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Etiology of Dementia Dementia refers to a syndrome of global or diffuse brain dysfunction characterized by a gradual, progressive, chronic deterioration of intellectual

function. The persistent and stable nature of the impairment distinguishes it from the altered levels of consciousness and fluctuating deficits of delirium. Although dementia has many causes, the majority of cases (up to 75%) are of two main types: dementia of the Alzheimer's type and vascular dementia (Peskind & Raskind, 1996; Sadock & Sadock, 2003). Etiology of Dementia of the Alzheimer's Type (DAT) The search for the causes and treatment of dementia of the Alzheimer's type (DAT) continues. Several theories exist. A complete discussion of these theories is beyond the scope of this chapter. However, current theories regarding the causes of dementia are cited below and include:

The genetic theory proposing a genetic link to DAT focuses on three genes on three separate chromosomes (1, 14, 21). The immune system theory suggesting that DAT is the result of immune system malfunctions. The oxidation theory stating that the buildup of damage from oxidative processes in neurons results in the loss of various body functions. The virus and bacteria theory proposing that DAT may be due to a viral- or bacterialinduced condition secondary to the breakdown of the immune system (eg, herpesvirus). The nutritional theory postulating that poor nutrition and lack of mental stimulation during childhood may predispose one to DAT later in life. P.358

The metal deposit theory speculating that an accumulation of aluminum ions replacing iron ions may contribute to existing dementia. The neurotransmitter theory hypothesizing that DAT is due to a decrease in acetylcholine, dopamine, norepinephrine, or serotonin levels, limiting neuronal activity. A second theory postulates that excessive stimulation of glutamate damages neurons. The membrane phospholipid metabolism theory proposing that DAT is due to an abnormality in metabolism that causes neuronal cell membranes to be less fluid or more rigid than normal. The beta-amyloid protein theory postulating that symptoms of DAT are the result of neuronal degeneration due to the neurotoxic properties of this protein (Peskind & Raskind, 1996; Medina, 2001; Sadock & Sadock, 2003).

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Researchers continue to work diligently to determine the cause of DAT and proposed treatments. Neuroimaging has been used in the diagnostic evaluation of clients with memory and cognitive

impairment. The predominant finding of bilateral posterior temporal and parietal perfusion defects is thought to be highly predictive of DAT. Computed tomography (CT), positron emission tomography (PET), and single photon emission computed tomography (SPECT) scans show atrophy and lowered blood flow and energy consumption in the brains of clients with DAT. Deterioration appears first in the superior parietal cortex, the temporal lobes, and the hippocampus. In late stages, atrophy is severe (Figure 24.3). At present, brain imaging can distinguish early DAT fairly well from depression but not always from other brain diseases. Microscopic findings (postmortem) show senile plaques, neurofibrillary tangles, neuronal loss, synaptic loss, and granulovascular degeneration of neurons (Holman & Devous, 1992; Sadock & Sadock, 2003). Etiology of Vascular Dementia Vascular dementia is thought to result from infarction of small- and medium-sized cerebral vessels causing P.359 parenchymal lesions to occur over wide areas of the brain. Plaques or thromboemboli from distant organs such as heart valves are presumed to be the cause of the infarction. Binswanger's disease is a type of vascular dementia that is characterized by the presence of many small infarctions affecting the white matter of the brain that spare the cortical regions (Peskind & Raskind, 1996; Sadock & Sadock, 2003).
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Etiology of Diseases Associated With Dementia Several diseases are often associated with dementia (APA, 2000; Busse & Blazer, 1996; Sadock & Sadock, 2003). They include:

Familial multiple system taupathy (eg, a buildup of tau protein in the neurons and glial cells) occurring in individuals in their forties or fifties; thought to be carried on chromosome 17 and shares some brain abnormalities with DAT; often referred to as presenile dementia. Lewy body disease resulting from neurohistologic changes in the brain stem and widespread throughout the cerebral cortex; clinically similar to DAT, presenting with some features of Parkinson's disease. Pick's disease, progressive disorder of middle and late life characterized by atrophy and microscopic changes of the frontotemporal regions; difficult to differentiate from DAT. Parkinson's disease due to the presence of neurohistologic lesions in the basal ganglia; associated impairment of cognitive abilities; commonly associated with dementia.

mentia Dementia is characterized by impaired judgment, orientation, memory, cognition, and attention, which are affected either by a pattern of simple, gradual deterioration or by rapid, complicated

deterioration. Impaired judgment, or the inability to make reasonable decisions, is one of the earliest signs of dementia. It may occur during business dealings or social functions; for example, the person engages in a reckless business venture or displays a disregard for conventional rules of social conduct. Disorientation to person, place, and time is one of the most common signs of brain dysfunction. An individual becomes more disoriented as the impairment becomes more extensive. A person with minimal impairment may misjudge the date by weeks or months. Moderate impairment generally involves confusion about geographic location such as city or state as well as time, whereas severe impairment is demonstrated by disorientation P.362 with respect to time, place, and person. Short-term memory, attention, and concentration deficits are observable in this disorder because the person loses his or her train of thought, forgets what was said just a few minutes earlier, and may be unable to repeat the information just communicated (Peskind & Raskind, 1996; Sadock & Sadock, 2003). Other characteristics or associated features include confabulation, perseveration, concrete thinking, and emotional lability. Confabulation is the filling in of memory gaps with false but sometimes plausible content to conceal the memory deficit. Perseveration is the inappropriate continuation or repetition of a behavior such as giving the same details over and over even when told one is doing so. Abstraction skills are impaired; therefore, the person tends to think in concrete terms. The tendency to manifest rapid, inappropriate, exaggerated mood swings often occurs, and marked anxiety or depression may be seen in mild cases. Personality changes are often seen in clients with dementia. The normally active person may become withdrawn and apathetic when social involvement narrows. Psychotic or behavioral disturbances such as agitation, wandering, hallucinations, delusions, suspiciousness, reversal of sleepwake pattern, inappropriate sexual behavior, hostility, aggressiveness, and combativeness may occur. Clients with dementia often seem to exhibit increased confusion, restlessness, agitation, wandering, or combative behavior in the late afternoon and evening hours. This phenomenon, referred to as the sundown syndrome, may be due to a misinterpretation of the environment, lower tolerance for stress at the end of the day, or overstimulation due to increased environmental activity later in the day. Clients may also exhibit a reversal in their sleep pattern, sleeping during the day and staying awake during the night (APA, 2000; Peskind & Raskind, 1996; Sadock & Sadock, 2003). The DSM-IV-TR lists 12 subtypes of dementia:

Dementia of the Alzheimer's type Vascular dementia Dementia due to human immunodeficiency virus (HIV) disease Dementia due to head trauma Dementia due to Parkinson's disease Dementia due to Huntington's disease Dementia due to Pick's disease Dementia due to Creutzfeldt-Jakob disease Dementia due to other general medical conditions Substance-induced persisting dementia

Dementia due to multiple etiologies Dementia, not otherwise specified

The reported prevalence of dementia varies among epidemiologic studies. However, most studies have shown that the prevalence of dementia increases with age. Clinicians now estimate that about 50% to 60% of persons with dementia aged 65 years and older have pure AD and about 15% to 30% have pure vascular dementia. An overlap between these two dementias occurs in approximately 10% to 15% of clients. Between 5% and 10% of clients with dementia suffer from diffuse Lewy body disease, and the remaining 10% of dementia cases represent progressive supranuclear palsy, Parkinson's dementia, and other less common diagnoses (Geldmacher, 1996; Sadock and Sadock, 2003). Clinical symptoms of DAT and vascular dementia are discussed because these two disorders constitute the majority of all dementias. Definitions of Parkinson's disease, Huntington's disease, Pick's disease, and Creutzfeldt-Jakob disease appear in the glossary in the back-of-book CD. Dementia of the Alzheimer's Type (DAT) Alzheimer's disease is considered the fourth most common cause of death for people older than 65 years of age in the United States. It is not a natural course of aging. Rather, it is a silent epidemic characterized by the development of multiple cognitive deficits including memory impairment, aphasia (language disturbance), apraxia (impaired ability to carry out motor activities despite motor function), agnosia (failure to recognize or identify objects despite intact sensory function), and disturbances in executive functioning (eg, planning, organizing). Figure 24.4 depicts some of the pathophysiologic changes that are believed to occur with DAT. The course is characterized by gradual onset. The client is aware of the loss of mental abilities as they occur. The diagnosis is coded or labeled based on when and what symptoms appear. If clinical symptoms appear before age 65 years, the diagnosis is coded as DAT with early onset; after age 65, the coding with late onset is used. Additional coding indicates with delirium, with delusions, with depressed mood, or uncomplicated. The DSM-IV-TR also may include the phrase specify if. This specifier enables the clinician to select additional information from the criteria, such as with behavioral disturbance, during the collection of data. Although the specifier is not coded, it can be used to indicate clinically significant behavior such as wandering, throwing items, or combativeness (APA, 2000). As the dementia progresses, personality changes, paranoia, P.363 stooping gait, loss of voluntary functions, seizures, and violent behavior may occur. Death can result from neglect, malnutrition, dehydration, incorrect diagnosis, inappropriate treatment, or suicide.

FIGURE 24.4 Pathophysiologic changes associated with DAT: (A) granulovascular degeneration; (B) neurofibrillary tangles; (C) amyloid plaques as physical changes in the cortex. Risk factors associated with the occurrence of AD include advanced age, female gender, head trauma, low educational level, and family history of Down syndrome (Cummings, 1995). Stages of Dementia of the Alzheimer's Type. Attempts to describe the progression of DAT have resulted in two frequently used classification systems. The first system groups clinical symptoms into three progressive stages described as mild, moderate, and severe in nature (Williams, 1986; Blazer, 1996; Cummings, 2001). The second system describes seven stages of AD according to functional consequences (Reisberg, 1986). For example, stage 3 lists functional manifestations of normal aging such as forgetting names and the location of objects and exhibiting decreased ability to recall appointments. In comparison, stage 7 lists functional manifestations such as progressive loss of all verbal and psychomotor abilities, at which point the individual eventually requires total assistance in all activities. See Clinical Example 24-2, The Client With Dementia of the Alzheimer's Type, Stage 6. Knowledge of both classification systems is important when providing care for individuals with the diagnosis of AD. Vascular Dementia Vascular dementia, formerly known as multi-infarct dementia, is the second most common cause of dementia after DAT. The disorder is more common in males than in females. The onset of vascular dementia is usually earlier than that of DAT. Onset is generally abrupt with fluctuating, rapid changes in memory and other cognitive P.364

impairment. Apathy, unsteady gait, weakness, dizziness, and sensory loss generally occur. Clients with vascular dementia often exhibit the same clinical symptoms seen in DAT: aphasia, apraxia, agnosia, and disturbances in executive functioning. Medical conditions associated with the development of vascular dementia include long-standing arterial hypertension, valvular heart disease, cerebrovascular disease, and extracellular vascular disease that may precipitate cerebral emboli. Risk factors also include diabetes mellitus, cardiac arrhythmias, smoking, hypercholesterolemia, and genetics (APA, 2000; Peskind & Raskind, 1996; Sadock & Sadock, 2003). CLINICAL EXAMPLE 24.2 The Client With Dementia of the Alzheimer's Type, Stage 6 MM, an 80-year-old female client, was admitted to the special care unit of a long-term care facility because her family is no longer able to meet her needs. During the assessment process, MM scored 8 of 30 points on the Mini-Mental State Exam. Deficits were noted in the area of orientation, recall, inability to spell world backwards, inability to write a complete sentence, and the inability to copy a diagram. MM also had difficulty performing activities of daily living while residing at home. She had become incontinent of bowel and bladder and was resistant to care provided by her family. MM had wandered outside at night and was returned home by the police. During the interview process, MM used confabulation during responses to questions about her husband, family, and past employment as an executive secretary. After the completion of a dementia workup, the diagnosis of Dementia of the Alzheimer's Type, Late Onset, Stage 6, was noted. Vascular dementia may be classified into subtypes due to the presence of large vessel disease, lacunar infarct, strategic infarct, microvascular disease such as Binswanger's disease, small vessel disease, and hypoxic conditions. The DSM-IV-TR diagnosis of vascular dementia is used when focal neurologic signs and symptoms or laboratory evidence indicative of cerebrovascular disease are judged to be etiologically related to the disturbance. The focal neurologic signs and symptoms include exaggeration of deep tendon reflexes, extensor plantar response, pseudobulbar palsy, gait abnormalities, or weakness of an extremity. Computed tomography scan usually reveals multiple infarcts involving cortex and underlying white matter. Anxiety, depression, delirium, delusions, socially inappropriate behavior, lack of inhibition, and increased agitation related to environmental stimulation may occur (APA, 2000). See Clinical Example 24-3. CLINICAL EXAMPLE 24.3 The Client With Vascular Dementia BW, a 49-year-old male, was admitted to the subacute unit of a rehabilitation center. Upon admission, he presented with expressive aphasia, left-sided hemiparesis, and had an indwelling catheter in place. His wife of 20 years accompanied him. She provided information regarding his recent hospitalization and current plan of care. According to the client's wife, BW had experienced his first stroke at the age of 48 years. His recovery was uneventful; however, she noted that BW had difficulty expressing himself and would become frustrated because he was unable to recall previously learned information. At that time, he also exhibited apraxia, the inability to carry out motor activities despite intact motor function. Approximately 8 months ago, BW had another stroke that left him with his current symptoms. During his course of rehabilitation, BW was able to speak but continued to exhibit some expressive aphasia. He was oriented to person and knew that he was not at home, but could not state where he was. He was

unable to state the date. Prior to discharge, a follow-up neurologic evaluation was conducted. BW was able to complete a modified Mini-Mental State Exam. The results indicated the presence of vascular dementia. Dementia Due to Other General Medical Conditions This classification is used to diagnose dementia due to general medical conditions (eg, HIV, traumatic brain injury, and Parkinson's disease); endocrine, nutritional, and infectious conditions; structural lesions of the brain; and renal or hepatic dysfunction. Identifying the specific type of dementia is important because treatment guidelines have been developed for the different types of dementia. For example, persons with Lewy body dementia may be misdiagnosed as having DAT. Often individuals with Lewy body dementia have extreme adverse reactions (eg, stiff, rigid movements or immobility) to drugs commonly used to treat behavioral problems associated with dementia. These adverse reactions can be serious and could jeopardize the person's health.