Chronic brain failure, also known as dementia or major neurocognitive disorder, is a syndrome of progressive functional decline characterized by both cognitive and neuropsychiatric symptoms. It can be conceptualized like other organ failure syndromes and its impact on quality of life can be mitigated with proper treatment. Dementia is a risk factor for delirium, and their symptoms can be similar. Patients with dementia can present with agitation that can lead to injury. Logic and reason are rarely successful when attempting to redirect someone with advanced dementia. Interactions that offer a sense of choice are more likely to succeed.
Key points
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Chronic brain failure is also known as dementia or major neurocognitive disorder. Like other organ failure syndromes, its impact on quality of life can be mitigated with proper management.
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In addition to cognitive symptoms such as memory loss, chronic brain failure involves neuropsychiatric symptoms such as psychosis, mood lability, and agitation.
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The symptoms of chronic brain failure can mimic those of acute brain failure (delirium), but there are major distinctions to help guide diagnostic thinking.
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Neuropsychiatric symptoms are best managed nonpharmacologically. Medications to treat agitation should be used only as a last resort.
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The use of logic and reason are rarely successful when attempting to redirect someone with advanced dementia. Interactions that offer a sense of choice are more likely to succeed.
Case
Pertinent history : A 78-year-old man with a history of Alzheimer’s disease (AD) was sent to the emergency department (ED) after becoming aggressive toward staff at his assisted living facility (ALF). Over the prior week, he had been leaving the facility repeatedly, accusing staff of stealing his belongings and poisoning his food. On the day of presentation, he struck a staff member while she was attempting to redirect him. He has had no recent medical illness.
Past medical history: Hypertension, osteoarthritis, and benign prostatic hyperplasia. AD diagnosed by his primary care provider approximately 5 years ago. Currently, he receives his primary care through the ALF’s nurse practitioner.
Surgical history: Left total hip arthroplasty 6 months prior
Medications: Hydrochlorothiazide 25 mg twice daily, tamsulosin 0.4 mg daily, trazodone 25 mg nightly, quetiapine 100 mg nightly (started 1 week prior), lorazepam topical gel 2 mg twice daily as needed for anxiety/agitation (started 2 days prior)
Family history: Mother died of AD at age 80, father died of myocardial infarction at age 54
Social history: 10th grade education, retired assembly-line worker, divorced, one daughter who lives out of state and is designated as his power of attorney; 30 pack-year history of smoking
Pertinent physical examination: Temperature: 37.2˚C; blood pressure: 141/93; heart rate: 110; respiratory rate: 20; oxygen saturation by pulse oximetry: 99% on room air
General: Alert, thin, disheveled, smelling of urine. Oriented to self only. Minimally cooperative, attempting to leave ED, states that there is nothing wrong with him.
Skin: No rashes or petechiae
HEENT : Pupils equal, round, reactive to light, mucous membranes dry, conjunctivae pale
Neck : Full range of movement (ROM), neck veins flat
Cardiovascular : Regular rhythm, tachycardic, no murmurs/rubs/gallops, distal pulses equal bilaterally
Pulmonary : Rate increased, lungs clear with no wheezes, rales, or rhonchi
Abdominal : Soft, nontender, nondistended, normal bowel sounds
Musculoskeletal : Normal pulses throughout, full ROM of all extremities, no peripheral edema
Neurologic : 5/5 strength and normal sensation throughout, gait slightly unsteady
Psychiatric : Dysphoric mood, irritable affect, paranoid delusions, no hallucinations
Montreal Cognitive Assessment (MoCA) = 11/30
Diagnostic testing : A delirium workup was initiated.
| WBC | 6.6 k/μL |
| Hgb | 13.2 g/dL |
| Hct | 39.5% |
| Plt | 190 k/μL |
| Na | 128 mmol/L |
| Potassium | 4.2 mmol/L |
| Cl | 97 mmol/L |
| CO 2 | 19 mmol/L |
| Glucose | 139 mg/dL |
| BUN | 20 mg/dL |
| Creatinine | 1.51 mg/dL |
Electrocardiogram : Sinus tachycardia, normal axis and intervals, no ST elevation or depression, T waves normal
Chest radiograph : Normal
Head CT : There is global parenchymal volume loss. The ventricles are enlarged commensurate with parenchymal loss. No acute intracranial process is identified.
Clinical course : The patient was thought to be at high risk for falls given his agitation and minimal cooperation with staff. He was also physically threatening. He repeatedly asked for his wife and was told that she was “out of town.” After several failed attempts to administer oral medication for acute agitation, the patient received olanzapine 5 mg intramuscularly. He remained agitated after 15 minutes so was assigned a one-to-one staff companion for constant monitoring. After approximately 40 minutes, he was awake and alert, remaining seated in a recliner.
The patient was a poor historian, so collateral history was obtained from a nurse at the ALF. She reported that she had known the patient since he moved there 2 years ago. He had some moderate memory loss that worsened precipitously 6 months ago following an extended hospitalization for a hip replacement with postoperative delirium. He was ordinarily quiet and “a loner,” but over the past few months he had become more anxious and irritable. He was frequently restless, especially late in the day, and began accusing staff of stealing clothing and personal items from his room. He had difficulty falling asleep, and awoke several times throughout the night, usually to urinate. Within the past few weeks, he was sleeping very little overall. He normally had a good appetite but was eating and drinking less over the past few weeks, accusing staff of poisoning his food. The ALF medical provider had prescribed quetiapine and lorazepam gel without any benefit. The patient had wandered away from the ALF several times recently, but always returned willingly. On the day of presentation, however, he struck a staff member who attempted to direct him back inside the building.
The nurse reported that she had consulted with the ALF Director; they felt that the patient required a “higher level of care” and thus would be discharged from their facility.
Several attempts to contact the patient’s daughter were unsuccessful.
Introduction and Background
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Chronic brain failure is a complex, acquired syndrome characterized by progressive and usually irreversible decline in multiple areas of higher mental function, resulting in a gradual loss of independent daily living activities. This loss of independent function has a wide-ranging impact on individuals, families, and health care systems.
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Chronic brain failure is an umbrella term, used to describe a class of disorders more commonly known as dementias and major neurocognitive disorders (these terms are used interchangeably in this article, reflecting their use in the literature). These terms emphasize consequences rather than causes; the syndrome can result from a number of different disease processes.
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Chronic brain failure is predominantly, although not exclusively, a disorder of later life, typically affecting persons older than 65 years.
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Older adults with dementia are frequent ED visitors who have greater comorbidity, incur higher costs, are admitted to hospitals at higher rates, return to EDs at higher rates, and have higher mortality after an ED visit than patients without dementia. They are also associated with higher rates of delirium, falls, behavioral problems, and physical decline.
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Patients with dementia are viewed as particularly challenging to ED clinicians. Although most patients do not present with a chief complaint of dementia, their dementia and related symptoms often overshadow the primary reason for the ED visit.
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Chronic brain failure is distinct from acute brain failure (ie, delirium); the initial diagnosis cannot be made if delirium is present. Nevertheless, dementia and delirium are frequently encountered in tandem. As illustrated by the preceding case example, dementia is a risk factor for delirium, and delirium can accelerate the progression of dementia.
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Diagnosis and management of major neurocognitive disorder spans multiple medical specialties, including neurology, psychiatry, geriatrics, and general internal medicine, yet no one specialty clearly “owns” the diagnostic class. This can complicate treatment and disposition planning.
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The nature of their symptoms makes people with dementia more dependent and vulnerable, both socially and in terms of physical and mental health.
Prevalence/Incidence
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Within the United States, approximately 5.7 million people are living with dementia.
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As the population worldwide continues to age, the number of individuals at risk will also increase, particularly among the very old.
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The prevalence doubles every 5 years after the age of 65, with more than 20% of 80-year-olds having moderate-to-severe dementia.
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AD is the most prevalent subtype of dementia, accounting for 60% to 80% of cases.
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ED-based studies of cognitive impairment report that up to 70% of older adults seen with cognitive impairment have undiagnosed dementia.
Physiology/Pathophysiology
The clinical syndrome of major neurocognitive disorder can be caused by several different pathophysiological processes that alter or damage nerve cells and synapses in the brain. The diagnostic category is broken down into corresponding subtypes based on clinical, genetic, and neuropathological features. The most common of these is AD, followed by vascular dementia, dementia with Lewy bodies, and frontotemporal dementia ( Table 1 ). Less common causes include Huntington disease, normal pressure hydrocephalus, Parkinson disease, traumatic brain injury, substance/medication use, human immunodeficiency infection, and prion disease.
| Disease | Pathology | Time Course | Clinical Features |
|---|---|---|---|
| Alzheimer’s disease | Characterized by plaques, tangles, and neuronal loss. | Insidious onset. Slow progressive cognitive and functional decline. | Almost always includes neuropsychiatric symptoms in later stages. |
| Vascular dementia | Results from cerebrovascular disease. | Stepwise progression with variable rates of decline. | Often associated with focal neurologic signs such as spasticity, hemiparesis, and extrapyramidal signs. Apathy and depression are common. |
| Dementia with Lewy bodies | α-Synuclein aggregates in neurons. | Progressive cognitive decline. | Includes fluctuating cognition, visual hallucinations, parkinsonism, rapid-eye movement sleep disorder, and hypersensitivity to antipsychotics. |
| Frontotemporal dementia (includes Pick disease) | Focal degeneration of frontal and temporal lobes. Involves hyperphosphorylated tau protein inclusion bodies. Knife-edge atrophy on MRI. | Typically a more rapid rate of decline than with Alzheimer’s disease. | Progressive change in personality, behavior, and language. Motor impairment syndromes co-occur. |
The clinical symptoms and pathophysiological processes of these diseases overlap significantly. For example, research indicates that at least one-third of AD cases are complicated by some degree of vascular pathology. Determining what specific subtype of major neurocognitive disorder a patient has can be important for prognosis and long-term treatment planning. For the emergency physician evaluating a patient with cognitive impairment in the ED, however, it is more important to differentiate between acute/potentially reversible causes and chronic/irreversible ones.
Clinical Characteristics
Dementia subtypes can be difficult to diagnose clinically because of their multifactorial causes, overlapping symptoms, and inconsistent clinical presentations. Within the category, the most commonly used diagnosis code is Dementia Not Otherwise Specified, which likely reflects these diagnostic challenges.
Fortunately, patients usually present to the ED with an existing diagnosis of dementia; the initial diagnosis is not commonly made by emergency physicians. Nevertheless, an understanding of the clinical characteristics of dementia can aid the emergency physician in managing the patient with chronic brain failure.
Although the hallmark of dementia is memory loss, symptoms typically involve multiple domains of brain function:
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Complex attention
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Experiences difficulty processing multiple stimuli (television, radio, conversation)
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Gets easily distracted by competing events in the environment
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Is unable to perform mental calculations
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Executive functioning
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Abandons complex projects
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Needs to focus on one task at a time
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Needs assistance with activities of daily living and making basic decisions
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Learning and memory
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Repeats self in conversation, often within the same conversation
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Cannot keep track of short lists of items such as shopping lists or plans for the day
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Requires frequent reminders to orient to task at hand
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Language
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Experiences significant difficulties with expressive and/or receptive language
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Often uses vague, general phrases such as “that thing” and “you know what I mean”
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Prefers general pronouns rather than names
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With severe impairment, may not recall names of friends and family, and may lose fluent language
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- 5.
Perceptual-motor
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Struggles with previously familiar activities (using tools, driving a car)
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Gets lost in familiar environments
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Often gets more confused at dusk, when shadows and lowering levels of light change perceptions
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- 6.
Social cognition
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Exhibits behavior clearly out of acceptable social range
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Demonstrates lack of sensitivity to social standards of modesty in dress and restraint in political, religious, or sexual topics of conversation
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In addition to impairments in the preceding cognitive domains, dementia can involve noncognitive neuropsychiatric symptoms, which include the following:
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Mood disturbance (depression, irritability)
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Psychosis (hallucinations, delusions)
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Agitation
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Aggression
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Apathy
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Sleep disturbance
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Disinhibition
Despite being almost universally present in chronic brain failure, noncognitive neuropsychiatric symptoms have not been included in the diagnostic criteria for dementia in the current classification system ( Box 1 ).
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