case study 2 for neurocognitive disorders alice

Module 14: Neurocognitive Disorders

Case studies: neurocognitive disorders, learning objectives.

• Identify neurological disorders in case studies

Case Study: Sarah

Sarah is a 78-year-old female and is very outspoken. Sarah has been an actor in off Broadway shows as well as working as a consultant in the education industry for 30 years prior to retirement. Sarah and her family members over the past year have noticed that she is not remembering things as well. Sarah even admitted to her husband that she is more and more having forgetful moments over the past two years. Sarah’s mother and aunt were diagnosed with a neurocognitive disorder several years before they passed away. Sarah agreed to go to the doctor and was a bit worried about the biological impact of her mother’s disorder, but kept an open mind. Sarah went to the doctor and discussed what was going on, and the doctor referred Sarah to specialists who focus on memory. Sarah was asked if she partook in any substances, which she said she occasionally has wine to drink to unwind some evenings, but nothing problematic. Sarah was administered several memory tasks and the doctor said the results were OK, as she remembered two out of four items and said that he wanted to see her again in three months.

Sarah went back in three months and there was no change, but at six months she was only able to remember one out of four items on a memory task and he suggested starting treatment. Sarah was administered an acetylcholine agent that could help limit memory loss for a period of time. Over the next three years, Sarah remained with mild cognitive loss, but after three years on the medication, the effectiveness was not showing, unfortunately. Sarah was then told that she needed permanent care as her memory was progressing at a negative rate. Sarah was at home for another three years, but then was unable take care of herself and was put in a nursing home facility.

Figure 1 . Gina has seen a decline in her desire to participate in her usual activities alongside a decline in cognitive abilities.

Case Study: Gina

Gina is 76 years old and went to her doctor for a regular physical as she did each year. Gina told the physician that she recently has socially isolated herself and has not felt comfortable visiting and spending time with family. Gina also was having hallucinations and found that she had symptoms that were consistent with Parkinson’s disease, but was not sure. Gina also told the physician that she seems to forget things a great deal more than she used to and wanted to find out why. Gina also discussed with her doctor that her alertness and attention varied quite heavily. Gina said her family members have said that they noticed a 20–25% decrease in her cognition over the past six months. Gina discussed as well that she is not as active as she was six months ago, and sometimes she does not have the energy to go outside and go for a walk as she has done in the past. Gina took part of a memory task at the physician and was only unable to recall one out of four items that the doctor presented to remember. Gina’s doctor suggested that she receive an opinion from a specialist and referred her to them.

Think it Over

What are the treatment options that could be part of the process in helping Gina and why? Also, if you were the physician, which specialist or specialists would you refer Gina to and why? What tests/exams should Gina have in relation to further diagnosis?

Also, in Sarah’s case, do you feel going to the doctor helped her cause in relation to memory loss and if so why? What treatment would you focus on for Sarah and why?

• Sitting alone. Authored by : Arek Socha. Located at : https://pixabay.com/photos/woman-senior-citizen-elderly-old-3213761/ . License : Other . License Terms : Pixabay License

MINI REVIEW article

Does alice live here anymore autonomy and identity in persons living and dying with dementia.

• Geriatric Psychiatry Unit, Department of Psychiatry, National Institute of Mental Health and Neuro Sciences, Bangalore, India

Conventional scientific definitions of dementia, or its newer proposed alternate—neurocognitive disorders place emphasis upon cognitive function, particularly memory. The changes in thought, emotion, behavior, personality, and biological function are usually considered only of secondary importance. At the core of the illness, however, lies a progressive loss of self, and by extension, of personhood, identity, autonomy, and agency. The identity of the person living with dementia, and the deterioration of a sense of self assumes significance in planning end of life care, including palliative care. A consideration of self and identity is also significant where physician assisted death, incorporating euthanasia, has legal sanctity. As dementia progresses, there is usually a progressive loss of personal decision making capacity and legal competence. Shared decision making, advance care directives and proxy representatives are options available to safeguard autonomy and agency in such cases. Advance care directives are often treated as static documents. The loss of self and deterioration of identity in persons with dementia means, that there is a psychological discontinuity across time and space, though biological continuity is retained. The discontinuity in self and identity however, imply that the person with dementia changes considerably and so too may values and beliefs. A document which best reflected the wishes of the person with dementia in the past, may not always do so now. Advance directives and proxy representatives may need to be dynamic and evolve over time, particularly where end of life care and physician assisted death is being invoked.

Background—Dementia and Identity

Dementia has been classically defined, by Jeffrey L. Cummings, in 1992, is as an acquired syndrome of intellectual impairment produced by brain dysfunction, usually organic. ( 1 ) A more elaborate, alternative definition of the condition, by the ICD-10 Classification of Mental and Behavioral Disorders (ICD-10), also offered in 1992, is that of a syndrome due to disease of the brain, usually of a chronic or progressive nature, in which there is disturbance of multiple higher cortical functions, including memory, thinking, orientation, comprehension, calculation, learning capacity, language and judgement ( 2 ).

These definitions, while useful in the clinical, academic and research context, however, run the risk of a narrow and excessively cognitive function based view of the omnibus of diseases that compose dementing illnesses. ( 3 ) Further, the word dementia, in common and general public usage, is often understood as a loss of one specific cognitive function—memory. It is thus considered synonymous by implication, and often indistinguishable from amnesia or a loss of memory. Indeed the very term, dementia, derives from the Latin root word demens , implying mad, raving, or out of one's mind. ( 4 , 5 ) The newer term—neurocognitive disorders, proposed by the Diagnostic and Statistical Manual of Mental Disorders, in its 5th edition (DSM-5) in 2013, while broadening the scope of the definition with emphasis upon cognitive functions other than memory, continues to limit itself primarily to cognition ( 6 ).

A criticism of this approach to dementia, articulated by Lawrence Cohen, among others, is that it fails to highlight adequately the changes in perception, thought, emotion, behavior, and biological functions that accompany cognitive impairment in persons with dementia. ( 7 ) These are considered of secondary importance and lumped together under the rubric of the behavioral and psychological symptoms associated with dementia (BPSD). Yet, the BPSD occur in 50% of persons diagnosed with dementia within the first year of the illness and affect up to 95% of persons diagnosed with dementia within the fifth year of the illness. ( 1 ) The BPSD are also the most important predictor of quality of life in the person living with dementia, of caregiver burden, and of placement in nursing homes or other long term residential care facilities—in turn a proxy predictor of survival in persons with dementia ( 8 ).

Even further removed from the conceptualization of dementia, is an understanding of the personhood, identity and autonomy of the person living with dementia. ( 7 ) Despite the incorporation of the four principles of medical ethics–beneficence, non-maleficence, respect for autonomy and justice into clinical practice and research; and the growing emphasis on patient centered rather than disease centered management; an appreciation of the identity of person with dementia is often lacking. ( 9 – 11 ) The person, so to speak, recedes behind the disease. However, it is this erosion of personhood, identity, and by extension, autonomy, that persons living with or at risk dementia and their caregivers most often express fear of. As noted by Bianca Brijnath, and Cohen, the person with dementia experiences death twice, once in the loss of identity; and then again at the end of life ( 7 , 12 ).

Identity, End of Life Care and Physician Assisted Death

The question of personhood, identity and autonomy, as well as of death, and the multiple forms it takes in persons living with dementia and their caregivers becomes even more relevant in a discussion of patient rights. ( 13 ) This includes, but is not limited to the right to live vs. right to die discourse often invoked in dementia ( 14 ).

The deterioration in cognition, emotion, behavior and functioning that occurs, in what is noted by the ICD-10 as a syndrome, usually of a chronic or progressive nature, is associated with an erosion of personhood and identity, and by extension, autonomy and competence in decision making. ( 15 ) The loss of identity—of a shared self, is recognized by some sociologists, including Brijnath and Cohen as a living death, or the first of the two deaths the person with dementia and their family must undergo ( 7 , 12 ).

Thus, there is increasingly a need for shared (and assisted) decision making, advance directives (either care directive or a proxy directive or both). These provisions allow for and attempt to preserve patient autonomy for as long as is possible. ( 16 ) Yet, they often remain underutilized services that are not easily accessible to patients and families by virtue of incomplete penetration in the community, scarcity of opportunities and resources, and social inequity. ( 16 ) These services become increasingly relevant with the progression of dementia, loss of capacity and around the period of delivery of end of life care ( 13 ).

The National Cancer Institute (NCI), part of the National Institute of Health (NHI) defines end of life care as care given to people who are near the end of life and have stopped treatment to cure or control their disease. This includes physical, emotional, social, and spiritual support for patients and their families. ( 17 ) The end of life care is broadly divided into the following three, overlapping categories:

1. Palliative Care

2. Supportive Care

3. Hospice Care.

End of life care, including palliative care is therefore most often performed by physicians and care teams to the corpus of the person with dementia, based upon what is considered to be best practice and therefore in the best interests of the person. The caregiver, formal or informal, often has varying degrees of influence in the decision making process; depending upon the clinical context, level of risk associated with the decision or intervention, ethos of the dementia care team, legal provisions, and social (including individual and family) mores. Difficult decisions that are engaged with, though not often taken, during end of life care include prolongation of life, withdrawal of life support and assisted death ( 17 ).

Physician assisted death is defined by the Hastings Center as referring to the practice where a physician provides a potentially lethal medication to a terminally ill, suffering patient at their request they can take (or not) at a time of his own choosing to end his life. ( 18 ) The American Medical Association, in their Code of Medical Ethics Opinion 5.7 state that physician-assisted suicide occurs when a physician facilitates a patient's death by providing the necessary means and/or information to enable the patient to perform the life-ending act (e.g., the physician provides sleeping pills and information about the lethal dose, while aware that the patient may commit suicide). ( 19 ) The NCI goes on to define euthanasia (colloquially, though inaccurately known as mercy killing) as an easy or painless death, or the intentional ending of the life of a person suffering from an incurable or painful disease at his or her request. ( 20 ) Euthanasia by nature, as is widely known, can be active or passive ( 20 ).

The potential stakeholders in physician assisted death in persons with dementia may include:

1. Physicians

3. Mental health professionals

4. Case workers

5. Patients

6. Caregivers (formal and informal)

7. Legal Representatives

8. Political and Legislative Representatives

9. Advocacy Groups

10. Professional Bodies.

While all the various stakeholders (formal and informal) in physician assisted death in general, and physician assisted death in persons in dementia in particular, are divided on the ethicality, morality, and legality of the same, this had been covered reasonably well in literature ( 14 ). Of these stakeholders, however, the least autonomy (and associated decision making capacity) often rests with the person with dementia, sometimes contemporarous with late stage dementia and end of life care ( 11 ). The potential for erosion of autonomy may indeed be more so in dementia than other terminal illnesses due to the inevitable decline of decision making capacity with the progression of the disorder ( 15 ).

This article intends to examine the thesis of how questions about the personhood, identity and autonomy of the individual may impinge on physician assisted death in persons with dementia. The approach is meant to place the identity of the person with dementia at the center of the discussion rather than take sides in the ever evolving right to live vs. right to die debate.

At the core of personhood, identity, autonomy which play a role in guiding and shaping end of life care and the debate on physician assisted death is the self. The self can be loosely defined as an integration of the ideas of who we are, our existence in relation to other people, and a sense of worth and meaning derived from all of these ( 21 ).

At the core of an appreciation of the self in ourselves and in others, therefore, is the ability to realize the existence of self and delineate the self from the other or the environment. While the Cartesian duality of the mind and body may be artificial, an appreciation of the self is also associated with an anchoring of the self in the mind and the body. I am myself, and not this person or that. I occupy this body and mind .

This must be accompanied by the ability to localize the self in time and space, while paradoxically also maintaining a sense of continuity of the self over time and space. I am 60 years old and am currently standing at this place. I am not who I was at 20 or who I will be at 80. Yet, I am a person who has lived through and experienced being 20 and may live through and experience being 80. I am at my house and not outside it, yet if I leave my house and go outside, I will still be myself .

Further, this must be associated with a sense of perception, processing and praxis as the individual navigates their life in relation to others and the environment over time and space. I can smell sense smells coming from the garden, which I associate with flowers and grass, and which are pleasant. By this, I realize that spring is here and I shall go the window or to the door to drink in the smells and sights of the garden .

While the interaction with others and the environment occurs, there is also, constantly, a monitoring of and evaluation of the self. This appraisal of the self may be experienced in as a summing of strengths and weaknesses, of successes and failures, of mastery and pleasure, of what is in one's control and what is not, and of what one has achieved and of what is yet left undone. I have worked and rested. I have loved and hated. I have raised children and grandchildren. I have been happy and sad. I have fulfilled these of my responsibilities but not those. I have experienced these difficulties and those pleasures. I have done this and there is that yet to do .

Finally, there is a sense of meaning and of purpose, which emate from all the above layered appreciations of the self. My life served this purpose. I had this meaning to my journey. I had this meaning to my life and that meaning to my death. I will be remembered for a while and by some after I die. I may eventually fade from memory, as all people do .

These five concepts of the self are integrated into a whole, with a sense of oneness over time and space despite the changes accrued, and of ownership of the whole of the self. Personhood is a recognition that this self exists. Identity is a recognition of the contents of the self—of the whole and of its parts. Autonomy is governing over the self and the decisions that must be taken regarding the self. Agency stems from autonomy and is the ability to take action and experience outcomes based upon those decisions.

The Self in Dementia

As would be clear from the above discussion of the components of the self and of its relationship to personhood, identity, autonomy and agency—these are complex cognitive function based abilities. While a large part of such an appreciation of the self-occur reflexively and often outside awareness, these nevertheless require a continuous utilization of cognitive resources. These cognitive resources also track the emotions, behavior and biological functions that the self-experience and constantly process and respond to them ( 15 ).

Dementia, with its progressive deterioration of cognitive function, in most cases, depletes the cognitive resources required for the integration of ideas that constitute the self. The impairment in memory and other cognitive abilities—amnesia, agnosia, apraxia, aphasia, alexia, agraphia, acalcuila, and executive dysfunction—are therefore not just primary losses of specific abilities of the brain, in and of themselves. They also cause a secondary inability to appreciate or integrate the self. I used to be able to read the newspaper and remember the names of those in my family and purchase groceries. If I can no longer do this, whom am I?

Persons with dementia differ in the level of insight and in the presence or absence of anosognosia, though decision making capacity declines with the progression of the illness ( 22 ). There is usually, however, some degree of distress about the loss of abilities and the changes in emotion, behavior and biological functions. If I can no longer do this, what good am I to others? I was never this prone to losing my temper before, what has happened to me? Why do I now do this and not that?

Who is Alice? Does Alice Live Here Anymore?

The radical discontinuity over time and space that occurs in dementia then raises the question of whether the person now living with (and by extension dying with) dementia is the same person or someone else. Families and caregivers often articulate this when they report that they no longer recognize the person they are living with. While Brijnath and Cohen call this the first of the two deaths or the living death, Winston Chiong articulates this as the someone else problem, from the point of view of the other ( 13 ).

This postulation of living death has been contested. The slow decline in dementias, even when rapidly progressive, has no definite demarcations as are observed between life and death. It is therefore, unclear when exactly personhood undergoes sufficient erosion so as to constitute a loss of selves or a discontinuity in identity. Further, the living death is used in some countries, such as India, to indicate brain death. This paper uses the term in the common language sense (life emptied of joys and satisfactions in Merriam-Webster; an extremely poor quality of life, for example when someone is so ill that they are unlikely to recover in Collins) rather than in its narrower medical definition ( 23 , 24 ).

Alice is a woman in her seventies. Alice was once healthy. Alice now has dementia. Alice cannot now remember events that occurred in the past. Alice also cannot now form or retain new memories. Alice also finds it difficult to recognize people close to her or maintain her relationships with them. Alice once drove a car and balanced her check book. Alice cannot no longer do these activities. Alice had no fondness for sweets before developing dementia, but now has a marked sweet tooth. Alice can no longer regulate her bowel or bladder as she could do before .

Who then, is Alice? Is Alice then, still Alice now? Does Alice live in this body anymore?

The continuity of self and of identity over time and space has been conceptualized as a question of:

1. Psychological continuity or continuity of the mind, vs.

2. Biological continuity of continuity of the body

To this may be added

3. Social continuity or a continuity of roles and relationships in society

4. Ethical and moral continuity or a continuity of values and beliefs held by the person.

Rebecca Dressler argues that the psychological discontinuity (loss of self) that occurs in persons with dementia, despite the biological continuity (habitation of the same corpus) implies that the person who existed before the occurrence of dementia, is not the same person who now lives with dementia ( 25 ). An alternate viewpoint, by Ronald Dworkin, has been that although there is a loss of self and erosion of identity, since the person still inhabits the same body, they are to be considered continuous ( 26 ). Therapies such as reminiscence therapy and narrative therapy tap into this discussion when they attempt to maintain psychological continuity over the lifespan of the individual by constructing a narrative out of shared memories between the person with dementia and caregivers ( 27 ). Medicines prescribed to the person with dementia also, by attempting to defer the decline in functioning, or minimizing the BPSD associated with the dementia, similarly attempt to maintain psychological continuity ( 28 ).

Further complexities are added by the change in the nature of relationships with dementia, from bilateral to inevitably unilateral, with disease progression ( 13 ). If the person with dementia in Alzheimer's disease can no longer fulfill the roles of a teacher or a parent or friend, are they still the same person they once were? Similarly, there often occurs a change in values and belief systems with cognitive decline and personality change. If the person with dementia in Pick's disease now believes physical aggression is acceptable in order to get one's way or if the person with dementia in Parkinson's disease now believes there is nothing wrong with gambling at cards or shopping excessively, are they still the same person they once were?

How many changes must Alice accumulate in body and mind, until she stops being Alice? Does Alice cease to exist when she loses her abilities to think and relate and express or when her body dies?

How Discontinuity in Self and Identity Affects Personal Decision Making Capacity and Legal Competence

In situations where the person with dementia's personal decision making capacity is compromised by virtue of the illness and there is the possibility of legal incompetence, written advance care directives (living will), and/or nominated representatives, where available, are invoked. The preference, in medical care and in legal purview, is for shared decision making, and for a supporting patient autonomy and agency for as long as is safe and feasible. The two types of autonomy available to the patient in such a situation are ( 16 ):

1. Contemporaneous autonomy: This is the ability to utilize the self and identity to take a decision at the time (concurrent) when such a decision is required, such as whether to consent to undergo a surgery or to execute a deed of transfer of assets

2. Prospective autonomy: This is the utilization of information currently available in the public domain about the person's previous sense of (retrospective) self and identity, which are used to take a decision in the current context (prospective), such as whether based on previously held beliefs and values, the person would want to have now opted for prolongation of life.

There are two forms of prospective autonomy that are available to the person with dementia are for medical decision making are ( 16 ):

1. Documentary Directives: Written documents that contain instructions on how the person would (positive directives) and would not (negative directives) want to be treated

2. Proxy Directives: The appointment of a state sanctioned nominated representative (partial surrender of autonomy) or legal guardian (complete surrender of autonomy) who guides decision making based upon their conceptualization of the person's prior self and identity.

Both shared decision making and proxy decision making imply that there is sufficient discontinuity over time and space, by virtue of the dementia, to compromise the person's decision making ability in the specific context that is currently being invoked. Further, since current autonomy is compromised, previous autonomy is used as a substitute. Alice can no longer express an opinion on whether she wants to undergo a joint replacement surgery for her arthritis or not. Yet, enough is known of the person Alice once was, and of the values and beliefs she held dear, to know that she would want to experience a comfortable and pain free life for as long as possible. Thus the clinician discusses with her family and decides to go ahead with the surgery .

Yet, both also assume, that there is sufficient continuity over time and space that the person's core values and beliefs remain intact and do not alter as a consequence of the dementia. Where the person behaves in a manner contrary to previously held values and beliefs, such as in aggression or other harm-invoking behaviors, such is attributed to the disease rather than the person. Alice enjoyed life and wished to lie a long, healthy and comfortable life. Now, there are occasions where she expressed, in bursts of anger that she would be better off dead and sometimes tries to hurt herself. When she says this, the clinician decides that these are part of the emotional and behavioral symptoms of dementia rather than an expression of the desire for a physician assisted death, and treats this accordingly with medicines and behavioral management .

The expression of or demonstration of self harm is often transient and resolves with appropriate addressal and management of biopsychosocial factors as well as appropriate pharmacological management. Further, such agitation is usually indicative of an impairment of capacity to a sufficient degree so as to render the expressed desire to die invalid; though there is theoretical scope to interpret this in the context of previously enacted prospective autonomy. It is nevertheless, an indicator of a significant deterioration in subjective quality of life and merits examination of the goals of palliative care.

The discussion of continuity of identity becomes of particular importance where the current wishes of the person with dementia diverge from their previously expressed wishes. This is particularly so in end of life care and physician assisted death where the right to live vs. the right to die debate is invoked ( 29 ). In countries and states where the right to die in the form of the expression of directives for do-not-resuscitate (DNR) and/or euthanasia are not yet legalized, the clinician may be spared this potential conflict. Similarly, in countries and states where only negative, but not positive advance care directives are recognized, the burden of responsibility upon the physician is relatively less ( 30 ).

Discontinuity and Physician Assisted Death

However, the discussion on self and identity ceases to be merely intellectual and enters the clinical context when physician assisted death is accorded legal sanctity. A dilemma may arise in the case of a person living with dementia, who had previously articulated a request for physician assisted death (in this case, euthanasia) while retaining decision making capacity. They now express a desire to continue life or deny making the previous request while decision making capacity is currently compromised by virtue of the dementia ( 13 ).

In delirium or functional mental illnesses, decision making capacity is assumed to be only temporarily compromised by the illness and treatment would ideally, restore the person to this capacity. However, the disintegration of self and discontinuity in time and space are far more lasting in persons with dementia.

In such cases, are the person's current wishes or previous ones more valid? Further, if there is a gross enough psychological discontinuity, do the advance directives now accurately reflect the wishes of the person living now, who clearly wishes to live?

Applying Dressler's argument, the discontinuity is sufficient that the current wishes of the individual be honored and given more merit than the previous directives enacted by the person who used to be prior to the dementia ( 25 ). The alternate argument, proposed by Dworkin, however, implies bodily continuity is sufficient to give precedence to the advance care directives over the current wishes of the person ( 26 ). Thus, ironically, identity becomes most important when considering the person with dementia, when it is least apparent, and mired by the disease.

The reverse scenario though, holds equally important dilemmas. Consider the person with dementia who had indicated through advance care directives that they would want to continue with end of life care dementia while retaining capacity, but now expresses a pervasive desire to die, while in compromised capacity. Do the latter instructions merit dismissal and treatment as symptom, by virtue of the illness?

If we no longer know who Alice is or whether Alice lives here anymore, can we then, make out with any degree of conviction whether Alice would have chosen to live or to die?

Identity, Choices and the Dynamic Nature of Consent

The interaction between identity, dementia and end of life care (or where legal, the possibility of physician assisted death) may appear impenetrable, and a consideration of the same at length, pedantic. However, the intent behind such a deconstruction is to demonstrate the dynamic and ever changing nature of personhood, identity and autonomy in persons living with dementia.

As the person with dementia navigates the disease and deals with the progressive loss of abilities, disintegration of the self, and loss of decision making capacity—shared decision making and advance care directives, where available, offer support and the prospect of prospective or precedent autonomy. However, these options are best approached as dynamic processes rather than static events. There must be scope for the directives and decisions made by persons with dementia to change over time and space, in order to better reflect who they are at present ( 13 ).

Multiple recursive discussions may often be required, between the person with dementia, the clinical care team and caregivers (formal and informal) to evaluate capacity in each clinical context ( 31 ). This is best combined with repeated visitations of the person's advance care directives and proxy representatives, in a manner free from undue influence. This is important despite the asymmetry of power inherent to the clinical relationship ( 16 ). Where possible, it would be preferable to map the changes that may occur in values and belief systems due to discontinuity in identity over space and time ( 32 ).

Attempts must be made to understand and integrate these changes, where possible and to choose which of the decisions made by the person over time best represent their current identity and best interests. Advance care directives must be interactive and bear the potential for evolution.

Fixed vs. Flexible Directives

Mental Healthcare Acts across most countries indicate the presumption of capacity unless proven otherwise. The Mental Capacity Act, 2005 in the United Kingdom, in addition to the presumption of capacity goes on to require that all practicable steps be taken to help persons make decisions and that they not be treated as incompetent merely because they make an unwise decision. It goes on to require that, in the absence of capacity, decisions made for persons be in their best interests and in the least restrictive manner. Similar civil safeguards are laid down in the Mental Health Care Act, 2017, in India.

Healthcare ethics also require that a clear clinical indication for evaluation of capacity be present and that an assessment be considered only when the behavior or decision to be made has significant consequences.

While guidelines for the evaluation of contemporaneous autonomy are clear, they are less so for when the creation of prospective autonomy must be considered. Both forms supplement each other and inform the clinical decision making process. The Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatment (SUPPORT) indicates that the awareness of and utilization of advance directives and proxy representatives is low in persons with terminal illness and merely enhancing patient-physician interactions fails to address this deficit ( 33 ).

The author suggests that a discussion of prospective autonomy be included in the clinical interview of persons with dementia and their caregivers (formal and informal). Personal choices and preferences defining the terms on which people with dementia wish to live and die must be elicited during patient consultations, in a sensitive and meaningful manner. Physicians often wait for persons with dementia and their families to bring up the question of capacity (and autonomy), often in the context of a crisis such as the person being unable to live independently or manage their finances. This may mean that prospective autonomy is offered after, and not before capacity is compromised.

A pre-emptive discussion of advance directives and proxy representatives may enable collaborative decision making. Persons with dementia may require repeated discussions to facilitate education of rights and utilization of prospective autonomy. An improvement in the uptake of prospective autonomy may help patients navigate the degenerative process better. Inputs from the physician (such as the knowledge that negative, but not positive directives are legally binding) may also help persons with dementia draft and frame directives better.

Once established, it is also recommended to revisit care directives at periodic intervals, across the discontinuities in personhood, to ensure that prospective autonomy (once it takes effect) best represents the interests and wishes of the person they are now. There is merit in fixed intervals (such as 6 monthly or annual) for revisitation of prospective (but not contemporaneous) autonomy as well to elicit choices regarding palliative and end of life care. However, flexible revisitations, based upon clinical judgement and tailored to the individual may be more representative in person centered care. Further, the manner of framing by the physician may matter with experimental studies showing that expressed wishes for future care differ significantly between deficit focused and resource focused educational models ( 34 ).

Indicators for Evaluation of Continuity

Potential indicators which may merit an evaluation of continuity of personhood and autonomy are:

1. Disease based—Transition in stages of cognitive decline (based upon instruments such as the Clinical Dementia Rating Scale) or changes in personality (as may occur in frontotemporal lobar degeneration)

2. Decision based—Such as when the person is required to make a treatment choice or decide upon care arrangements

3. Person based—When there is an indication that the person's outlook on life has changed significantly. This would include the disability paradox—since people often adapt to and find meaning in living with chronic illnesses that they may have once thought intolerable.

Need for Instruments

There is a paucity of instruments to assess the continuity of personhood and identity across the spectrum of cognitive decline in older adults. Tools which may be useful in supplementing the clinical interview are ( 35 , 36 ):

1. Identity Based: Personality Inventory; Present Behavioral Examination

2. Outlook Based: Philadelphia Geriatric Center Morale Scale; Measurement of Morale in the Elderly

3. Awareness Based: Knowledge of Memory Aging Questionnaire; INSIGHT

4. Capacity Based: McArthur Competency Assessment Tool; CURVES Capacity Assessment Tool.

Conclusion and Future Directions

Much of the work on personhood and continuity of identity in persons with dementia remains theoretical. Clinical research, especially longitudinal studies examining the evolution of the sense of self, personhood, identity, capacity and choices would provide further insights. Another area of interest would be examining how attitudes toward dementia change with the availability of treatments which may modify or reverse the course of illness, such as the recently approved but controversial Aducanumab ( 37 ).

Where possible, end of life decisions must proceed with the consent or assent of the individual with dementia. Requests for physician assisted death, where legal, must be considered carefully, and at length, bearing in mind the potential for changes in identity and choice over the course of the illness. The living (and dying) will, if it is to fulfill its purpose and represent the person with dementia, should be truly living .

Author Contributions

The author confirms being the sole contributor of this work and has approved it for publication.

Conflict of Interest

The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Acknowledgments

The author wishes to acknowledge Dr. Debanjan Banerjee for his valuable inputs.

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Keywords: dementia, neurocognitive disorders, euthanasia, physician assisted dying, physician assisted death, decision making capacity, legal competence, consent and refusal to treatment

Citation: D'cruz MM (2021) Does Alice Live Here Anymore? Autonomy and Identity in Persons Living and Dying With Dementia. Front. Psychiatry 12:700567. doi: 10.3389/fpsyt.2021.700567

Received: 26 April 2021; Accepted: 22 June 2021; Published: 22 July 2021.

Reviewed by:

Copyright © 2021 D'cruz. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

*Correspondence: Migita M. D'cruz, migitadcruz@gmail.com ; mig3632@nimhans.ac.in

Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Application of the Person-Centered Care to Manage Responsive Behaviors in Clients with Major Neurocognitive Disorders: A Qualitative Single Case Study

Affiliations.

• 1 Occupational Science and Occupational Therapy Department, University of Toronto, Toronto, Canada.
• 2 School of rehabilitation, Faculty of Medicine, Université de Montréal, Montréal, Canada.
• 3 Faculty of Nursing, Université de Montréal, Montréal, Canada.
• 4 Centre de recherche de l'Institut universitaire de gériatrie de Montréal, CIUSSS Centre-sud-de-l'île-de-Montréal, Montréal, Canada.
• 5 Institut universitaire de gériatrie de Montréal, CIUSSS Centre-sud-de-l'île-de-Montréal, Montréal, Canada.
• 6 Department of Psychiatry and Addictology, Université de Montréal, Montréal, Canada.
• 7 Department of Family Medicine, Université de Montréal, Montréal, Canada.
• 8 School of Physical and Occupational Therapy, McGill University, Montréal, Canada.
• 9 Centre for Research and Expertise in Social Gerontology, CIUSSS West-Central Montreal, Cote Saint-Luc, Canada.
• PMID: 36591952
• DOI: 10.1080/07317115.2022.2162468

Objectives: Our study aimed to describe "how" and "why" the person-centered care (PCC) approach was applied within a long-term care (LTC) community to manage responsive behaviors (RBs) in individuals with major neurocognitive disorders.

Methods: A descriptive holistic single case study design was employed in the context of an LTC community in Quebec, using semi-structured interviews and non-participatory observations of experienced care providers working with clients with RBs, photographing the physical environment, and accessing documents available on the LTC community's public website. A thematic content analysis was used for data analysis.

Results: The findings generated insight into the importance of considering multiple components of the LTC community to apply the PCC approach for managing RBs, including a) creating a homelike environment, b) developing a therapeutic relationship with clients, c) engaging clients in meaningful activities, and d) empowering care providers by offering essential resources.

Conclusions: Applying and implementing the PCC approach within an LTC community to manage clients' RBs is a long-term multi-dimensional process that requires a solid foundation.

Clinical implications: These findings highlight the importance of considering multiple factors relevant to persons, environments, and meaningful activities to apply the PCC approach within LTC communities to manage RBs.

Keywords: Person-centered care; care providers; long-term care settings; neurocognitive disorders; responsive behaviors.

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Update on Major Neurocognitive Disorders

Dementia is a syndrome characterized by a gradually progressive course that spans a continuum from preclinical symptoms to major impairment in two or more cognitive domains with functional decline. In this review, the author examines some of the more common dementia syndromes from among dozens of different diseases. Findings show that as the U.S. population continues to age, the number of Americans with dementia is expected to rise drastically over the next several decades. This upsurge will contribute to increased health care costs and will have a significant public health impact. Neurodegenerative disorders such as Alzheimer’s disease, frontotemporal degeneration, and alpha-synucleinopathies (e.g., Lewy body disease and Parkinson’s disease) are some of the more prevalent causes for dementia. In recent years, advancements in neuroimaging, understanding of genetic contributions and pathological changes, and the development of novel biomarkers have fueled clinical understanding of these disorders. However, substantial disease-modifying therapies are still lagging. The advent of future interventions hinges on the ability to discern the distinct clinico-pathologic profiles of the various dementia syndromes and to identify reliable biomarkers for utilization in clinical trials.

Dementia is a syndrome characterized by difficulties with memory, language, problem solving, and other cognitive domains, which lead to functional impairment in the individual. In clinical practice, the nomenclature used to describe dementia is often defined by the DSM . The DSM-5 introduces the neurocognitive disorders category as a continuum of cognitive decline spanning from mild neurocognitive disorder, or preclinical dementia, to major neurocognitive disorder, or dementia. In doing so, this new nomenclature helps to facilitate the recognition and treatment of a neurocognitive impairment before its manifestation into a dementia syndrome. The DSM-5 defines major neurocognitive disorder as a significant cognitive decline from a previous level of performance in one or more cognitive domains, including complex attention, executive function, learning and memory, language, perceptual-motor, and social cognition. The cognitive deficits interfere with independence in everyday activities, do not occur exclusively in the context of a delirium, and are not better explained by another mental illness ( 1 ). Minor neurocognitive disorder is evidenced by a modest cognitive decline from a previous level of performance in one or more of the same aforementioned cognitive domains listed for major neurocognitive disorder. In mild neurocognitive disorder, the cognitive deficits do not interfere with capacity of independence in everyday activities ( 1 ).

Epidemiology

As the number of Americans ages 65 and older continues to increase, the cases of dementia will continue to grow. The population of Americans ages 65 and older is projected to grow from 43.1 million in 2012 to 83.7 million by 2050. The baby boomers are largely responsible for this growth; moreover, by 2030, all baby boomers will be older than age 65 ( 2 , 3 ). The number of people ages 65 and older also continues to increase worldwide, from 617 million in 2015 to a projected 1.6 trillion by 2050 ( 4 ). The prevalence of dementia doubles every 5 years between ages 65 and 85, and it continues to increase after age 90 ( 5 , 6 ). In 2010, it was estimated that 4.7 million individuals ages 65 and older had dementia caused by Alzheimer’s disease. Of these individuals, 0.7 million were between ages 65 and 74, and 2.3 million were between ages 75 and 84. By 2050, the number of individuals with dementia caused by Alzheimer’s disease is projected to be 13.8 million, with 7 million age 85 or older ( 7 ).

Advanced age and sex, the two most prominent risk factors for dementia, are not modifiable. Gender has an influence on dementia risk when societal factors, such as differences in healthy lifestyle and opportunities for advanced educational attainment, come into play ( 8 ). Lifestyle factors such as smoking, excessive alcohol intake, and poor diet modulate susceptibility to dementia in both men and women. However, more women than men have dementia, with almost two-thirds of Americans with Alzheimer’s disease being women ( 9 ). Overall, little difference has been found between the sexes in incidence of dementia; however, by absolute numbers, more women than men have the disease because of differences in life expectancy ( 10 ).

Racial-ethnic minority groups appear to make up a greater number of dementia cases. Studies in the United States showed incidence rates that were higher for African Americans and Latinos than for Whites; however, these differences disappeared after adjustment for education level or socioeconomic status ( 11 , 12 ). This finding demonstrates that racial-ethnic differences are likely related to variations in medical conditions, such as higher incidence of cardiovascular disease and diabetes mellitus; health-related behaviors, such as perceptions of normal and abnormal aging and long-standing issues of trust between racial-ethnic minority groups and the medical establishment; and socioeconomic risk factors, such as access to adequate medical care, higher education, health literacy, and access to adequate nutrition. Genetic factors do not appear to play a role in the differences in prevalence or incidence among racial-ethnic minority groups ( 13 ).

Impact of Disease

Because of the long duration of illness before death, dementia contributes significantly to the public health impact because much of that time is spent in a state of disability or dependence, especially in lieu of any currently available treatments to halt disease progression. Undeniably, this long duration of disability will have a far-reaching impact on national health care costs, including the increasing need for caregivers to shoulder the burden of care provided to older adults with dementia. In 2020, the total national cost of caring for people with dementia reached $305 billion. Individuals with dementia have twice as many hospital stays, more skilled nursing facility stays, and more home health care visits per year than older adults without dementia ( 9 ). In 2010, 83% of the help provided to older adults in the United States came from family members, friends, or other unpaid caregivers ( 14 ). In 2019, dementia caregivers provided an estimated 18.6 billion hours of unpaid assistance valued at $244 billion ( 9 ).

Evaluation and Diagnosis

The initial evaluation and diagnoses of dementia should include a thorough clinical assessment with a collateral history, if available; a neurological examination with assessment of mental status; selective labs to rule out metabolic and physiologic abnormalities; and structural brain imaging, preferably with magnetic resonance imaging (MRI) instead of computed tomography (CT) because these images generate a higher resolution image of cortical structures, enabling a more detailed assessment of atrophy ( 15 ). Collateral informants may include spouses, adult children, or other primary caretakers who can attest to the natural progression of symptoms and functional changes over the course of months or years. Having this additional information can be vitally important, especially if, because of degree of clinical severity, the patient is unable to give a thorough history or lacks insight into their impairments. Common labs important to a dementia workup include basic chemistries, a thyroid panel, a B12-folate test, and a vitamin D test. On the basis of the patient’s clinical history, additional tests may be warranted, including Treponemal pallidum antibody or venereal disease research laboratory, human immunodeficiency virus-ab, antinuclear antibody, erythrocyte sedimentation rate, and a heavy-metal screen. T1-weighted structural MRI is very useful in detecting focal loss of gray matter volume loss, otherwise indicated as atrophy, which is a common feature of neurodegenerative dementias ( 16 ).

Clinical Assessment

The emphasis of the clinical interview should be placed on establishing a thorough patient history backed by reliable collateral sources, when available, and creating a detailed timeline of clinical symptoms and functional decline. This timeline should include the rate of symptom onset (e.g., rapid vs. gradual) and the pace of symptom progression (e.g., decline over months vs. years) ( 15 ). For example, human prion diseases, such as Creutzfeldt-Jakob disease, typically progress quite rapidly, over weeks to months. In contrast, dementias caused by Alzheimer’s disease, diffuse Lewy body disease, and frontotemporal diseases progress much slower, over a period of years. As a result of the rapid or gradual cognitive decline, patients also lose the ability to perform complex instrumental activities of daily living (IADLs) and eventually basic activities of daily living (ADLs). For example, patients who have progressed to moderate and severe stages of dementia have marked impairments in cognitive function and have usually lost the ability to carry out most IADLs ( 17 ). At these stages, the accurate reporting of symptoms and functional decline will necessitate the use of reliable caretakers or family members. Accurately measuring functional loss is important because the nature and extent of functional losses associated with disease progression help determine the type and level of care needed, ranging from medication management in early stages to full-time care or institutionalization in later stages ( 18 ).

Cognitive Evaluation

A detailed mental status examination should assess several cognitive domains, including attention, memory, executive functioning, and visuospatial ability. Several instruments have been developed to help clinicians assess the presence and severity of cognitive impairment. Tools such as the 30-point Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MoCA) are more commonly used and are helpful in assessing the presence and severity of dementia. The MoCA offers a broader assessment of cognitive domains and can be more sensitive than the MMSE for early detection of neurodegenerative diseases. Although commonly still used, the MMSE has a ceiling effect for higher functioning older adults and is poorly sensitivity for distinguishing mild cognitive impairment (MCI), which increases the likelihood that people in predementia stages will score within the normal range (≥24) ( 19 – 21 ). Full neuropsychological testing may be helpful in instances in which screening tests and clinical impression remain ambiguous.

Alzheimer’s Disease

Alzheimer’s disease remains one of the leading causes of progressive dementia; moreover, as the number of Americans ages 65 and older continues to increase, the cases of Alzheimer’s disease and other dementias will also continue to grow. In 2020, an estimated 5.8 million Americans ages 65 and older were living with the disease; 80% were ages 75 or older ( 7 ). Out of the total U.S. population, one in 10 people (10%) ages 65 and older has Alzheimer’s disease; moreover, the percentage of people with Alzheimer’s disease increases with age: 3% of people ages 65–74, 17% of people ages 75–84, and 32% of people ages 85 and older ( 7 , 9 ). It is also important to note that people younger than age 65 can also develop Alzheimer’s disease, but it is much less common.

The Continuum of Disease

The Alzheimer’s disease continuum reflects the progression from brain changes that are not noticed by the affected person to brain changes that cause problems with memory and eventually physical disability. The continuum has three phases: preclinical Alzheimer’s disease, MCI due to Alzheimer’s disease, and dementia due to Alzheimer’s disease. Each of these phases is further broken down into mild, moderate, or severe stages that reflect the impact of the disease on an individual’s daily function. How long individuals spend in each part of the continuum varies and can be influenced by age, genetics, gender, and other factors ( 22 ).

Preclinical Alzheimer’s Disease and Biomarkers

During the preclinical Alzheimer’s disease phase, individuals have the earliest signs of the disease, which are detectable with biomarkers; however, they have yet to develop symptoms such as memory loss or change in functionality. Biomarkers, the earliest measurable brain changes, include measures of beta-amyloid (Aβ) deposition and glucose metabolism in the brain. Positron emission tomography (PET) scans use an Aβ-specific ligand, known as Pittsburgh compound B, to detect levels of cerebral load in the brain. Analysis of cerebrospinal fluid (CSF) can detect drops in Aβ levels, indicating a reduced clearance of Aβ into the CSF and increased aggregation of the peptide into amyloid plaques in the brain ( 23 ). PET imaging with the 2-deoxy-2-fluoro-D-glucose (FDG) tracer measures the cerebral metabolism of glucose and has a specific topographic distribution pattern: bilateral reduction in FDG uptake in the temporal and parietal regions and especially in the cingulate cortex ( 23 ). It is important to note that not all individuals with evidence of Alzheimer’s disease–related brain changes go on to develop symptoms of MCI or dementia due to Alzheimer’s disease ( 24 ).

MCI Due to Alzheimer’s Disease

Individuals with MCI due to Alzheimer’s disease have biomarker evidence of brain changes plus subtle changes in memory and thinking that do not interfere with overall daily function. These changes occur when the brain can no longer compensate for the damage and nerve cell death caused by Alzheimer’s disease. Eventually, these patients can progress to full Alzheimer’s dementia, with several studies indicating a range of conversion rates anywhere from 15% to nearly 40% within 2–5 years of initial MCI diagnosis ( 25 – 27 ). The 2018 study by Petersen et al. ( 25 ) demonstrated a cumulative dementia incidence of 14.9% over 2 years in individuals with MCI older than age 65. In some cases, MCI will revert to normal or remain stable over time.

Clinical Alzheimer’s Disease

Patients diagnosed as having dementia due to Alzheimer’s disease have biomarker evidence of brain changes with very clear changes in memory, thinking, or behavior that affect an individual’s daily function. They can exhibit multiple symptoms that progress over years and reflect the degree of nerve cell damage in different parts of the brain. The rate at which dementia symptoms progress varies from person to person but is generally categorized in stages ranging from mild, moderate, to severe. In mild stages of Alzheimer’s disease, many people can function independently in many areas but typically require assistance with some activities to optimize safety and independence. In moderate stages of Alzheimer’s disease, which tend to last the longest, individuals may have trouble with communication and performing routine tasks, such as ADLs. Some may develop incontinence at times. Others may start to demonstrate personality and behavioral changes, which are often some of the first symptoms recognized by family members or caretakers that will prompt their desire for a clinical evaluation by a medical professional. In severe stages, individuals need help with ADLs 24 hours per day, 7 days per week, and the effects of Alzheimer’s disease on their general health become apparent. Damage to areas of the brain that control movement lead to patients being more at risk for falls and becoming bed bound, which makes them more vulnerable to blood clots, bed sores, infections, and even sepsis. Damage to the areas of the brain that control swallowing leads to increased risk of aspiration, which can cause aspiration pneumonia.

Risk Factors and Genetics

Most people who develop dementia caused by Alzheimer’s disease do so at age 65 or older, which is often referred to as late onset. Several risk factors exist for late-onset Alzheimer’s disease, which is not thought to be related to any singular cause. Overall, age is the greatest risk factor for late-onset Alzheimer’s disease; moreover, the percentage of people with dementia caused by Alzheimer’s disease increases dramatically with age, as previously mentioned ( 7 ). Genetics is another risk factor, and researchers have found several genes that increase the risk of Alzheimer’s disease, with the apolipoprotein (APOE) e4 gene conferring the strongest impact on risk. Everyone inherits one of the three forms of the APOE gene from each parent, resulting in six possible pairs: e2-e2, e2-e3, e2-e4, e3-e3, e3-e4, and e4-e4. Having the e4 allele of APOE increases the risk of developing dementia in comparison with having the e3, but it does not guarantee that an individual will necessarily develop Alzheimer’s disease. Having the e2 form may decrease risk compared with having the e3 form. Those with just a single copy of the e4 allele have three times the risk of developing Alzheimer’s disease than those with two copies of the e3 form. Individuals who have two copies of the e4 allele are eight to 12 times more likely to develop Alzheimer’s disease ( 28 – 30 ). Finally, family history confers a risk on developing Alzheimer’s disease. Individuals who have a first-degree relative with Alzheimer’s disease are more likely to develop the disease, with an even higher risk for those who have more than one first-degree relative with the disease ( 28 , 31 ). Of course, when diseases run in families, shared nongenetic factors such as access to healthy foods and habits related to physical activity may also play a role.

Although age, genetics, and family history are considered nonmodifiable, certain risk factors can be changed or modified to reduce the risk of dementia. According to the 2019 published recommendations by the World Health Organization ( 32 ), cognitive impairment and dementia are associated with lifestyle-related risk factors, such as physical inactivity, tobacco use, unhealthy diets, and harmful use of alcohol. In addition, certain medical conditions, including hypertension, diabetes, hypercholesterolemia, obesity, and depression, also confer an increased risk of developing dementia. Other potentially modifiable risk factors include social isolation and cognitive inactivity.

Less commonly, patients can develop dementia due to Alzheimer’s disease before age 65, sometimes as early as age 30. This manifestation is called early-onset Alzheimer’s disease, which occurs in less than 1% of Alzheimer’s disease cases and develops from mutations to any three specific genes ( 33 ). Mutations in the genes for amyloid precursor protein, presenilin 1 protein, or presenilin 2 protein virtually guarantee the development of Alzheimer’s disease during a normal life span. Amyloid precursor protein and presenilin 1 mutations are associated with complete penetrance, whereas mutations in presenilin 2 are associated with a 95% penetrance ( 34 ). Each of the Alzheimer’s disease genes plays a role in the production, trafficking, and clearance of Aβ, where its deposition into amyloid plaques, along with neurofibrillary tangles, plays a major role in the pathologic process of Alzheimer’s disease.

Current therapies.

As previously described, the identification and use of biomarkers for Alzheimer’s disease allow for early disease detection. In addition, biomarkers have become a powerful tool in clinical practice and research by improving diagnoses, increasing clinical trials, and helping to accelerate the development of new therapies ( 9 , 23 ). None of the current pharmacologic treatments available today prevent the progression of neurodegeneration that causes Alzheimer’s disease. Acetylcholinesterase inhibitors (AChEIs) and N -methyl-D-Aspartate antagonists are the only drugs approved by the U.S. Food and Drug Administration (FDA) for the treatment of Alzheimer’s disease; however, no consensus has been reached that either drug even modestly delays progression ( 35 ). The drugs included within these two groups are rivastigmine, galantamine, donepezil, memantine, and memantine combined with donepezil. The modest benefits of these drugs have to be balanced against the potential adverse effects, including gastrointestinal intolerance, bradycardia, and gastrointestinal bleeding, to name a few.

Prospective therapies.

Since the discovery that Alzheimer’s disease may begin 20 years or more before symptom onset, a significant window has emerged allowing for future therapies to intervene earlier along the continuum. As of March 13, 2020, the IgG1 monoclonal antibody BIIB037 (aducanumab) is being used in a phase 3b open-label trial among participants with Alzheimer’s disease, who participated in previous aducanumab studies (including PRIME, ENGAGE, and EMERGE), as a potential new treatment for Alzheimer’s disease ( 36 ). The previous phase 3 efficacy trial with aducanumab, EMERGE, demonstrated that it had met its primary endpoint. Patients on the highest dose, 10 mg/kg, had significant reduction in decline on the Clinical Dementia Rating Scale-Sum of Boxes, a tool that the FDA uses as a single primary efficacy endpoint and to assess disease progression ( 37 , 38 ). The current phase 3b trial is projected to run through September 2023.

Frontotemporal Degeneration (FTD)

FTD is a heterogenous spectrum of neurodegenerative disorders with diverse clinical presentations, genetic attributes, and neuropathological characteristics ( 39 ). They are linked by the selective degeneration of the frontal and temporal lobes. In the past considered to be a rare disease, FTD has been shown to be more frequent than previously thought, with an estimated lifetime risk of one in 742 by recent epidemiological studies and updated clinical criteria ( 39 , 40 ). FTD has been established as the second most common cause of dementia for people younger than age 65, with a prevalence that approximates that of Alzheimer’s disease ( 40 – 42 ). However, the overall prevalence increases beyond age 65, with a rate doubling that of those ages 40–64 ( 40 , 42 ).

Clinically, FTD is characterized by behavioral abnormalities, language impairment, and deficits of executive function. In accordance, the current revised diagnostic criteria list two different clinical forms: behavioral variant of FTD (bvFTD) and primary progressive aphasia (PPA; the two most common forms include the semantic variant of PPA [svPPA] and the agrammatic variant of PPA [avPPA]). Frequently thought of as a tauopathy, or a neurodegenerative disease involving aggregation of tau protein in the brain, the heterogeneity in both the clinical presentations and the neuropathological hallmarks of FTD subtypes are all primarily related to neuronal protein tau or transactive response DNA-binding protein 43 (TDP-43) depositions.

Considered the most common clinical form of FTD, bvFTD is predominately characterized by personality changes, apathy, disinhibited or compulsive behaviors, executive dysfunction, as well as stereotypic speech and motor behaviors ( 15 , 43 ). According to the new consensus criteria for the diagnosis of bvFTD, the degree of diagnostic certainty can be ranked as possible, probable, or definite. To reach a diagnosis of possible bvFTD, three of six core criteria must be met. To reach a diagnosis of probable bvFTD, patients have to first meet possible criteria and also show changes in frontal or temporal regions on neuroimaging. When pathologic or genetic confirmation has been made, the bvFTD diagnosis is considered definite ( 43 , 44 ).

Social cognition is core to the syndrome and refers to the ability to recognize emotion in others, mentalizing about other people’s state of mind (i.e., theory of mind), empathy, knowledge of social norms, moral reasoning, reward sensitivity, evaluating relevance of incoming social and emotional information, and flexibly using this information to behave appropriately within social contexts ( 41 ). Dysfunction in social cognition is a key feature in bvFTD, with changes indicating a progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation, and decision making ( 45 – 47 ).

Apathy is very common and manifests as inertia, reduced motivation, lack of interest in previous hobbies, and decreased social interest leading to progressive social withdrawal. Disinhibition often coexists with apathy, leading to impulsive actions such as excessive spending, sexually inappropriate remarks, and other socially tactless behaviors. Repetitive or stereotypic behaviors might present with perseveration and tendency to repeat phrases, stories, or jokes. Excessive hoarding leading to a state of squalor, new-onset pathological gambling, or (even more rarely) hyper-religiosity can be presenting features ( 48 ). Hyperorality, typically seen as increased food consumption with predominate sweet cravings, is another defining feature reflecting early involvement in the hypothalamus ( 41 ). Mental rigidity is also common, and patients can have difficulty adapting to new situations or routines.

Onset is often difficult to determine because of limited or absent insight on the part of the patient. Therefore, the history of a caregiver is essential during the interview process because prominent changes in social comportment, appropriateness, and apathy are often reported ( 48 ). Early in the disease process, patients with bvFTD can perform well on formal neuropsychological tests despite the presence of significant personality and behavioral changes ( 48 ). Later, the neuropsychological profile is characterized by deficits in executive function. Prior studies have emphasized a relative sparing of episodic memory and visuospatial skills on neuropsychological testing ( 44 ). However, more recent evidence from a synthesis of neuropsychological studies, in light of neuroimaging and neuropathological findings, demonstrates involvement of structures known to be crucial for episodic memory ( 43 , 49 ). It was initially presumed that episodic memory difficulties in bvFTD reflected degeneration of prefrontal cortical regions; however, it is becoming increasingly clear that anterior and medial temporal regions, including the hippocampi, are heavily involved ( 50 , 51 ).

Psychotic symptoms in FTD, previously considered rare, occur more frequently than previously thought ( 52 ). Currently, psychotic symptoms are increasingly recognized as a presenting or early feature of FTD ( 53 ). More recent studies have demonstrated a linkage between patients with FTD and a hexanucleotide repeat expansion in the chromosome 9 open reading frame 72 (C9ORF72) gene; C9ORF72 is often specifically associated with bvFTD and motor neuron disease ( 54 , 55 ). The prevalence of the mutation in FTD varies depending on the population studied and ranges from 5% to 35% ( 56 ). Prominent features among most patients with this mutation are highly abnormal behaviors of a psychotic nature. Many patients presenting with florid psychosis are initially classified by their psychiatrist as having primary psychotic disorders, such as paranoid schizophrenia ( 54 ). Delusions are more frequently present than hallucinations and are mainly persecutory or paranoid in nature; however, erotomania, somatic delusions, and Cotard’s syndrome can also occur ( 53 , 55 ).

PPA is the second major form of FTD that affects language skills, speaking, writing, and comprehension. Neuroimaging shows asymmetric atrophy of the anterior temporal lobe among affected individuals, usually the left side is more involved than the right side ( 43 ). The two most common forms of PPA are svPPA and avPPA.

The progressive breakdown of semantic memory, which stores knowledge about objects and words, is a characterization of svPPA. Speech remains fluent with normal grammar, but it increasingly contains meaningless content, prominent anomia, and impaired word comprehension ( 43 ). Individuals lose the ability to understand or formulate words in a spoken sentence. Over time, patients develop impaired recognition and worsening behavioral symptoms, as seen in bvFTD.

In avPPA, a person’s speech is very hesitant, labored, or ungrammatical. Speech distortion is due to the breakdown in motor planning, or speech apraxia, causing impairment of rhythm and the normal stress patterns of speech ( 43 ). Word comprehension is normal, but sentence comprehension is impaired because of problems with grammatical structure. Word repetition is also often impaired because of errors in articulation ( 57 ).

Frontotemporal dementia can be caused by a highly heritable group of neurodegenerative disorders, with nearly 30% of patients having a strong family history ( 58 ). Autosomal dominant transmission accounts for the majority of heritability, with mutations in the C9ORF72, progranulin, and microtubule-associated protein tau genes. However, other genes are associated with rare FTD cases, such as valosin-containing protein mutations, charged multivesicular body protein 2B, fused in sarcoma (FUS) protein, TDP-43, sequestosome 1 protein, TANK-binding kinase 1 protein, and ubiquilin 2 protein ( 39 ). C9ORF72 seems to be the most common worldwide cause of genetic FTD, followed by progranulin and then microtubule-associated protein tau genes ( 58 ). In each of the gene groups with amyotrophic lateral sclerosis, bvFTD is most common; among C9ORF72 carriers, bvFTD is also common.

Frontotemporal pathology in FTD is characterized by severe focal atrophy of frontal and temporal regions, subcortical gliosis, and neuronal loss ( 43 ). Neuroimaging studies, such as MRI and FDG-PET, can show gray matter atrophy and hypometabolism at least 10 years before symptom onset ( 58 ). Initially, pathological diagnosis was based on atrophy and neuronal and glial cell inclusions of hyperphosphorylated tau protein; however, more than 50% of patients with FTD proved to be tau negative but ubiquitin positive ( 59 ). Most FTD-ubiquitin inclusions are composed of TDP-43. The remaining 5% are tau and TDP-43 negative with inclusions of FUS protein, referred to as FTD-FUS ( 59 ).

Because of its heterogeneity, the diagnostic process in FTD can be fraught with difficulty; moreover, having a correct diagnosis is essential to the future of clinical trials for disease-modifying treatments. Great efforts have been made over the last 2 decades to identify biomarkers sensitive to FTD, with a predominant focus on fluid biomaterial and neuroimaging.

FTD studies have primarily focused on structural changes assessing gray matter atrophy and hypometabolism, which have been fairly consistent and validated diagnostic biomarkers between studies. However, white matter changes are probably more sensitive for the earliest changes in FTD than gray matter changes ( 60 ). Studies within the last 5–10 years have examined white matter integrity using diffusion tensor imaging. This technique measures the microstructural integrity of white matter by determining the rate of diffusion in different directions; the changes in different diffusion tensor imaging metrics are thought to reflect different pathological changes in microstructure. In FTD, white matter diffusivity has been found to precede gray matter atrophy and with a more widespread distribution in the brain. Four potential areas of application have been identified: differentiating between individuals with FTD, individuals with other types of dementia, and individuals without dementia; differentiating between subtypes of FTD; disease monitoring; and detection of early changes before disease onset ( 60 ).

A combination of fluid biomarkers in the CSF are likely to yield more information than single markers. For example, phosphorylated TDP-43 constitutes one of the major pathological subtypes of FTD; moreover, neurofilaments are a major constituent of the neuroaxonal cytoskeleton and play an important role in axonal transport and in the synapse ( 61 ). Neurofilament light chain blood and CSF levels are 2.5–11 higher among patients with FTD than among control groups and are thought to reflect axonal damage ( 62 – 66 ).

No treatments are available that will change the course of FTD, so the focus of medical therapy is on symptomatic relief using pharmacological and nonpharmacologic methods. Nonpharmacologic interventions should focus on patient safety and well-being. Discussions with family should include management of financial accounts and credit cards, driving safety, and environmental factors to ensure physical safety. Exercise and physical therapy can be helpful for patients with motor problems. Among patients with hyperorality, special attention to diet should be made by caretakers to avoid excessive weight gain.

No FDA-approved pharmacologic interventions exist for any of the FTD subtypes. Anticholinesterase inhibitors are not recommended because no evidence supports a deficit of acetylcholine in this disease ( 67 ). Studies in bvFTD have shown serotonergic network disruption, with decreased serotonin levels and 5-HT1A and 5-HT2A receptors in frontotemporal regions and neuronal loss in the raphe nuclei ( 68 , 69 ). Evidence suggests that selective serotonin reuptake inhibitors (SSRIs) are effective in helping with various symptoms of FTD, including disinhibition, impulsivity, repetitive behaviors, and eating disorders ( 69 – 71 ). Evidence also exists for dopaminergic disruption, with low levels of dopamine metabolites and severely reduced presynaptic dopamine transporters in the putamen and caudate of patients with FTD ( 70 , 72 ). Some patients with bvFTD will require the use of antipsychotics for the treatment of severe neurobehavioral symptoms; however, they are usually reserved if no success was achieved with nonpharmacologic interventions or SSRIs. These patients tend to be more sensitive to extrapyramidal side effects because of diminished dopaminergic function; therefore, agents with less D2 receptor antagonism, such as quetiapine, are preferred ( 73 ).

Alpha-Synucleinopathies

Parkinson’s disease (PD) and Lewy body disease (LBD) are both neurodegenerative diseases caused by filamentous aggregates of misfolded alpha-synuclein protein, which form the eosinophilic intracytoplasmic inclusions known as Lewy bodies in the brain ( 74 ). Alpha-synuclein is normally present in synapses and encoded by chromosome 4 ( 75 ). Although its function is not fully understood, it seems to play a role in vesicle production. Staining for alpha-synuclein is now used routinely for identifying Lewy bodies and Lewy neurites ( 76 ). As with most neurodegenerative disorders, no disease-modifying drugs exist, limiting treatment options to symptomatic relief and palliative measures.

Parkinson’s disease dementia (PDD) and LBD affect cognition, behavior, movement, and autonomic function. In both dementias, the accumulation of misfolded alpha-synuclein protein in the form of Lewy bodies and Lewy neuritis leads to the loss of dopaminergic and cholinergic cells, often with a variable degree of coexisting Alzheimer’s disease pathology. Both have overlapping clinical features. However, the main difference is in the sequence of symptoms. In PDD, motor symptoms precede dementia or develop within 12 months of dementia. In LBD, motor symptoms either occur simultaneously or follow dementia. The two disorders represent two entities on a continuum, with many similarities but some differences ( 77 ).

Dementia With Lewy Bodies (DLB)

Epidemiology..

LBD is currently considered the second most common type of neurodegenerative disorder leading to dementia among older people, accounting for 10%–15% of cases at autopsy ( 78 ). However, DLB is often underrecognized and underdiagnosed because of difficulties differentiating DLB from Alzheimer’s disease. As a result, the true prevalence and incidence rates of DLB in the community are difficult to estimate ( 79 ).

Lewy bodies are the primary lesions found in degenerating neurons of the limbic system, brainstem (substantia nigra, locus coeruleus), and neocortex of patients with DLB. As previously mentioned, Lewy bodies consist of abnormal eosinophilic intracytoplasmic filamentous aggregates of misfolded alpha-synuclein protein. These abnormal neurofilaments have been found to also contain tau and ubiquitin ( 74 ). However, patients with DLB may also have amyloid deposition because of the high occurrence of mixed Alzheimer’s disease and PD pathology found postmortem ( 79 ). This finding is supported by evidence that alpha-synuclein stimulates the fibrillation of Aβ and tau proteins ( 80 ). Some structural changes are similar to those seen among patients with Alzheimer’s disease, with wide cerebral atrophy being a feature of both Alzheimer’s disease and DLB ( 81 ). However, unlike Alzheimer’s disease, the medial temporal lobe is relatively spared in DLB ( 82 ).

Most patients with DLB show a loss of pigmented, dopaminergic neurons in the substantia nigra, similar to what is seen in PD ( 83 ). However, the main pathological changes in DLB affect the neocortex and limbic system. On the basis of current international neuropathological staging systems, it is impossible to distinguish DLB from PDD, which shares similar clinical, neurochemical, and morphological characteristics with DLB. However, imaging and postmortem studies suggest that patients with DLB exhibit elevated limbic and striatal Alzheimer’s disease–related pathologies as well as a lesser degree of dopaminergic cell loss compared with patients with PDD ( 84 – 86 ).

Clinical features.

In 2017, the DLB Consortium published its revised recommendations for the clinical and pathologic diagnosis of DLB in its fourth consensus report, updating its previous report that was used widely for the last decade ( 87 ). Probable DLB is diagnosed if two or more core clinical features of DLB are present with or without the presence of indicative biomarkers, or if only one core clinical feature is present with one or more indicative biomarkers. Possible DLB is diagnosed if one core clinical feature is present with no indicative biomarker evidence, or if one or more indicative biomarkers are present with no core clinical features.

Essential to the diagnosis of DLB is dementia, a progressive cognitive decline sufficient to interfere with function or daily activities. Prominent or persistent memory impairment may not occur in the early stages but is evident with progression. However, disproportionate deficits on tests for attention, executive function, and visuospatial ability may be prominent and occur early. Dementia screens such as the MMSE and the MoCA are useful to characterize global impairment in DLB; however, neuropsychological assessment should include tests covering the full range of cognitive domains affected. Tests of processing speed and divided-alternating attention are measures of attention and executive function that can help to differentiate DLB from Alzheimer’s disease and normal aging. Examples include the Stroop tasks, trail-making tasks, phonemic fluency, and computerized tasks of reaction time. Tasks of figure copy can help to detect spatial and perceptual deficits that often occur early in DLB. Examples include block design or puzzle tasks, spatial matching, and perceptual discrimination.

The core clinical features of DLB include fluctuating cognition, recurrent complex visual hallucinations, rapid eye movement (REM) sleep behavior disorder, and parkinsonism. Fluctuating cognition, visual hallucinations, and REM sleep behavior disorder tend to occur early and can persist throughout the course of DLB.

The supportive clinical features include severe sensitivity to antipsychotic agents, postural instability, repeated falls, syncope, severe autonomic dysfunction, hypersomnia, hyposmia, hallucinations, delusions, apathy, anxiety, and depression.

Biomarkers.

According to the 2017 consensus report of the DLB Consortium, direct biomarker evidence of Lewy body pathology is not yet available for clinical diagnosis; however, several useful indirect methods exist ( 87 ).

Indicative biomarkers include reduced dopamine transporter uptake in the basal ganglia by single photon emission computed tomography (SPECT) or PET imaging; reduced uptake on metaiodobenzylguanidine myocardial scintigraphy, which quantifies postganglionic sympathetic cardiac innervation and is reduced in LBD; and polysomnography confirmation of REM sleep without atonia, which is highly predictive of Lewy-related pathology.

Supportive biomarkers are consistent with DLB and help the diagnostic evaluation even though they lack clear diagnostic specificity. These biomarkers include relative preservation of medial temporal lobe structures on CT or MRI scans, generalized low uptake on SPECT-PET perfusion-metabolism scans, reduced occipital activity and the posterior cingulate island sign on FDG-PET imaging, and prominent posterior slow-wave electroencephalography activity with periodic fluctuations in the pre-alpha-theta range.

Cognitive impairment is the most common nonmotor feature of PD ( 88 ). Although subtle cognitive deficits may be detected in the early stages of PD, overt cognitive deficits usually manifest in the later stages as the age advances ( 77 ). The point prevalence of PDD is estimated to be up to 30%, but longitudinal data demonstrate dementia prevalence rates as high as 80% at more advanced stages of PD; these data support the theory that dementia is an inevitable manifestation of the disease ( 89 – 91 ).

Old age at the time of disease onset or at the time of evaluation is the most established risk factor for PDD. Older patients and those with severe disease are 12 times more likely to develop dementia compared with younger patients with mild disease ( 92 ). Low baseline cognitive scores, early development of confusion or hallucinations on dopaminergic medications, postural imbalance, and excessive daytime sleepiness have also been associated with increased risk of dementia ( 77 ).

Similar to DLB, the cognitive profile of PDD is a dysexecutive syndrome with early and prominent impairment of attention and visuospatial functions, moderately impaired episodic memory, and relatively preserved core language function. Similar to DLB, a range of neuropsychiatric symptoms can be displayed in PDD; the most common symptoms include hallucinations, apathy, depression, and insomnia. Visual hallucinations, similar to those displayed in DLB, are well-formed and vivid, often with preserved insight and little emotional content ( 77 ). Delusions are also common. Autonomic disturbances such as urinary incontinence as well as orthostatic and postprandial hypotension are frequent in PDD.

As previously discussed, PDD is characterized by a variable combination of synucleinopathy (Lewy body degeneration), cellular loss in subcortical nuclei, and Alzheimer’s disease pathology (Aβ plaques and tau protein forming neurofibrillary tangles). Degeneration of subcortical nuclei results in various neurochemical abnormalities, including cholinergic, dopaminergic, serotonergic, and noradrenergic deficits, of which cholinergic loss is the most prominent. Loss of cholinergic neurons in the nucleus basalis of Meynert (nbM) is greater than that displayed in Alzheimer’s disease, and Lewy bodies are more frequently found in the nbM ( 77 ).

As in all of the neurodegenerative disorders discussed previously, no cure exists for dementia caused by either PD or LBD. Symptomatic treatment remains the focus of medical interventions.

Patients with PDD or DLB have significant cholinergic deficits; therefore, AChEIs such as donepezil or rivastigmine represent the logical choice for pharmacological treatment of dementia in both cases. Donepezil is the most widely used AChEI in the treatment for dementia, and it selectively inhibits acetylcholinesterase (AChE). Rivastigmine is unique in that it has both AChE and butylcholinesterase inhibitory activity. In 2012, the FDA approved rivastigmine for mild to moderate dementia associated with PD on the basis of the 2004 study by Emre et al. ( 93 ), a large, randomized, double-blind, placebo-controlled study of rivastigmine lasting 24 weeks among 541 patients with mild to moderate dementia that developed at least 2 years after receiving a clinical diagnosis of PD. Patients treated with rivastigmine had a mean improvement of 2.1 points on the 70-point Alzheimer’s Disease Assessment Scale, with a baseline score of 23.8, compared with a 0.7-point worsening in the placebo group, with a baseline score of 24.3 (p<0.001). The study also demonstrated clinically meaningful improvements in the scores for the Alzheimer’s Disease Cooperative Study-Clinician’s Global Impression of Change; improvements were observed in 19.8% of patients in the rivastigmine group and 14.5% of those in the placebo group, and clinically meaningful worsening was observed in 13.0% and 23.1%, respectively (mean score at 24 weeks=3.8 and 4.3, respectively; p=0.007).

When the psychotic symptoms of DLB and PDD become more distressful or threatening, the use of low doses of second-generation antipsychotics with low D2 potency, such as quetiapine, should be considered to minimize motor side effects. Pimavanserin, a novel antipsychotic drug that is a selective 5-HT2A receptor inverse agonist, was recently approved by the FDA for the treatment of psychosis in PD ( 94 ). It has negligible effects on dopamine and histamine receptors; therefore, it avoids the motor and sedative side effects common with other antipsychotics. However, risks are still associated with pimavanserin, especially the prolongation of the QT interval. Like other antipsychotics, risk of death is increased with pimavanserin among elderly patients with dementia.

Conclusions

Dementia is a significant contributor to the U.S. burden of disease, and it is projected to significantly increase over the next few decades as the U.S. population continues to grow. In this review, I apprised some of the more common dementia syndromes from among dozens of different diseases. The clinician’s ability to understand and diagnose distinct types of neurodegenerative disorders on the basis of core clinical and biological features is essential for the success of future research trials and therapeutic interventions.

The author reports no financial relationships with commercial interests.

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Case study 2 for neurocognitive disorders: Alice ? Name: Alice Age: 74 Sex: Female Family: Widowed,. 1 answer below »

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Case study 2 for neurocognitive disorders: Alice Х Name: Alice Age: 74 Sex: Female Family: Widowed, two adult children Occupation: Retired philosophy professor Presenting problem: Memory impairment, irritability, confusion Alice&#39;s adult children have brought her in for an evaluation following a recommendation from her physician. According to her children, Alice has become increasingly forgetful and irritable and has been doing and saying strange things. Her children report that Alice&#39;s husband of 45 years, Bob, died nearly six years ago. Her family initially believed that her increasing forgetfulness, such as failing to pay bills, was merely caused by grief, but they say that her condition has gotten worse. Alice&#39;s children grew increasingly concerned as Alice showed signs of general lack of self-care and increased irritability that were out of character for her. For example, according to her daughter, Alice has always styled her own hair but now isn&#39;t able to recall how she likes it and goes days without fixing her hair. Alice has lived in the same house for more than 50 years but has gotten lost in her neighborhood several times during the past year. Recent developments have brought acute concern to the family: Less than two months Alice began telling her children that their father was going to be coming home soon from his overseas business trip. When her children tried to remind Alice of their father&#39;s passing, Alice told them that they were wrong, and their father was just away on another business trip. According to her son, when he arrived today to drive Alice to her appointment, she answered the door and did not seem to recognize him. During the evaluation, Alice appears slightly disheveled but alert. When asked questions, her responses are appropriate but don&#39;t demonstrate the quick wit and energy her children say Alice was known for. Her scores on short-term memory and sequencing tasks show substantial deficits and impairment. Alice is repeatedly unable to recall the clinician&#39;s name. General physical examination, lab results, and MRI findings were all normal for Alice&#39;s age range. Alice takes multivitamins and medication for mildly elevated blood pressure. There is no history of alcohol or drug use, no history of psychiatric disorders, and no history of stroke. Alice&#39;s condition will have a significant impact on her whole family. Which of the following is not an example of the stresses her family may face due to Alice&#39;s neurocognitive disorder?

O As Alice requires more attention and supervision, her children may fight over how to split up responsibilities for her long-term care.

O Alice&#39;s children may grow frustrated as the relationship with their mother changes-no longer is Alice taking care of them; instead, her children will be taking care of her.

O Because Alice&#39;s death is imminent, her children will fight over specific items in her inheritance.

O Strong feelings about whether Alice can or should continue to live independently may cause conflict within the family. Family members may disagree about whether to tell a person in an advanced stage of major neurocognitive disorder about her diagnosis. Which of the following represents the best reason to share this information with Alice?

Alice&#39;s doctors have an obligation to discuss her diagnosis and treatment options with her directly because she is the patient. Alice will not be able to understand the diagnosis or treatment options. Alice won&#39;t remember the diagnosis anyway, so it will not be upsetting to her.

O If they tell Alice about her diagnosis, she will become upset every day when she is reminded of the diagnosis, as if learning it for the first time. Diagnostic criteria checklist Some of the symptoms associated with various neurocognitive disorders are listed in the following table. In the Present column, indicate which symptoms are clearly present in Alice&#39;s case. Check all that apply. Symptoms Present Fluctuating level of attention and consciousness

O Experiencing short-term memory impairment Gradual onset of progressive symptoms

O Symptoms causing significant impairment in functioning

O Indications of cerebrovascular disease Potential diagnosis According to the full diagnostic criteria listed by the DSM-5 for neurocognitive disorders (not just the symptoms highlighted in the previous question), Alice appears to meet the criteria for a diagnosis of Grade It Now Save & Continue Continue without saving nt in functionin neurocognitive disorder due to Alzheimer&#39;s disease vascular neurocognitive disorder neurocognitive disorder due to Parkinson&#39;s disease neurocognitive disorder due to traumatic brain injury oms highlighted in th ed by the gnosis of

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