Alzheimer’s Disease (Dementia of Alzheimer’s Type or DAT), vascular dementia (multi-infarct dementia or MID), Dementia with Lewy Bodies (LBD), frontotemporal dementia (FTD) , and dementia due to HIV/AIDS are the most commonly seen types of dementia. Dementia can be categorized as cortical or subcortical, with DAT being a classic example of a cortical dementia. Cortical dementias are typically characterized by amnesia, disorientation, and relatively preserved personality. Whereas subcortical dementias show relatively preserved memory with difficulties in executive function, attention, and concentration. Dementia associated with Parkinson’s disease is an example of subcortical dementia. It is important to remember that with increasing age persons more frequently suffer from more than one type of dementia, and this is referred to as mixed dementia [6].

DAT is the most common cause of dementia and is estimated to account for 55–75 % of all cases [1, 3, 7] and classified as a neurocognitive disorder (NCD) due to Alzheimer’s Disease in DSM-5 [8]. In DAT, personality and attention are preserved in the early stages of disease as opposed to other types of dementia where a more rapid deterioration of personality may occur. Executive functioning declines progressively over time in DAT, but progression of behavioral symptoms is the most common reason for admission to a memory care unit or nursing facility.

The second most common etiology for dementia is vascular disease (Vascular NCD in DSM-5) and accounts for 13–16 % of affected individuals [1, 3, 7]. Vascular or multi-infarct dementia (MID) is characterized by early onset of decreased attention accompanied by blunting of affect and memory disturbances. Patients usually have known risk factors for vascular disease (e.g., hypertension, diabetes mellitus, hyperlipidemia) and behavioral risk factors (e.g., smoking, obesity, sedentary lifestyle). Vascular dementia frequently progresses in a stepwise fashion as opposed to the gradual progression seen in DAT. Microcirculatory deficits accumulate over time as a result of the vasculopathic nature of the underlying disease process. On physical exam evidence of arterial vascular compromise is commonly seen as well as focal neurologic abnormalities.

Lewy Body Disease (LBD) is the third most common dementia seen and accounts for 20–25 % of all dementia, LBD NCD in DSM-5. LBD usually occurs at an earlier age and has a faster progression than DAT and is associated with psychiatric symptoms, fluctuations in level of alertness, and an increased sensitivity to antipsychotic medications [9]. Unfortunately psychiatric symptoms are very common and include anxiety, depression, and perceptual disturbances. Perceptual disturbances are characterized by hallucinations that are usually visual and delusions or fixed false beliefs that are frequently paranoid. LBD is closely associated with Parkinson’s disease and is characterized by many of the features of the Parkinson’s disease. These features include a motor syndrome (bradykinesia, rigidity, tremors, and gait difficulties leading to falls) and autonomic dysfunction (constipation and orthostatic hypotension leading to syncope). Loss of smell and sleep disturbances are frequently seen as well. Sleep disturbances include excessive daytime drowsiness and nighttime difficulty not only staying asleep but also REM sleep behavior disorder (RBD) . RBD manifests as the patient acting out his or her dreams, a symptom often first recognized by a caregiver. Persons diagnosed with Lewy body dementia are also sensitive to antiparkinsonian drugs that can increase confusion and problematic behaviors. Therefore when prescribing these medications, it is important to weigh the risk of worsening cognition versus the potential benefit of improving function.

Some dementias are rapidly progressive such as Creutzfeldt–Jakob disease (Prion disease NCD) which is caused by a prion or infectious protein. Symptoms of this dementia develop quickly often accompanied by myoclonic jerks and eventually seizures [10]. The progression of symptoms is less obvious in frontotemporal lobe dementias (FTDs), classified as frontotemporal lobar degeneration NCD in DSM-5. FTD manifests as mood swings and impulsivity, with personality coarsening as well as a concomitant deterioration in functional status. Affected individuals also demonstrate inattention and affective flattening. A more unusual cause of dementia is Huntington’s disease , which is inherited and begins at a very young age (e.g., 35–45). Huntington’s disease causes subtle mood changes and cognitive problems that progress fairly rapidly to dementia with behavioral disturbances, which are accompanied by a lack of coordination and chorea (i.e., involuntary writhing movements).

Other etiologies of dementia to consider in the differential diagnosis include HIV disease, Parkinson’s disease (where the Parkinson’s motor symptoms will precede cognitive decline by several years), Wilson’s disease, traumatic brain injury (i.e., from falling in older adults), or other neurological conditions (e.g., ALS, multiple sclerosis, Korsakoff’s syndrome /alcoholic encephalopathy). Medical conditions that can cause cognitive dysfunction include obstructive sleep apnea, metastatic disease, neurosyphilis, and substance abuse. The possibility of substance abuse or prescription medications influencing cognition should always be considered in anyone presenting with memory complaints, even if the medication had been taken for many years [8]. Remember to look for the more common potentially reversible causes of dementia: B₁₂ deficiency, thyroid disease, subdural hematoma, normal pressure hydrocephalus, and primary tumors of the brain.

When the staff in the PA/LTC facility is trained and experienced in caring for persons with dementia, those residents who develop early symptoms of dementia are more readily recognized. In nursing facilities (NFs) MDS evaluations can be compared to determine changes in resident cognition, memory, and other manifestations such as:

The diagnosis of dementia starts with a patient history, often obtained from a family member and/or staff in PA/LTC. It is important to determine if the onset of the condition was insidious and difficult to pinpoint in time or if the progression was gradual or stepwise (i.e., DAT vs. MID). Any specific neurologic and psychiatric symptoms that have occurred also need to be considered [3]. The diagnosis can then be refined on the basis of the neurologic signs and symptoms, mental status, physical examination and the results of neuropsychologic testing (though often not needed), brain imaging, and laboratory studies.

It is important to thoroughly evaluate a resident with suspected dementia, as many medical conditions can cause or worsen dementia, some of which can be reversed (See Table 3). In particular, a patient presenting with new symptoms of memory or cognitive impairment should be evaluated for depression as well. Depression can cause a form of cognitive impairment referred to as pseudodementia (for further discussion see section on depression).

The “gold standard” for the diagnosis of dementia is clinical, based upon the patient history and physical assessment, with ancillary information such as formal neuropsychologic testing [11] and brain imaging performed as needed. A basic evaluation of the resident with new cognitive impairment includes laboratory tests such as a complete blood count with differential, comprehensive metabolic panel, vitamin B₁₂ level, thyroid function panel including a TSH, and possibly blood levels of medication such as digoxin, lithium, theophylline, anticonvulsants (e.g., phenytoin, valproic acid), and tricyclic antidepressants (e.g., amitriptyline). Other tests to consider are: HIV, RPR or perhaps a test for Lyme disease (depending on local prevalence and other risk factors) as possible causes for a change in cognition [3, 4].

It is also necessary to evaluate underlying medical conditions and to optimize their management. Medical conditions commonly implicated in cognitive dysfunction include: recent coronary artery bypass grafting, hypertension, nutritional deficiencies including B vitamins, diabetes mellitus, stroke, Parkinson’s disease, and diseases that cause hypoxia such as COPD and obstructive sleep apnea [4].

Quantifiable scales that may be used as benchmarks for diagnosing and monitoring dementia progression include the Montreal Cognitive Assessment (MOCA) , the Folstein Mini Mental Status Exam (MMSE) , the St. Louis University Mental Status Exam (SLUMS) or other commercially available instruments [11, 12]. The MMSE is a simple 30-point screening tool frequently used to evaluate for cognitive impairment. Recently, the official form has been copyrighted and must be purchased (www.​minimental.​com.) Because of this expense, some practitioners have chosen instead to use the SLUMS available at http://​medschool.​slu.​edu/​agingsuccessfull​y/​pdfsurveys/​slumsexam_​05.​pdf. More tools can be found in “Other Resources” at the end of this chapter. Keep in mind that the MDS in skilled nursing facilities will also provide an evaluation of the resident’s cognitive status (Brief Interview for Mental Status or BIMS) and functional status.

Finally, neuro-imaging of the brain may be performed to rule out structural lesions such as a neoplasm or other potentially reversible conditions such as normal pressure hydrocephalus or a subdural hematoma. A neuro-imaging study should be considered especially when the onset of the dementia has occurred within the past 6–12 months, is rapidly progressive, or is following an unpredictable course.

If there is a question of diagnosis, or if the resident or their family has difficulty accepting the diagnosis of dementia, neuropsychologic testing may be done to more definitively diagnose the resident’s cognitive status. Not only may neuropsychologic testing help determine the type of dementia, but it can also help to delineate the patient’s strengths and weaknesses; to identify particular areas of cognitive dysfunction in order to suggest compensation strategies, and to aid in behavioral management [5]. The diagnosis of dementia can aid in the care of the resident. Its diagnosis will provide a framework for prognostication and will aid in health care decision-making by both resident and family. Although dementia is a chronic, progressive, and ultimately terminal illness, it is often unrecognized in its early stages. The goals of care in the management of dementia are to provide a safe environment, educate caregivers and family, and provide emotional support for the healthcare team, patient, and family.

Different types of dementia have varied manifestations at onset, with the common pathway being one of progressive loss of ability to perform daily tasks of living (i.e., ADLs and IADLs). Residents eventually become unable to care for themselves, incontinent of bowel and bladder, and unable to safely swallow and to maintain sufficient nutrition to sustain life. Even when carefully hand fed, the resident with end stage dementia will eventually develop progressive weight loss and be at high risk for developing pressure ulcers. The demented resident may also develop urinary retention, constipation, and repeated urinary or respiratory infections (the latter due to aspiration of food and secretions) [5]. It is imperative that the family be educated on the disease process of dementia and its course. This may be difficult for families to accept, as many do not understand that dementia is a terminal illness (see Chaps. “Integrating Palliative Care into Practice” and “Weight and Nutrition” for further reading).

Pharmacologic treatment of dementia should focus on the underlying medical and psychological condition(s) affecting the individual, with the goal being maximization of his or her functional well-being. For example, in those with coexistent depression, the use of antidepressants with serotoninergic activity may improve both depressive symptoms and cognition [3].

Pacing, wandering, hoarding, agitation, insomnia, aggression, hypersexuality, perseveration, hallucinations, paranoia, and emotional labiality are challenging behaviors seen in dementia [4, 15]. Agitation frequently is seen with “sundowning ,” which is a syndrome of disorientation, confusion, and agitation that often starts in the middle to late afternoon and progressively worsens through the evening into the night. An environment and medical evaluation often gives insight into ways in which these behaviors can be mitigated or prevented. Frequently, these behaviors are a natural manifestation of the dementing process, but they may be exacerbated by stimuli in the environment or a medical illness. On one hand, commonly seen environmental causes of disruptive behavior include a new caregiver, an absent family member or another disruptive resident. On the other hand, commonly seen medical causes of disruptive or changed behaviors include pain, constipation, urinary retention, drug effect(s), dehydration, or infection. It is important to first evaluate any reversible environmental and/or physical stimuli that may be implicated in precipitating the behavior before initiating any treatment interventions for the behavior [4]. Communication becomes increasingly difficult for patients as their dementia progresses. Often what may be deemed as “agitation” or other abnormal behavior might be reformulated to questions of, “What is this behavior attempting to communicate?” “What are their unmet needs, if any?” “What is this behavior attempting to convey?” Whether it is pain or discomfort due to constipation or inability to urinate, boredom or hunger, attempting to identify and address the antecedents to these underlying behaviors should be a first-line intervention rather than prescribing a pharmacologic agent for “agitation.”

Psychosocial interventions constitute a mainstay for the creation and promotion of a sense of well-being for those with progressive dementia. Learning about and acknowledging individual resident preferences and personhood, as well as providing care with dignity can help establish a fulfilling and meaningful activity program. Physical activity, even if limited to the upper extremity for wheelchair-bound residents, may be a welcomed and enjoyable part of the day. Social activities such as listening to reading, singing, or reminiscing and reality orientating may bring solace and peace to patients, families, and staff. Respect for privacy, sleep and meal preferences, and effective verbal, visual, and tactile cueing may further benefit the resident with dementia and others at the end of life [16].

Behaviors in dementia may be addressed using non-pharmacologic and/or pharmacologic treatment.

The average nursing facility resident takes seven medications, and nearly a third of residents take nine or more medications [16]. Therefore, when evaluating cognitive impairment it is crucial to consider medications and medication interactions that can alter cognitive function. Medications that are commonly implicated include antiarrhythmics, hypnotics, psychotropics, sedatives, analgesics, and medications with significant anticholinergic properties such as those used for urinary urge incontinence. Toxicity from certain medications such as digoxin can also cause changes in cognition. In 1991, Dr. Mark Beers created a list of potentially inappropriate medications used in the geriatric population. The Beers list continues to be updated and is used for guidance by practitioners in PA/LTC and by state surveyors (See Chap. “Medication Management in Long-term Care for further discussion”) [13].