized cognitive impairment in primary care practices at the University of Wisconsin, we examined 200 “healthy” General Internal Medicine patients (Hermann, Sager, 2002). The study population averaged 75 years of age and was identified by their primary care physicians as not having a diagnosis of dementia or complaints of memory loss. Twenty-one percent of those undergoing extensive neuropsychological testing were found to have unrecognized dementia or mild cognitive impairment, a potential precursor to the development of AD (see Table 1). The cumulative evidence including this study suggests that the current medical approach to dementing illnesses is primarily one of neglect.
There are many reasons for the high rate of undiagnosed cases of dementia. Families are often reluctant and apprehensive about seeking medical consultation after signs of cognitive impairment appear in a family member. On average, families have been found to delay seeking medical help for almost three years after the onset of symptoms. Reasons for the delay in seeking physician consultation are that families are not sure of the meaning and severity of initial symptoms, think that memory impairment is part of normal aging, are afraid to discuss symptoms with the patient, are frightened by the anger of the patient when symptoms are discussed and are afraid of the diagnosis of AD. In a recent Metropolitan Life Foundation survey, AD was listed as one of the most feared diagnosis in medical practice. In addition to reluctance of families to seek help early, most patients may be unaware that they have significant problems with cognition. Early symptoms of memory loss or other changes in cognition are very subtle and are often attributed to old age. Finally, cognitive impairment is given a low priority in primary care medical practice, further complicating the identification of dementing disorders. The Barriers to Diagnosis and Treatment of DementiaTo better understand the barriers to the diagnosis and treatment of dementia in medical practice, the Wisconsin Alzheimer’s Institute and the University of Wisconsin Office of Continuing Professional Development in Medicine and Public Health conducted a statewide needs assessment. There were multiple methods of data collection, including surveys, telephone conversations with opinion leaders and focus group meetings occurring in four sites throughout the state. The results of the needs assessment were as follows:• The participants in the needs assessment identified very specific gaps in current knowledge that served as barriers to the diagnosis and treatment of dementing disorders. These barriers included the inability to identify persons needing evaluation (the absence of routine screening); absence of readily available and easily interpretable diagnostic tools; inability to monitor the effectiveness of current therapies; and lack of knowledge about available resources for patients and families affected by dementing disorders. • Although deficiencies in current knowledge created well-defined barriers to the diagnosis and treatment of persons with dementia, these were accentuated by obstacles identified in medical practice. Practice obstacles included 15 minute appointments, inadequate reimbursement for the time required to make a diagnosis, reliance on families to bring symptoms of cognitive impairment to the attention of the physician (the absence of screening and diagnostic tools), administrative pressure to “produce” and the absence of a social worker and/or supportive staff to assist families and patients with dementia. • Deficiencies in current knowledge in practice obstacles also caused uncertainties about the clinician’s ability to diagnosis dementing disorders, manage complications of dementia and deal with the multiple needs of families. These uncertainties about the ability to diagnose fueled skepticism about the benefits of treatment and an attitude of “Why bother?” One important comment was that making a diagnosis of dementia was like “opening a can of worms” creating an added burden on an already busy primary care practice. The Importance of Early Diagnosis of Alzheimer's DiseaseWhy bother? Although the effectiveness of current therapies for AD are less than ideal, there is emerging evidence that therapies for AD improve cognition, slow functional decline, lower Medicare costs and reduce the rate of institutionalization. In addition, there is increasing evidence that one benefit of acetylcholinesterase therapy may be to slow progression of AD and as a result, reduce the risk of nursing home placement. Lopez et al., J Am Geriatr Soc 2005; 53:83-87, described a group of persons with AD (see Table 2) who had a slow progressive course defined as a loss of less than 2 points on the Mini Mental Statue Exam each year. They found that a major benefit of treatment was to increase the likelihood (by 2.4 times) that a person would have a slow instead of a rapid disease progression (see Figure 3). After 3 years, 50% of the untreated group was institutionalized versus 11% of the treated group. There is accumulating evidence that, for some patients, the effect of current treatment is to slow hippocampal atrophy which is a pathologic hallmark of disease progression.
Figure 3
The importance of the Lopez study was to suggest that current therapies may not have dramatic benefits in cognition, but over time, may have an effect on the rate of progression for some persons. Slowing the rate of progression and lowering the use of nursing homes are important outcomes. Eighty percent of AD expenditures are for nursing home care. The rate of institutionalization for AD patients is 10% per year versus 1% per year for non-demented persons (see Figure 4). In Wisconsin, there are 18,000 persons living in nursing homes who meet the criteria for dementing illnesses. Depending on source of payment, the costs of providing institutional care range between $595 million for Medicaid and $829 million for private pay persons. As our society ages, these expenditures for nursing home care and other forms of long-term care will climb dramatically with an increasing prevalence of dementing disorders. Figure 4
Early diagnosis also provides the opportunity for caregivers to be educated about the disease and to find support for their caregiving role. Recent studies (Mittelman, et. al., Neurology, 2006;67: 1592-1599) site greater caregiver access to effective programs of counseling and support can yield considerable benefits including delayed institutionalization. The local Alzheimer’s Association is a resource that provides knowledgeable staff to answer the many questions that caregivers may have early in, and throughout, the disease process, e.g. what is happening to the brain, how to communicate when words fail the person with dementia, how to plan for financial and legal issues in the future, what to do about driving, etc. Their toll-free, 24 hour/7 days a week phone number is 800-272-3900. Memory Screening and Early Diagnosis Projects in WisconsinBecause of the potential economic and social consequences of dementing disorders in the future, the Wisconsin Department of Health and Family Services is conducting a pilot cognitive screening program exploring how to promote earlier recognition, better treatment and management of persons with dementing disorders.The Wisconsin Memory Screening Initiative is now providing cognitive screening for older adults in 10 Wisconsin Counties (see Figure 5). It is a collaborative project of the Wisconsin Department of Health and Family Services (DHFS), the Wisconsin Alzheimer’s Institute (WAI), and participating county long-term care programs and is supported by grants from the Centers for Medicare and Medicaid Systems and the Administration on Aging to the Wisconsin DHFS. The overall plan entails administration of a simple and effective memory screening tool on persons over age 65, additional testing of those who screen positive, and subsequent referral to physicians. Other aims of the project include improving communication between physicians and county care managers, and increasing awareness of patients and family members of community-based dementia care resources. Figure 5 Screening ToolsSeveral screening tools have been developed for detecting cognitive deficits in older adults. The best known is the Mini-Mental State Examination (MMSE) developed by Marshall Folstein and colleagues (sample MMSE form). Although the MMSE is useful in detecting dementia at moderate stages of severity, several studies have shown that it not sensitive to mild stages of dementia. For example, in data collected through the network of memory diagnostic clinics affiliated with the WAI, 40% of patients found to have AD on full diagnostic workup scored 24 or above on the MMSE, which is the cut-score typically used for detecting cognitive impairment (see Table 3).
Clock Drawing is another commonly used cognitive screen (sample clock). This screen takes only two to three minutes to administer, and it has been shown relatively high sensitivity and specificity for detecting cognitive impairment. In our WAI clinics study, Clock Drawing detected 87% of cases found to have AD on full diagnostic workup (see Table 4). Although this is a useful screen, we decided not to include it in the Memory Screening Initiative for two reasons: (1) there is no single scoring system used by all or most practitioners; and (2) Clock Draw is sensitive to mild dementia only if stringent scoring rules are applied (i.e., near-perfect drawings are required), and not all practitioners are comfortable with such stringent scoring.
Another quick but useful screening tool is verbal fluency. A 60-second Animal Naming screen has been shown to have good sensitivity and specificity for detecting the semantic memory impairments that occur in AD and several other dementias. In our WAI clinics study, Animal Naming detected 91% of cases of AD and 90% to 100% of other common dementias when a cut-off score of 17 is used (see Table 5).
Screening Tools Used in the Memory Screening InitiativeThe Wisconsin Memory Screening Initiative selected Animal Naming as the initial brief screen in a 2-step cognitive screening process. Individuals who screen positive on Animal Naming are asked to complete a second, more comprehensive screen, the Cognistat (also known as the Neurobehavioral Cognitive Status Examination). The Cognistat was developed by Ralph J. Kiernan, PhD, Jonathan Mueller, MD, and J. William Langston, MD to provide a brief, differentiated assessment of several areas of cognition that are important for everyday function. Individuals who have deficits on the Cognistat are encouraged to make an appointment with their physician for an evaluation of possible cognitive problems.Animal Naming: In this project, Animal Naming scores of fewer than 14 appropriate words are considered positive outcomes in order to minimize false positive results. This cut score is based on data collected in the WAI clinics study, which showed a sensitivity of 85% and specificity of 88% for this cut score (see Table 6). Instructions for administering Animal Naming are very simple. The examiner says, “Tell me the names of as many animals as you can think of as quickly as possible” and then records responses for 60 seconds. Repetitions are not counted toward the total score, nor are non-animal words. In this example, the total correct score is 11….a positive outcome (sample Animal Naming form).
The Cognistat: When a person screens positive on the Animal Naming screen, a more detailed Cognistat is administered. The Cognistat tests seven areas of cognitive ability and takes 10 to 30 minutes to administer (about 20 minutes for outpatients). • Orientation
The memory item is a particularly important item for dementia screening (see Table 7). Patients are asked to remember four words that they had learned 8 to 10 minutes earlier. They are given a chance to remember these words on their own (free recall), and for items not recalled, they are given cues and/or multiple choices to jog their memories. This permits identification of different types of memory problems (e.g., retrieval problem only vs. inability to retain new information). Another valuable task on the Cognistat is evaluation of judgment (practical reasoning) (see Table 7). Patients are asked to provide the best course of action for three everyday problem situations. Most cognitive screening tools do not evaluate practical reasoning in a standardized way, even though this skill is crucial for independent living.
Another useful aspect of the Cognistat is the profile format for summarizing results. A first sample profile shows results for an older adult with normal cognitive abilities (see Figure 6). The second sample profile shows results for a patient with mild dementia (see Figure 7). The low point on this profile is for memory, and construction scores are also below expected levels. The “mild” level of dementia in this case is worth emphasizing. Often, individuals with this level of cognitive impairment will appear normal in general conversation, especially in brief appointments pertaining to familiar conditions or situations. Cognitive screening is needed to identify difficulties at this stage. Figure 6
Figure 7 The Cognistat has been used as a cognitive screening tool in many geriatric clinics and memory diagnostic centers across the U.S. It is a copyrighted test and is available for purchase from the Northern California Neurobehavioral Group, Inc. (www.cognistat.com or 800-922-5840). In a recent clinical study (van Gorp et al., 1999), the Cognistat’s sensitivity to dementia (Alzheimer or vascular types) was 100% and specificity was 83%, when impairment was defined as a deficit on one or more component scales. Other studies contrasting diverse “brain-damaged” samples with normal older adults have also found significant differentiation, but sensitivities and specificities have been lower (e.g., Roper et al., 1996). The steps in the Wisconsin Memory Screening Initiative are summarized in this flow diagram (see Figure 8). Only those individuals who obtain low scores on both Animal Naming and the Cognistat are referred to physicians for a diagnostic workup. Mild memory problems can be caused by many factors, and a medical workup is needed to identify possible treatable causes or coexisting conditions that can exacerbate cognitive symptoms. For individuals found to have AD, vascular dementia, or dementia with Lewy bodies, treatment with a cholinesterase inhibitor may help to slow the rate of progression, as discussed previously. For dementias of all types, family and patient education and other community supports (e.g., respite care for primary caregivers, early-stage activity based programs for patients, etc.) are crucial for enabling individuals and their families to live as well as possible with their disease. The Memory Screening Initiative encourages ongoing communication between medical professionals and community support programs offered through county Health and Human Services departments and local chapters of the Alzheimer ’s Association. Figure 8
Findings being monitored in the Memory Screening Initiative include screening outcomes and rates of follow-through on referrals for dementia workups. Ultimately, we also hope to measure the impact of this screening program on utilization of health care and long-term care services and their associated costs. If you have questions about cognitive screening tools or preliminary outcomes of the Wisconsin Memory Screening Initiative, contact Asenath La Rue, PhD, at the Wisconsin Alzheimer’s Institute (larue@wisc.edu or 608-829-3308). Dementia: Differential DiagnosisThe prevalence of different dementing disorders varies depending upon the patient population being studied. This graph summarizes an autopsy-based review of dementing disorders in a geriatric practice. In geriatric and primary care practices, Alzheimer’s disease (AD) accounts for 60-80% of all dementia while the prevalence of Lewy body and vascular dementias is relatively low. In a neurologic practice, the prevalence of Lewy body and vascular dementias would be much higher. Regardless of the patient population, AD is the most common form of dementia (see Figure 9). It is also a heterogeneous disease frequently found in combination with other conditions, such as Lewy body dementia and cerebrovascular disease. Because AD occurs in older adults, most persons with AD have a vascular component to their dementia that may not be the primary cause of the disease, but may contribute to cognitive impairment.Figure 9 Diagnostic ProtocolThe DSM-IV diagnostic criteria (see Table 8) for dementia include documented memory impairment and one or more of the following: aphasia, apraxia, agnosia and a disturbance in intellectual functioning. These cognitive deficits must be associated with functional impairment and must be present in the absence of delirium. This suggests that the new diagnosis of a dementing disorder should not be made in the context of acute illness or without a review of potential causes of delirium, e.g., drug reactions. In addition, the diagnosis of dementia requires that other potentially contributing medical and psychiatric conditions, such as schizophrenia, be ruled out.
Evaluation of a patient who has a potential dementing illness should include a history, mental status evaluation, physical examination, limited laboratory testing, and in many cases, neuroimaging, more extensive neuropsychological testing and a depression screen. By far, the history is most important because most dementing disorders have characteristic clinical manifestations that can only be determined in the history. Importantly, the history must be obtained from both the patient and reliable observers, such as family members. Many persons with significant memory impairment will not be aware of their cognitive deficits and frequently deny that they have any problem and should not be considered reliable historians.
The mental status evaluation as discussed in the first module should include
an Animal Naming screen and a Cognistat or equivalent screen, and if desired,
a Mini Mental State Exam and Clock Draw. The physical examination is done
to rule out potential medical and neurological causes of dementia, especially
evidence for cerebrovascular accidents or space-occupying lesions.
The laboratory testing has been simplified and as recommended by the American Academy of Neurology, includes a CBC, electrolytes, glucose, BUN and creatinine, serum B12, thyroid stimulating hormone and liver function tests.
Because of the small yield of positive results, neuroimaging is not recommended on a routine basis for obtaining a dementia diagnosis. Either an MRI or CT of the brain should be considered in cases in which the onset is before the age of 65, the onset or progression of cognitive symptoms is abrupt, atypical features are present that might suggest a brain lesion and seizures are present. These recommendations are designed to alert clinicians to the possible presence of space-occupying lesions, infection or cerebrovascular infarction.
The standard neuropsychological test battery used in the assessment of cognitive impairment can be extensive and frequently 3-4 hours long. Many older adults with dementia are intolerant of extensive testing. In addition, many primary care physicians may not have ready access to comprehensive neuropsychological testing. Because of that, comprehensive neuropsychological testing is recommended for cases in which the onset of dementia is before age 65, there are atypical patterns of cognitive problems (e.g., varying courses), there is a discrepancy between clinical findings and history, and there are combined mood and cognitive disorders. Comprehensive neuropsychological testing may be important in establishing a baseline for future comparison and is always required for research level diagnoses.
Case #1: Mild Cognitive ImpairmentThe first case is used to illustrate the importance of doing additional tests when the more common cognitive screening measures are normal in the presence of new onset memory loss. Case #1 is that of a 78 year old woman with 12 years of education. She is married and formerly worked as a bank teller. She presents with a 1½ year history of short-term memory loss that is new, but is not associated with significant functional changes. She remains independent in all instrumental activities of daily living. She has continued to drive and has not gotten lost, but has increased the use of lists as memory aids. As a result, there are no significant functional changes noted on history, either from the patient or from her husband. The laboratory evaluation and physical examination were normal. Her medical history was positive for hyperlipidemia, coronary artery disease and treated hypothyroidism.Her screening tests as indicated are normal in spite of the history of new cognitive impairment (see Table 9). The Cognistat, however, is abnormal and shows an isolated and moderately severe memory deficit (see Figure 10). The isolated memory impairment without functional consequences rules out the diagnosis of dementia. This person is suffering from amnesic (memory only) mild cognitive impairment (MCI).
Figure 10
As originally described, the syndrome of mild cognitive impairment (MCI) included subjective memory complaints that were accompanied by objective memory impairments found on cognitive testing. Since the original description by Peterson, the requirement for subjective memory complaints has been dropped. Many cases of MCI are without subjective complaints. The diagnosis of MCI also requires that screening instruments such as the MMSE are normal, and that memory complaints are accompanied by little or minor functional impairment. Complex functional tasks are most likely to be affected (e.g., preparing tax returns, learning new software programs), and it is important to ask if the individual is attempting tasks of this type. There may also be subtle changes in mood or personality, including increased anxiety, self-centeredness or diminished interest in usual activities. Our understanding of MCI as a clinical entity has also become more sophisticated to recognize that there are different types of mild cognitive impairment (see Table 10). The amnesic or isolated memory type of MCI was the first described. There are also cases of MCI in which memory and other cognitive domains, such as executive functioning or visuospatial abilities are affected. These MCI variants may or may not progress to a dementing disorder.
As shown in Figure 11, the annual rate of conversion of amnesic MCI to AD is between 12-16%. Over a 4-year period of time, approximately 48% of persons diagnosed with amnesic MCI would be expected to convert to AD. Recent studies suggest that multiple domain MCI (memory plus another cognitive domaine) is the form of MCI most likely to convert to AD. Figure 11
The National Institutes of Health (NIH) recently conducted a clinical trial evaluating the effectiveness of an antioxidant, vitamin E, and donepezil in the treatment of MCI. This was a national study that randomized persons into three groups: control, donepezil or vitamin E. All persons received a multi-vitamin and were followed for three years with cognitive testing every 6 months. The donepezil arm of the clinical trial experienced a 36% reduction in the rate of converting to AD in the first 24 months (see Table 11). However, at the end of 36 months, this statistically significant benefit disappeared. For those persons who had the apolipoprotein ε4 gene, a risk factor for AD, the reduced rate of conversion to AD gradually diminished over time, but persisted for the 36-month study period. Persons with the APOE ε4 allele had a 34% reduction in the risk of converting to AD over the 3-year study period. When corrections for multiple comparisons were made, the APOE ε4 effect was only marginally significant at p = .07. Persons with APOE ε4 accounted for 76% of conversions to AD in this study. Because of this, researchers hypothesize that this study may have been underpowered to detect a statistically significant effect except for those with the ε4 allele.
Oral vitamin E was ineffective and did not influence the conversion rate to AD (see Table 12). It is unclear yet whether vitamin E may play a role in prevention of MCI or AD, although several epidemiologic studies show a decreased risk of AD when higher levels of vitamin E are obtained through diet, as opposed to supplements.
Case #2: Alzheimer's DiseaseCase #2 is presented to illustrate the importance of history in making a diagnosis of dementing disorders. The case is that of a 72 year old woman who is married and has a Bachelor of Science degree. She worked as a school secretary and presents with a 9-month history of word-finding difficulty and short-term memory loss. She and her husband deny any significant progression and insist that memory loss has not been affecting her level of function in everyday life. Her laboratory, MRI and physical examination were unremarkable. Her medical history also was negative.The results of the cognitive screens included a normal Mini Mental State Exam, an abnormal Animal Naming and an abnormal Clock Draw (see Table 13).
Because of the abnormal cognitive screens, a Cognistat was done and showed moderate-to-severe deficits in multiple areas of cognition: memory, construction or visuospatial skills and orientation (see Figure 12). For someone who has a Bachelor of Science degree, this represents a significant decline from a previous level of cognitive functioning. However, a diagnosis of dementia could not be made because of the absence of functional impairment. Figure 12
Because the history was inconsistent with the findings on the cognitive testing, her daughter was contacted and reports a decline in her mother’s functioning. The daughter reports that her mother has lost interest in reading, has problems shopping for groceries and has gotten lost while driving. In addition, her mother now uses lists to remember routine events and appointments. The daughter also reports that the cognitive impairment has been slowly progressive over the course of the last two years. Because this additional history provided evidence of functional decline and a progressive course, the presentation now meets the diagnostic criteria for Alzheimer’s disease (see Table 14).
What if the history of the preceding case had suggested a relatively distinct and abrupt onset of memory changes, noticeable fluctuations in memory from one day or week to the next, and some lateralized weakness? A history of this type raises a possibility that cognitive change may be due to vascular dementia or that vascular factors may be a contributing factor. The diagnostic criteria for vascular dementias include the DSM-4 criteria for dementia and the presence of focal neurological signs or symptoms or evidence of cerebrovascular disease judged to be related to dementia (see Table 15). A typical case of vascular dementia might include a history of neurological symptoms and abrupt onset of cognitive impairment.
The modified Hachinski scale is sometimes used by clinicians to assess the probability of a vascular dementia (see Table 16). A score of ≥ 4 suggests cerebrovascular contribution to dementing illnesses. Note that the most heavily weighted items are all associated with stroke.
Persons with vascular dementia may benefit from treatment with acetylcholinesterase inhibitors, as well as from the usual recommendations for reducing risk of stroke. Case #3: Frontotemporal Lobe DementiaCase #3 is used to illustrate the importance of a history that is atypical for AD. Case #3 is that of a 63 year old man who is married with 13 years of education. He has worked as a farmer all of his life and over the last 4 years, has experienced a progressive decline in short-term memory and abnormal weight gain. He has gained over 60 pounds in the preceding year. His wife notes that he can eat enormous amounts of food at one sitting and that this is new. In addition to changes in eating habits, his wife also notes personality changes. He has become increasingly argumentative and anxious with paranoid ideations. He has become very rude in public, and they no longer go to restaurants for meals because of his rude comments to the waiters or waitresses. She notes he has also become very repetitious and has gotten lost while driving. He recently had an accident and overturned his tractor which has never happened before. Atypical features include age of onset <65 and prominent behavior symptoms early in the course of the disease.
The results of the cognitive screen included a normal MMSE and an abnormal
Animal Naming and Clock Draw (see Table 17).
The cognistat showed moderate-to-severe
impairment in memory. because of atypical features, an MRI was done and showed
bilateral temporal lobe atrophy consistent with frontotemporal lobe dementia
(see
Figure
13).
Figure 13
The core features of frontotemporal lobe dementia are listed in Table 18. Like AD, the onset is insidious and the disease is gradually progressive. Unlike AD, frontotemporal lobe dementia tends to occur in younger persons, most commonly <65 years of age, and is associated with early changes in personal conduct, loss of insight and emotional blunting.
Features that are consistent with frontotemporal lobe dementia include the presence of behavioral disorders including changes in hygiene, grooming, dietary habits, rigidity and repetitive behavior. The second pattern of supportive features for frontotemporal lobe dementia include changes in speech and language and include perseveration, gradual loss of ability to carry on conversations and progressive economy of speech. Physical signs that can be present include akinesis, rigidity, tremor and labile hypertension. There is no effective treatment for frontotemporal lobe dementia. However, increasing structure in the person’s living environment and daily routine and educating caregivers in basic behavior management strategies can help in managing symptoms. Case #4: Lewy Body DementiaCase #4 is used to illustrate the significance of Parkinsonian symptoms in persons with dementia. Case #4 is a 79 year old man with 12 years of education. He worked as a farmer and is now widowed and living alone. He presents with a 2-year history of memory complaints and reduced ability to complete complex tasks. Importantly, he also has noted the presence of visual hallucinations. His family reports falls, occasional syncopal episodes, problems with balance and dexterity and getting in and out of chairs. He also complains of episodic daytime sleepiness and reduced alertness. He admits to being mildly depressed.Neurologic exam shows signs of mild Parkinson’s including rigidity, balance problems, mild cog wheeling in upper extremities, but no tremor. The MMSE was normal, the Animal Naming was abnormal (see Table 19).
The Clock Draw shown here is definitely abnormal (see Figure 14).
Figure 14
The Cognistat documents deficits in multiple areas (see Figure 15).
Figure 15
The history and cognitive findings are consistent with dementia with Lewy bodies.
Central features include progressive cognitive impairment leading to functional
impairment. Unlike AD, prominent memory impairment may not occur until later
stages. Two of the following core features are required for the diagnosis
of probable Lewy body dementia: fluctuating cognitive deficits, visual hallucinations
and Parkinsonian features especially gait abnormalities and falls.
Supportive features for dementia with Lewy bodies include repeated falls, syncopal
episodes, transient loss of consciousness, neuroleptic sensitivity and systematized
delusions.
Treatment with acetylcholinesterase inhibitors is the treatment of choice.
Persons with Lewy body have only a modest response to L-dopa. Anti-psychotics
should be avoided in persons with Lewy body dementia because of their neuroleptic
sensitivity. SummaryA recent survey by Metropolitan Life Foundation found that AD was one of the most dreaded diagnoses in medical practice. Nevertheless, studies show that most patients want to be told if they have a dementia. Many patients are unable to understand the diagnosis of dementia and may not be aware that they have a problem. It is important to understand that families and patients require time to accept and adjust to the diagnosis. There are multiple ways of coping with the diagnosis and the disease, but the strategies always involve both patient and family. Checking over time to see how families are implementing recommendations and coping with the illness is crucial because there are many opportunities for misinterpretation as exemplified by this story told by a physician from a local memory diagnostic clinic. A patient with significant and progressive cognitive deficits at his initial diagnostic assessment had been cautioned not to drive, and the need for this had been explained to his family. When seen six months later for follow-up, the doctor inquired to make sure he was no longer driving and was told “Oh, yes, we won’t let him drive the Cadillac now…only the pickup!”
Post-Test and Evaluation |
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File last updated: November
17, 2006
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